A previously healthy 69 year old male was evaluated in Outpatient Allergy Clinic. He reported a history of sudden onset of lip and tongue swelling, throat tightness, shortness of breath, cough and wheeze within five minutes of sucking on a peppermint candy. He took two tablets of diphenhydramine 25 mg and his symptoms progressed to difficulty with talking and swallowing. He was urgently transported to Emergency Department where he was administered epinephrine intramuscularly, parenteral antihistamines and steroids. His vitals remained stable throughout the episode and he denied any urticarial lesions or gastrointestinal symptoms. His symptoms resolved within 3–4 hours. On history, he reported frequent consumption of foods with peppermint flavour such as chocolates, candies, ice cream, as well as peppermint tooth paste, which he had tolerated well. However, a few weeks prior to this episode, he had developed isolated tongue and lip swelling with the use of Colgate peppermint toothpaste and had avoided it since, although he did not consider the peppermint flavour to be the culprit. During summer months of the preceding three years, he had cultivated peppermint plants in his backyard and admitted to having experienced nasal congestion, cough and a slight wheeze when gardening. His past medical history was remarkable for gout, dyslipidemia and gastroesophageal reflux. He had no prior history of atopy. His medications were simvastatin, omeprazole and aspirin. Skin prick testing was performed to common inhalants and was positive to dust mites, but negative to other common inhalants, including eight types of moulds. Skin testing was negative to commercially available food allergens of egg white, cow’s milk, fish, shellfish, peanut, tree nuts, wheat, soy and sesame. Skin prick testing to a saline slurry of peppermint candy demonstrated a wheal and flare with largest diameters of 10 mm and 35 mm (W10/F25), respectively (Figure
a). Prick-to-prick test with fresh peppermint leaf provided by the patient revealed a skin test response of W25/F50 (Figure
b). Both peppermint tests were negative when applied on five healthy volunteers. Patient and healthy volunteers displayed appropriate histamine (W3F25) and negative control (W0F0) skin responses. Our patient was advised to avoid any traces of peppermint and menthol and carry an epinephrine autoinjector. He removed the peppermint plants from his backyard and returned to gardening the following summer without experiencing any symptoms. Later on, he had an accidental exposure to lip balm with traces of peppermint flavour causing solitary lip swelling that responded to oral diphenhydramine.


We report a case of a 20-year-old man transferred to our clinic under suspicion of myocarditis. He presented with dyspnea, fatigue, myalgia, fever, and myoglobinuria. The patient reported those three days before becoming symptomatic, he was subjected to a more intense physical work (unloading a truck). The next day he felt like coming down with a cold, he felt weak and had muscle pains. Later that day, he was admitted to the ER with fever (40°C) where he received a parenteral therapy with NSAIDs. After the parenteral therapy, he complained of worsening of the symptoms with strong myalgia, dyspnea, fatigue, sweating, and myoglobinuria.
Influenza B was identified, and increased markers of inflammation-fibrinogen, CRP, leukocytosis, plasma levels of CPK up to 114000 IU, CK-MB 5400 IU, and myoglobin up to 33000 IU were detected, resulting in acute renal failure (necessitating plasmapheresis and hemodialysis treatments), respiratory insufficiency (necessitating a noninvasive ventilation support), and a mild to moderate hepatic lesion.
During the whole period, the blood glucose levels were in the range of 3, 4-5, 4 mmol/L (lower normal limit), and no significant hypoglycemia was registered.
Muscle biopsy identified exacerbated chronic vasculitis with atrophy of certain muscle cells. The immunologic markers and hemoculture were negative. The pneumoslide was only positive to IgM for Influenza B.
The echocardiographic examination revealed enlarged left ventricle (LVEDd 65; LVEDs 48) with EF of 47% by the Simpson method. Strain analysis using speckle tracking identified GLPS average of −13.8% ().
After one-month hospitalization period, the patient was stabilized and discharged from the hospital. During the nine-month follow-up period he was stable, completely recovered, and asymptomatic, except for a mildly impaired left ventricular systolic function.
His prior history revealed that the patient used to feel cramps and pain in the legs after prolonged walking in the childhood. He experienced a similar episode three years earlier after prolonged exercise (body building for several hours), exposure to a cold temperature, and infection (febrile illness), when he was hospitalized in another institution with severe signs of rhabdomyolysis (creatinine phosphokinase up to 42000 IU) and hepatic lesion (AST, ALT up to ×10 over the upper limit) and was diagnosed with cardiomyopathy with increased LV dimensions LVEDd > 60 mm, EF 45%. There were no signs of renal impairment at that time. He was stabilized and discharged from hospital. During attack-free periods, the patient was asymptomatic.
The patient had a deceased father from heart failure of unknown origin and a sister that died at the age of 18 months in unclear circumstances after “being sick all the time” when she received an injection for fever.
During hospitalization, the diagnosis of CPT II deficiency was suspected and the patient was referred for genetic analyses. Sequencing analysis of exons 1, 3, and 4 of the CPT II gene revealed that the patient was homozygote for Ser 113 Leu mutation (). Diagnosis of adult myopathic form of carnitine palmitoyltransferase II deficiency was made based on the genetic findings and signs and symptoms as well as the age of onset.


A 78-year-old Japanese-Mongoloid woman (body weight: 48kg) with a 3-year history of hypertension and hyperlipidemia visited our hospital because of a persisting fever and generalized joint pains which had developed 2 weeks before initial presentation. She had been treated as having a common cold at a local clinic for 2 weeks previously, but her symptoms had not improved. After visiting our hospital, a drip infusion therapy of cefpirome with oral administration of loxoprofen was initiated on an out-patient basis. However, this treatment was not effective for the fever but caused toxic eruptions on the back of both her hands. Thus, this treatment was suspended 3 days later. She was admitted 4 days after initial presentation. Neither respiratory nor ocular symptoms were present. She had past history of panhysterectomy due to uterine cancer at the age of 38 and a fracture of her left wrist joint at the age of 68. She had no environmental or occupational history of beryllium or other metal exposure. A physical examination on admission showed bilateral inguinal and axillary lymph node swelling and erythematous eruptions on the back of both her hands. A chest X-ray showed minimal bilateral hilar lymphadenopathy (BHL); however, a chest computed tomography (CT) scan clearly revealed mild BHL without pulmonary infiltrates (Figure ). Since respiratory function tests were normal, bronchoscopy was not performed. Electrocardiogram and ophthalmologic evaluations were normal. A complete blood cell count showed slight anemia (red blood cell count, 3.70×1012/L; hemoglobin, 10.8g/dL), slight leukocytosis (white blood cell count, 11.9×109/L with 74% neutrophils, 13% lymphocytes, 11% monocytes, 1% eosinophils, and 1% basophils) and normal platelet count (360×109/L). Elevated levels of erythrocyte sedimentation rate (110mm/hour), C-reactive protein (CRP; 13.73mg/dL; normal range 0 to 0.26mg/dL), soluble-interleukin (IL)-2 receptors (s-IL2R; 1300IU/mL; normal range 124 to 466IU/mL), antinuclear antibodies (×640; normal range <×40) and ferritin (722ng/mL; normal range 39.4 to 340ng/mL), and reduced levels of serum iron (34μg/dL; normal range 54 to 181μg/dL) and albumin (2.5g/dL; normal range 3.9 to 4.9g/dL) were observed. Serum electrolytes and renal function indices were normal. Rheumatoid arthritis particle agglutination, anti-double-stranded deoxyribonucleic acid (DNA), anti-Sm, anti-thyroglobulin, anti-microsome, anti-Ro and anti-La antibody titers were within normal limits. No increments of serum angiotensin-converting enzyme and lysozyme were seen. The results of an anti-acid fast bacterium antibody and a tuberculin skin test were negative (0×0mm). Serologic tests for syphilis, hepatitis B virus, hepatitis C virus and human immunodeficiency virus were negative. Serum Epstein–Barr virus (EBV) and Chlamydia pneumoniae titers showed prior infection patterns. The results of serologic studies for Cytomegalovirus, Brucella, Legionella, Coxiella burnetii, Mycoplasma and Toxoplasma were negative. Her urine showed nothing remarkable.
A positron emission tomography-CT (PET-CT) scan, which was carried out 4 days after admission, showed 18F-fluorodeoxyglucose (FDG) uptakes in her peripharyngeal, axillary, mediastinal, hilar, iliac and inguinal lymph nodes with splenic involvement. In addition, remarkable FDG uptake at her submandibular dental roots wearing ceramic crowns was observed, suggesting chronic periodontitis (Figure ).
A right inguinal lymph node biopsy specimen, which was obtained 9 days after the initiation of CAM treatment, showed non-caseating epithelioid cell granulomas with abundant multinucleated giant cells (Langhans giant cells) in the background of epithelioid cells, macrophages, lymphocytes and plasma cells (Figure A). In the present case, the sarcoid granulomas were somewhat diminished in size and number, and ill defined from surrounding tissue (Figure B). In addition, some of the giant cells showed condensed heterochromatin (pyknosis; Figure A), multiple fragmented nuclei (apoptotic bodies; Figure A) and multiple nuclear remnants with indistinct nuclear configurations in the eosinophilic condensed cytoplasm (Figure B). These findings indicate that the giant cells were, although atypical, in the apoptotic process according to the features described by Wyllie et al.[]. These degenerated giant cells appeared to have transformed into eosinophilic homogenous substances with fibrotic configuration (that is, hyaline-like degeneration; Figure C). The degenerating/degenerated giant cells showed slightly increased stainability with periodic acid–Schiff (Figure D). Neither acid-fast bacterium nor fungus was demonstrated by Ziehl–Neelsen and Grocott stains (not shown). Immunohistochemical staining for Mycobacterium tuberculosis showed a negative result (not shown). EBV-encoded small ribonucleic acid (RNA) was not detected in the sarcoid granulomas (not shown). To demonstrate the presence of P. acnes in the granulomas, the lymph node specimens were tested by immunohistochemistry using a specific monoclonal antibody against P. acnes lipoteichoic acid (PAB antibody). PAB-positive reaction products were observed preferentially in the degenerated homogenous substances. Small positive dots were scattered sparsely in the sarcoid granulomas (Figure ).
Since a considerable number of plasma cells were admixed with lymphocytes in the lymph node, immunohistochemical staining was carried out for immunoglobulin (Ig) G, IgA, IgM, IgD and IgG4 to dispel the possibility of IgG4-related disease (Figure ). Infiltrated plasma cells were positive for IgG, IgA, IgM, IgD and IgG4 at different degrees. Positive reactions for IgG4 were only occasionally seen in the plasma cells, thus negating the possibility of IgG4-related disease. Some of the giant cells and degenerated homogenous substances were positively stained with anti-IgG antibody, and the reaction was more intense in degenerating/degenerated giant cells than in intact ones (Figure A). The giant cells were faintly stained with anti-IgD antibody (Figure B) but not with anti-IgA (not shown), anti-IgM (not shown) and anti-IgG4 (Figure C) antibodies.
In this case, an oral administration of CAM (200mg at 12-hour intervals) coupled with acetaminophen (400mg at 12-hour intervals) was empirically initiated on the day of admission. After the initiation of this treatment, her fever rapidly subsided coupled with the disappearance of joint pains and toxic eruptions within 1 week. The elevated levels of CRP returned to normal within 2 weeks and elevated s-IL2R levels were remarkably decreased within 3 weeks (Figure ). She was discharged 25 days after admission and followed as an out-patient. Administration of acetaminophen was suspended at the time of discharge. The levels of s-IL2R returned to normal within 2 months. The follow-up PET-CT scan 2 months after admission showed minimal FDG uptake in her bilateral hilar lymph nodes and distinct uptake at submandibular dental roots and in peripharyngeal lymph nodes. The FDG uptake in her spleen also disappeared (Figure ). CAM treatment was discontinued 3.5 months after its initiation. No unfavorable adverse effects were observed with CAM. During the course of illness, the patient was found to have urinary bladder cancer, which was successfully removed by endoscopy in the urologic department of another hospital. Her levels of s-IL2R remained slightly elevated (less than 750IU/mL) after cessation of CAM treatment without any symptoms. She stopped visiting our hospital 1.5 years after initial presentation because of traffic inconvenience to visit the hospital. Recently, she visited our hospital to undergo medical check-ups and was told that she was in good condition.


A 30-year-old Japanese woman (height 161cm, weight 57kg) was diagnosed with an ovarian germ cell tumor at age 12 and underwent total hysterectomy and bilateral oophorectomy. The histological diagnosis was an immature teratoma with metastasis to her lymph nodes. She underwent chemotherapy for a short time after the surgery, but then dropped out of treatment and did not receive further therapy. She gradually developed a recurrent abdominal mass and remained under observation. At age 30, she presented to our hospital requiring an operation, and tumor extirpation was scheduled.
A physical examination revealed a huge abdominal mass and a chest X-ray revealed bilateral diaphragmatic elevation. She could not adopt a supine position because of the tumor causing respiratory distress (Figure ). A computed tomography scan showed a 40×40cm mass (Figure ). Her vital signs were blood pressure 100/70mmHg, heart rate 100 beats per minute, and pulse oximeter oxygen saturation (SpO2) 88 to 90%. Laboratory tests revealed an increased fibrin degradation product of 15.3μg/mL and D-dimer of 2.6μg/mL. Pulmonary function tests showed a low vital capacity (VC) based on the predicted value (%VC 37.7%) and arterial blood gas analysis revealed hypoxia (partial pressure of carbon dioxide, PaCO2, 44.6mmHg; partial pressure of oxygen, PaO2, 56.2mmHg while breathing room air).
Prior to induction of general anesthesia, an arterial catheter (JELCO I.V. Catheter®; Smith Medical Japan Ltd., Tokyo, Japan) was inserted into her radial artery and a central venous catheter (PreSep Oximetry Catheter®; Edwards Lifesciences, Irvine, CA, USA) was inserted in her left internal jugular vein. Furthermore, percutaneous sheaths (5 Fr Radifocus Introducer®; Terumo Corporation, Tokyo, Japan) were inserted in her femoral artery and vein to use for percutaneous cardiopulmonary support (PCPS) as necessary. Radial arterial pressure, electrocardiogram, capnography, SpO2, and cerebral regional oxygen saturation (INVOS 5100®; Somanetics, Troy, MI, USA) were continuously monitored throughout the surgery. After the percutaneous sheaths were inserted, lidocaine 4% was sprayed on her upper airway, while midazolam 1mg and fentanyl 50μg were given intravenously and awake fiberoptic intubation was performed in the Semi-Fowler’s position. We did not use muscle relaxant before the operation because we were concerned about ventilation if spontaneous respiration was lost. During intubation, her tidal volume (TV) decreased slightly because of weakness of spontaneous respiration, but SpO2 was maintained and intubation was performed uneventfully. After intubation, midazolam 4mg was administered and anesthesia was maintained with 1.5% sevoflurane. Her TV with spontaneous respiration controlled by pressure support ventilation and positive end-expiratory pressure (PEEP) of 5cmH2O was kept between 250 and 350mL.
After surgeons completed preparation for the operation and the surgical site was disinfected, a muscle relaxant (rocuronium 50mg), fentanyl 200μg and 0.3μg/kg/minute remifentanil were administered. Spontaneous respiration was lost and we were unable to obtain a sufficient TV with less than the 20cmH2O airway pressure under pressure controlled ventilation or manual ventilation. However, an abdominal midline incision was performed by the surgeon and her TV gradually increased. Her TV further increased and SpO2 recovered after median sternotomy. An inspiratory pressure of 20cmH2O and PEEP of 8cmH2O resulted in a TV of 300 to 350mL. Arterial blood gas analysis at a fraction of inspired oxygen of 0.6 gave results of PaCO2 41.8mmHg and PaO2 197.5mmHg.
Resection of her tumor caused massive blood loss that required blood transfusion. During the operation, 3 to 5μg/kg/minute dopamine was given and her mean arterial pressure was kept above 60mmHg. Finally, her tumor was removed completely and had a weight of 10.5kg. On histological examination, the tumor was diagnosed as a mature teratoma without malignancy. Her total blood loss was 9790mL and 22 units of red cell concentrate, 19 units of fresh frozen plasma, and 20 units of platelet concentrate were transfused. After removal of her tumor, the same TV was maintained with lower inspiratory pressures and PEEP to prevent re-expansion pulmonary edema (RPE). On completion of the operation, she was transferred to the intensive care unit (ICU) and ventilated with an inspiratory pressure of 18cmH2O and PEEP of 8cmH2O, which resulted in a TV of 350mL. After her spontaneous respiration recovered, she was ventilated with pressure support and PEEP to maintain a TV of 300 to 350mL. She was extubated on postoperative day (POD) 2 and discharged from the ICU without any complication on POD 5.


A 32-year-old G2P1 Caucasian expectant mother was referred for a fetal echocardiogram at an estimated gestational age of 20 weeks secondary to the presence of a fetal omphalocele. The echocardiogram demonstrated the fetus to have a large omphalocele, a lower sternal defect, and a midline cardiac apex with a small portion of the ventricles protruding through a pericardial and diaphragmatic defect onto the surface of the liver. The intracardiac anatomy was found to be consistent with tetralogy of Fallot. The care of the mother was transferred to a high-risk, maternal-fetal medicine physician with a plan for delivery at a tertiary care center and subsequent transfer to a nearby children’s hospital for postnatal management.
The mother presented in active labor at 38 4/7 weeks’ gestation. Rupture of membranes, with meconium staining, occurred one hour prior to delivery, and a male baby was delivered without complication via a vaginal birth after a Cesarean section. At delivery, the baby demonstrated no respiratory effort, and his heart rate was <100 beats per minute. The baby was placed in a plastic bag and intubated on the first attempt. His heart rate quickly improved. A physical examination demonstrated a sternal defect covered by intact skin and a palpable cardiac impulse just beneath his skin at the level of the sternal cleft. There was a large, epigastric omphalocele containing visible liver, stomach and intestines. The remainder of the physical examination was normal, including a three-vessel umbilical cord. His birth weight was 3630g and Apgar scores were 2, 6 and 7 at 1, 5 and 10 minutes of life, respectively. Our patient was hemodynamically stable and was then transferred to our institution.
In our neonatal intensive care unit, we adopted a conservative management plan of the omphalocele: to forgo surgical repair and undertake daily dressing changes with antiseptic ointment while awaiting spontaneous epithelialization. Transthoracic echocardiography confirmed the presence of tetralogy of Fallot, with mild valvar pulmonary stenosis, and continuous, mildly hypoplastic branch pulmonary arteries. With spontaneous closure of the ductus arteriosus, our patient maintained a normal oxygen saturation level, and we planned for a complete repair on an elective basis at 3 to 6 months of age.
A computed tomography scan of his thorax confirmed the deficiency of his lower sternum, with the anterior aspect of his right ventricle just beneath the skin, as well as the deficiency of his anterior diaphragm (Figure ). His liver, spleen and stomach were herniating through the anterior abdominal wall defect into the omphalocele. We found dependent atelectasis of his lungs. The remainder of the computed tomography scan and head and renal ultrasounds were normal. The baby was extubated on his 20th day of life, and continues to have daily dressing changes to the omphalocele at the time of writing.
A 180K Oligo Chromosome Microarray Analysis, performed by Ambry Genetics (Aliso Viejo, CA, USA) was significant for a gain in copy number (x3) in chromosome 15q21.3 with a minimum interval from 57,529,846 to 58,949,448. Parental fluorescent in situ hybridization analysis using probe RP11-345H13 revealed the presence of a 15q21.3 duplication within the mother, indicating maternal inheritance from a phenotypically normal mother with no known diaphragmatic, sternal, pericardial, abdominal wall or intracardiac defects.


A 43 year-old female with no allergies and with a history of osteoarthritis, osteoporosis and Gilbert’s syndrome was seen in a dental clinic for implant-supported restoration of the maxillary anterior teeth. During the clinical examination, a lesion with a diameter of 0.5 cm, a whitish-yellow aspect, of soft elastic consistency located in the oral mucosa in the left upper central incisor area was observed. The patient reported an episode of sudden acute pain and severe swelling in the upper lip during a previous root canal treatment of the above-mentioned tooth that was carried out on January 2008. During this procedure, the NaOCl solution used for root canal irrigation was extruded into the adjacent tissues, probably due to a root resorption of the apical area, an apical foramen of great diameter or to an unintentionally injection into the foramen (solution concentration, gauge and length of irrigation needle remain unknown). Immediately, the dentist performed an abundant irrigation with sterile saline solution, administered a single intramuscular dose of sodium diclofenac (Voltaren® 75 mg., Novartis, Barcelona, Spain), and prescribed amoxicillin and clavulanic acid 875/125 mg (Augmentine® 875/125 mg, GlaxoSmithkline, Madrid, Spain), 1 tablet every 8 hours for 7 days. During the following days, the pain decreased although swelling persisted for 2 weeks (Fig. ).
Four weeks following the accident, root canal instrumentation was completed, irrigation with sterile saline solution was performed and the canal was sealed by means of a lateral condensation technique using AH Plus™ root canal sealer (Dentsply DeTrey, Konstanz, Germany).
One year after the accident, the patient reported persistent pain in the apical area of the affected incisor that increased with pressure (Fig. ). Periapical surgery was proposed to solve this complication but the patient denied any additional treatments and wanted to extract the tooth. Thus, dental extraction was performed followed by a histological exam of the associated lesion.
During the dental extraction, an apical root resorption, as well as, a buccal bone plate defect was observed. Following the elimination of the periapical lesion, the dentist removed the debris of the root canal sealing material. The anatomopathological study revealed fibrous tissue, fibrohematic material and unidentified birefringent material with no foreign body granulomatous reaction.
The patient complained of a persistent paresthesia located in the left nasogenian area with lateral rhinorrhoea which still persisted at the time of her last appointment, 2 years after the initial treatment and 9 months after the extraction.


A 53 year-old female was referred to the Emergency Department of the Hospital Universitario de Bellvitge (Barcelona, Spain) with a complaint of abrupt swelling of the left cheek following root canal therapy in the left upper first premolar. This treatment was carried out on August 2008 and was not completed due to severe pain reported during the procedure. The clinical examination showed an important swelling that comprised the area between the periorbital region and the mandibular angle, with hematoma formation in the infraorbital region. The oral mucosa didn’t show any type of necrosis (Fig. ). Analgesics, corticoids and intravenous antibiotics were initially administered (dosage and administration route remain unknown).
Twenty-four hours after admission, the patient was discharged and showed some signs of recovery. Treatment consisted of a combination of amoxicillin and clavulanic acid 875/125 mg (Augmentine® 875/125 mg, GlaxoS-mithkline, Madrid, Spain) 1 tablet every 8 hours for 10 days; methylprednisolone (Urbason® 16 mg tablets, Sanofi Aventis, Barcelona, Spain) over a 6 day-period using a decreasing dosage; paracetamol (Xumadol® 1g soluble formulation, Kern Pharma, Barcelona, Spain) 1 tablet every 8 hours; omeprazole (20 mg tablets, Cinfa E.F.G, Navarra, Spain) 1 tablet every 24 hours, and local application of ice packs.
Ten days after the complication, the patient attended the Oral Surgery department at the Teknon Medical Center with persist edema and ecchymosis of the left cervical region, as well as, swelling in the second quadrant. A panoramic radiography was made to complete the examination.
After 2 weeks, the patient showed an important decreased of the ecchymosis and the radiographies failed to reveal any bone injuries.
Due to the improvement of the patient’s clinical situation, root canal treatment of the affected premolar was performed with working lengths of 18 mm for the vestibular canal and 17 mm for the palatal canal, both with an apical size of 50. In this case, irrigation was performed with a 2% chlorhexidine and EDTA solutions. Subsequently the canal was sealed following a lateral condensation technique using AH Plus™ root canal sealer. The final restoration was achieved using a metal-ceramic crown (Fig. ).
Six months later a new panoramic radiography was taken and no significant alterations were identified. Furthermore, the clinical examination was normal and the patient did not show any symptoms.
Follow-up appointments (last one took place on July 2009) revealed no evidence of pathology in the upper left first premolar.


A 17-year-old female with a 2-year history of erosive nodular seropositive RA followed Chinese herbal treatment for symptoms of repeated arthralgia in 2011. One year later, the patient developed jaundice, hepatosplenomegaly and enlarged lymph nodes. A physical examination revealed deformity in the proximal interphalangeal joints, metacarpophalangeal joints, wrists and ankles. Lymph nodes at the posterior neck, axillary fossa and inguinal area were enlarged with a diameter of 0.5–1 cm.
In August 2012, the patient presented with a fever >39°C. Blood tests revealed a white blood cell count of 4.12×109/l with an absolute neutrophil count of 3.15×109/l, hemoglobin levels of 80 g/l and a platelet count of 159×109/l. A bone marrow biopsy showed normocellular and maturing trilineage hematopoiesis. Glutamic oxaloacetic transaminase levels were 352 U/l, while glutamate pyruvate transaminase levels were 136 U/l. Bilirubin levels were 112.8 μmol/l and lactate dehydrogenase levels were 548 U/l. A rapid erythrocyte sedimentation rate (35 mm/h) and high C-reactive protein levels (60.2 mg/l) were revealed. Cultures of blood and secreta showed no infection by microorganisms. Tests for human immuodeficiency virus, syphilis, hepatitis B, hepatitis C and autoimmune hepatitis-associated antibodies, and a purified protein derivative skin test produced negative findings. The antinuclear antibody titer was 1:320, while results for anti-dsDNA, anti-Sm and ant-Rnp antibodies were negative. Levels of complement C3 and C4 were normal. Rheumatoid factor, antibodies to cyclic citrullinated peptides, anti-keratin antibodies and anti-perinuclear factor autoantibodies were negative. X-rays of the joints, including the wrists and hands, showed soft-tissue swelling, bone erosion and narrowing of the joint cavity (). Splenomegaly (54×124 mm) with uniform density and multiple enlarged lymph nodes distributed along the retroperitoneal space, omental bursa, mesentery root and surrounding hepatic hilar was shown by abdominal computed tomography (CT) (). A lymph node biopsy from the right cervical node revealed chronic inflammation, with positive results for cluster of differentiation (CD)79α and CD20 in interfollicular regions, and for CD5 in the paracortical area (). Fluorodeoxyglucose (FDG)-positron emission tomography revealed lymphadenectasis of the bilateral submandibular and superficial anterior cervical lymph node, axillary fossa, retroperitoneal space, pelvic wall and inguinal area without FDG uptake. The proliferation of hypermetabolic lesions was observed in the shoulder, hip, knee, ankle and interphalangeal joints, as well as knee joint-effusion, which was the manifestation of RA. FDG uptake in the liver and spleen occurred with a maximal standardized uptake value of 1.4 and 2.0, respectively. The pathological evaluation of liver tissue showed inflammatory infiltration containing swelling and spotty or fragmented necrosis in the hepatocytes (). Based on these findings, the provisional diagnosis was of FS.
Treatment was initiated with methotrexate (7.5 mg/week), hydroxychloroquine (400 mg/day) and methylprednisolone (16 mg/day). After tolerating overall treatment, the patient did no longer had a fever. The levels of liver-associated enzymes and bilirubin, and the erythrocyte sedimentation rate were decreased. This decrease is usually associated with liver and spleen shrinkage. Long-term follow-up is currently being performed.


A 67-year old Afghani woman was referred after an incidental finding of right hilar and paratracheal lymphadenopathy during investigations for left-sided chest pain. She reported breathlessness on climbing stairs. Past medical history included type 2 diabetes mellitus, hypertension and TB fully treated in Afghanistan 35 years previously. She was a lifelong non-smoker. Examination was unremarkable.
A T-Spot test was positive, consistent with her previous TB, but TBNA samples were auramine, culture and polymerase chain reaction (PCR) negative for TB. A computed tomography (CT) scan performed during inpatient investigations identified a left rib fracture in addition to incidental right-sided hilar and paratracheal lymphadenopathy. An FDG-PET scan demonstrated increased metabolic activity in the right paratracheal node with a maximum standardised uptake value (SUV) of 8.4 (normal values <2.7) (). On EBUS-TBNA of subcarinal, paratracheal and right hilar mediastinal lymph nodes, black pigment was obtained macroscopically (). On microscopic examination the aspirate was abundantly cellular with a population of anthracotic macrophages that were both singly dispersed and in variously sized aggregates (). There was a single foreign body type, multinucleated giant cell adjacent to one aggregate of anthracotic macrophages. No necrosis or malignant cells were seen. She retrospectively reported cooking over open fires.
A follow-up CT scan at 6-months showed completely stable appearances, with some pulmonary nodules, likely intrapulmonary lymph nodes, and part calcified mediastinal lymphadenopathy. Clinically she remained well having gained 2 kg in weight, with no night sweats, fevers, cough or breathlessness. She is due further follow-up at 18 months.


A 74-year-old woman with a 20-year history of non-productive cough underwent a CT scan as part of evaluation of a left lower lobe calcified nodule. She was otherwise healthy and examination was unremarkable. The CT scan identified curvilinear shadowing in the right middle lobe and a second non-calcified nodule inferiorly in the left lower lobe. Right middle lobe bronchial washings were culture negative for bacterial or fungal infection and there were no malignant cells. Auramine stain and TB cultures were negative.
An FDG-PET scan demonstrated normal metabolic activity in the nodules but identified increased activity in enlarged right paratracheal, para-oesophageal and bilateral hilar lymph nodes (SUV 5-7) and an incidental right adrenal adenoma. EBUS-TBNA of the right paratracheal lymph node macroscopically showed lightly bloodstained material that contained small black flecks up to two millimetres in diameter. Microscopically there were striking amounts of anthracotic macrophages, arranged in aggregates and as singly dispersed cells. No multinucleated giant cells, necrosis or malignant cells were seen and was auramine and culture negative for TB.
A follow-up CT scan at 12 months showed no significant change in the appearance of the lung with no new nodules or significant change in the existing nodules. She was asymptomatic and due further follow-up at 18 months.


A 68-year old Pakistani woman presented with persistent cough and wheeze. Past medical history included atrial fibrillation, hypertension, hypothyroidism, asthma and IgA nephropathy. Examination was unremarkable. CT chest demonstrated mediastinal and hilar lymphadenopathy, and a small, non-specific left-sided pulmonary nodule.
An EBUS-TBNA was performed on the mediastinal and hilar lymph nodes. No black pigment was seen macroscopically. Microscopically large numbers of anthracotic macrophages were seen, singly distributed and in dense clusters. There was no multinucleated giant cell reaction, necrosis or malignant cells and was culture, smear and PCR negative for TB.
A follow-up CT scan at 9 months revealed unchanged appearances of the pulmonary nodule and mediastinal lymphadenopathy. She remained clinically well and is due a further follow-up CT imaging at 24 months.


A 65-year old Punjabi woman with a 3-year history of cough was referred for investigation. She denied any associated symptoms. Ten months prior to presentation she had been treated for presumed TB, on the basis of enlarged mediastinal lymph nodes on CT imaging and a strongly positive mantoux test. She was fully compliant with anti-tuberculous medication. She had hypertension and had been treated for cervical carcinoma aged 28. She moved to the UK in her teens and had never smoked. Examination was unremarkable.
A CT scan performed 6 months after completion of TB treatment revealed persistence of right paratracheal, subcarinal, right hilar and aortopulmonary lymhadenopathy and partial atelectasis of the postero-lateral segment of the right middle lobe. EBUS-TBNA was performed on the right paratracheal and subcarinal lymph nodes. This was highly cellular black pigmented material microscopically with anthracotic macrophages in collections and as single dispersed cells. No multinucleated giant cells, necrosis or malignant cells were seen. Culture, smear and PCR were negative for TB.
At follow-up she was well with resolution of her cough. She is due follow-up lung function testing at 12 months.


A 73-year old Afghani woman presented with 3 weeks of productive cough and an 8-month history of hoarse voice and coughing whilst eating. She denied weight loss, fever or night sweats. She was a lifelong non-smoker and had a past medical history of hypertension and type 2 diabetes mellitus. Her father had TB aged 35. Examination identified a firm, enlarged thyroid, a fixed monophonic wheeze in left mid zone and a left vocal cord paralysis. She was biochemically euthyroid.
A CT scan identified mediastinal and left hilar lymphadenopathy, abnormal soft tissue surrounding the left main bronchus, multiple bilateral calcified pulmonary nodules and a multi-nodular goitre. PET/CT scan demonstrated activity in the mediastinal lymph nodes with SUV of 8.4.
Bronchoscopy identified an endobronchial soft tissue mass but endobronchial biopsies and washings failed to identify malignant cells or granuloma, demonstrating only inflammation and squamous metaplasia. Bronchial washings were auramine, culture and PCR negative for TB. EBUS-TBNA of the mediastinal mass showed black material macroscopically. On microscopy there were abundant anthracotic macrophages which were distributed singly and in aggregates. No multinucleated giant cells, necrosis or malignant cells were seen. On further questioning she admitted cooking on wood fires in Afghanistan, and remembered inhaling dust and sand during dust storms.
The patient declined further investigation with repeated EBUS or video assisted thoracoscopy surgery (VATS), preferring a period of symptomatic and radiological observation.
A follow-up CT scan showed no change in the size of mediastinal nodes at 10 months. Despite continuing to suffer a left vocal cord palsy secondary to aortopulmonary lymphadenopathy, she remains well at 18 months with no other aetiology found.


A 34-year-old man presented to his family practitioner with a two-month history of swelling and discomfort in the left lateral lower leg just above his ankle. There was no preceding history of trauma. Physical examination revealed soft tissue fullness at the junction of the proximal two-thirds and distal one-third of the left fibula which was painful to touch. The patient was sent for an ultrasound for evaluation and possibly to aspirate a presumed ganglion cyst.
Ultrasound was performed using a General Electric Healthcare Logiq E9 linear ML 6–15 MHz transducer (GE Healthcare Wauwatosa, WI). A cortically based lesion was noted arising from the anterolateral cortex of the fibula with elevation of the periosteum and a thin rim of echogenicity surrounding the mass, presumed to be a thin shell of bone () which appeared intact without adjacent soft tissue mass. Two fluid-fluid levels were noted within the mass which were mobile on patient rotation indicating the cystic nature of the lesion's contents. No internal soft tissue mass extending from the fibular medullary canal was noted. There was increased vascularity in the adjacent soft tissues on color Doppler evaluation consistent with inflammatory changes. The most likely diagnosis was a cortically based aneurysmal bone cyst (ABC) and not a soft tissue solid or cystic mass. In view of the periosteal elevation or sonographic “Codman's triangle,” the mass was thought to be centered and to have originated within the bone rather than to be a soft tissue mass such as a ganglion cyst which had eroded into the bone. A Brodie abscess or subacute osteomyelitis could have this appearance as symptoms can be indolent, but these tend to be metaphyseal and centrally located. The adjacent fibular cortex was normal with no adjacent soft tissue mass to suggest an underlying aggressive bone lesion such as osteosarcoma or metastasis with secondary ABC formation.
MRI was obtained for preoperative planning, consisting of axial and sagittal T1-weighted and fat saturated T2-weighted images with axial and sagittal fat saturated spoiled gradient echo sequence after gadolinium administration. This confirmed a cortically based mass arising from the fibula extending into the anterior compartment, with fluid-fluid levels seen on the T2-weighted sequence (). There was peripheral enhancement and increased T2 signal in the adjacent soft tissues and fibular marrow consistent with inflammatory changes corresponding to the increased vascularity seen sonographically. No primary lesion could be seen and this was presumed to be a primary cortical ABC.
Radiograph demonstrated a cortically based lesion with a narrow zone of transition, elevating the periosteum with a thin shell of bone (). No other lesions were seen in the fibula or tibia. The patient underwent surgical excision of the lesion with curettage. A histologic diagnosis of aneurysmal bone cyst was made forming a 3.2 × 1.7 × 0.9 cm cystic mass (). Microscopically, a cystic space with peripheral shell of reactive bone with numerous osteoclast giant cells and scattered histiocytes and lymphocytes was noted. Some of the histiocytes contain hemosiderin.


A 77-year-old male presented with weight loss, episodic altered sensorium, and mass in abdomen for 2 months. Computer tomographic (CT) scan of the abdomen revealed a 16×16 cm retroperitoneal mass extending to the kidney and lateral chest wall (). During hospitalization, he lost consciousness with blood glucose of 28 mg/dl. Ten years before, he had undergone thoracotomy and excision of a posterior mediastinal mass, which weighed 2 kg and was then reported as fibroma. During that presentation, he had no history or documented episode of hypoglycemia. On examination, he had multiple skin tags and acromegaloid features. On extended fasting, he developed hypoglycemic symptoms with blood glucose of 37 mg/dl at 3 h, plasma insulin 1.5 μU/ml (normal:<6 μU/ml), growth hormone (GH) 0.54 ng/ml, IGF1 48 ng/ml (normal: 94–190 ng/ml), and IGF2 1133 ng/ml (normal: 440–1249 ng/ml). Fine-needle aspiration cytology (FNAC) was suggestive of a fibroma. He was started on complex carbohydrate diet, prednisolone 20 mg/day with which his symptoms abated and was subjected to surgical debulking. Histopathology revealed some areas of atypia suggestive of sarcomatous change. In view of aggressiveness of the tumor and residual disease, he received local radiotherapy (20 Gy) in fractions and six cycles of combination chemotherapy (vincristine+cyclophosphamide+doxorubicin). Repeat CT scan revealed residual disease. On follow-up, he was on prednisolone 7.5 mg/day with no recurrence of symptoms for the next 6 years and later lost to follow-up.


A 24-year-old female presented with 6-month history of hunger, sweating, and tremors, which used to subside immediately after food intake. She had documented blood glucose values below 40 mg/dl during those episodes. On evaluation, she had a palpable mass in the abdomen and a CT scan of the abdomen revealed a well-defined lobulated, heterogeneous, hypervascular mass in the left upper abdomen measuring 15×8×8 cm with the epicenter in the retroperitoneum. The mass was abutting left adrenal gland, spleen, and celiac axis. The FNAC of the mass was suggestive of sarcoma. She received three cycles of chemotherapy and was referred to our institute. At blood glucose of 25 mg/dl, she had undetectable plasma insulin (≤0.2 μU/ml), low C-peptide 0.084 ng/ml (normal: 1.1–4.4 ng/ml), normal cortisol level (615 nmol/l; normal:>550 nmol/l during stress), and a GH level of 0.2 ng/ml. She had low IGF1 <25 ng/ml (normal: 116–358 ng/ml); with IGF2 level of 541 ng/ml (normal: 288–736 ng/ml) and high IGF2:IGF1 ratio of 21.64 (normal:<10). She underwent laparotomy and en bloc resection of the retroperitoneal mass with splenectomy, distal pancreatectomy, and left nephrectomy. Histopathology was suggestive of hemangiopericytoma (). After surgery, there was no recurrence of hypoglycemia. She is doing well for the last 3 years and follow-up 18FDG–PET scan is normal.


A 32-year-old female presented with recurrent episodes of neuroglycopenic symptoms for 2 weeks especially during the fasting state. On examination, she had coarse facial features, acral enlargement, seborrhea, and acanthosis nigricans. At 9 h of extended fast, her blood glucose was 38 mg/dl with undetectable insulin (≤0.2 μU/ml) and low C-peptide level (0.08 ng/ml; normal: 1.1–4.4 ng/ml), normal cortisol level (561 nmol/l; normal:>550 nmol/l during stress), and a GH level of 4.2 ng/ml. She had low IGF1 56.9 ng/ml (normal: 115–307 ng/ml), with IGF2 level of 489 ng/ml (normal: 288–736 ng/ml) and normal IGF2:IGF1 ratio of 8.6 (normal:<10). Serum GH was suppressible (<1 ng/ml) after glucose load. Contrast enhanced CT scan of the abdomen revealed a large lobulated, heterogenous, hypervascular right suprarenal mass of size 15.3×12.7×12 cm with necrosis and inferior vena cava compression without any local invasion. Her cortisol dynamics and urinary metanephrines were normal. She underwent open right adrenalectomy, following which hypoglycemic episodes ameliorated and her acromegaloid facial features regressed. Histopathology revealed adrenocortical carcinoma (). Follow-up contrast enhanced CT (CECT) scan of the chest and abdomen revealed lung metastasis. Patient is not able to afford mitotane-based chemotherapy; however, she is asymptomatic and under regular follow-up.


An 80-year-old male chronic smoker, hypertensive, and non-diabetic with no exposure to hypoglycemic agents presented with cough, breathlessness, recurrent episodes of neuroglycopenia, and seizures for 6 weeks. He had documented low blood glucose of 33 mg/dl during those episodes. He had digital clubbing with decreased air entry in left infrascapular region. During hospitalization, he developed neuroglycopenia with blood glucose of 33 mg/dl, plasma insulin <0.200 μU/ml, C-peptide 0.687 ng/ml (normal: 1.1–4.4 ng/ml), cortisol 446 nmol/l (normal:>550 nmol/l at the time of stress), and GH of 1.2 ng/ml. He had low IGF1 <25 ng/ml (normal: 55–166 ng/ml), with IGF2 level of 860 ng/ml (normal: 288–736 ng/ml) and IGF2:IGF1 ratio of 34.4 (normal:<10). CT of abdomen showed 21×20×12.5 cm mass in the left lower lung field with necrosis and calcification (). Ultrasound-guided FNAC was suggestive of pleural mesothelioma. Patient declined surgery and chemotherapy and was started on prednisolone 20 mg/day with which his hypoglycemic episodes subsided. However, he was lost to follow-up.


A 48-year-old male was admitted with history of recurrent abdominal pain one and a half years. Ultrasonography of the abdomen revealed a large retroperitoneal mass. He was admitted for surgery. On the day of surgery, he developed delirium following overnight fasting and surgery was canceled. Patient was started on i.v. glucose following which he improved. On the day of next surgery, patient developed similar features with right-sided hemiparesis. During this episode, blood glucose of 40 mg/dl was documented and endocrinology consultation was sought. On further evaluation, after 8 h of extended fast, he had blood glucose of 31 mg/dl with corresponding plasma insulin of <0.2 μU/ml (normal:<6 μU/ml), GH <0.05 ng/ml, IGF1 56 ng/ml (normal: 94–252 ng/ml), IGF2 623 ng/ml (normal: 288–736 ng/ml), and IGF2:IGF1 ratio of 11.1 (normal:<10). A diagnosis of hypoinsulinemic hypoglycemia was made. CECT scan of the abdomen revealed 15.2×9.2×9.5 cm homogenously enhancing retroperitoneal mass arising from the pelvis extending superiorly up to the umbilicus (). He underwent surgical removal of the tumor. Histopathology was suggestive of leiomyosarcoma. At 6 months, repeat imaging did not reveal any residual or recurrent disease. He is asymptomatic and under regular follow-up.


32-year-old, female.
Cough and sputum for 1 year.
The patient complained of intermittent cough and sputum for 1 year, and the patient also had dyspnea while walking upstairs, needing rest midway. For the symptoms, the patient had visited a local otolaryngologist occasionally and had received symptomatic treatments only. She visited our emergency room due to the sudden development of epistaxis. At the time, her systolic blood pressure was measured to be 190 mmHg, but no other specific findings were noted. She was discharged after epistaxis treatment with the recommendation to visit the outpatient clinic. Her blood pressure was again high at 170/120 mmHg, and admission to the department of cardiology was proposed to the patient. After admission, the blood pressures measured on both upper and lower extremities differed by more than 30 mmHg, and chest computed tomography (CT) was taken. On the CT, both lungs showed multiple centrilobular nodules. The patient was again referred to the pulmonary division for additional examinations. Bronchoalveolar lavage (BAL) and transbronchial lung biopsy were performed. Histologically, multinucleated giant cells were observed, coinciding with the findings of HP, and the pathology suggested dental technician’s pneumoconiosis. Thus, the pulmonologist referred the patient to the Department of Occupational and Environmental Medicine to evaluate any specific relationship of the disease with her occupation.
The patient had suffered from cough, sputum, and dyspnea when walking upstairs for 1 year. The patient had not been very concerned about her symptoms at the time, and she only looked for medical treatment when these symptoms had been aggravated. She visited a local otolaryngologist intermittently and was told that her vocal cords showed inflammatory changes and nodules. After taking oral medications, her symptoms improved. She had experienced several episodes of repeated aggravation and relapse of the symptoms. Her symptoms started to aggravate 10 days before admission to our emergency department, With the exception of a 130 mmHg systolic blood pressure measured during a routine health examination 2 years earlier, her past history was insignificant. She did not consume any alcohol or tobacco. Her height was 156 cm. She weighed 70 kg, and recalled no recent loss of weight. She was residing in a apartment building, and questioning about her residence, hobbies, and other activities revealed that she was not exposed to any risk of respiration of dust.
The blood pressure, pulse, respiratory rate, and temperature checked at the admission were 160/100 mmHg, 76 beats/minute, 19 respirations/minute, and 36.9°C, respectively. She had a consistent cough and intermittent expectoration of white-yellowish sputum. Other findings were unremarkable.
Her blood tests including a complete blood count, electrolyte, and endocrine serum tests, and urinalysis were within normal range. A simple chest radiograph had unremarkable findings, but chest CT revealed ground glass patterns in both lungs (Figure C). Small nodules with emphysematous changes around the mediastinum were observed in the lower lobe of left lung, and the lymph nodes in the hilar and mediastinal areas were enlarged in both lung fields (Figure B, D). The BAL results showed increased white blood cell counts to 2,080 per mm3. The fraction of lymphocytes was found to be high, at 80%. The CD4+/CD8+ ratio of lymphocytes was 7.82, signifying an increased number of CD4+ lymphocytes. Tissue samples were acquired through transbronchial biopsy, and histologic examination of the samples, showed microcalcification, noncaseating granuloma with foreign body-type giant cells, and macrophages containing metal particles. When the metal particles were observed under a polarizing microscope, the particles were positive for birefringence (Figure B, C). In addition, angiotensin-converting enzyme and rheumatoid factor tests were evaluated in order to differentiate from other interstitial lung diseases such as rheumatoid nodules and sarcoidosis that have similar manifestations of mediastinal lymph node enlargement, and the tests were within normal range.
After admission, 10 mg of amlodipine besylate was given to the patient daily. Her blood pressure was controlled under 130/90 mmHg, and her symptoms subsided with the exception of an intermittent cough. Her respiratory symptoms improved significantly 2 days after she was no longer exposed the work environment. Because the number of CD4 + T cells had increased noticeably, a T-cell-mediated immune response was suspected, and 0.5 mg/kg of prednisolone was prescribed. The dyspnea showed no significant improvement, but the cough and sputum improved somewhat. She returned to her job after discharge from the hospital, and her symptoms reappeared some time after she started working. Medications were continued to reduce her symptoms. In a chest CT taken at 2 months after admission, the lung lesions observed in the previous CT had nearly disappeared (Figure D, E). She quit her job for 3 months, and the use of prednisolone was tapered and eventually discontinued at 5 months after initiation of the therapy. Without further exposure, the respiratory symptoms including dyspnea on exertion virtually vanished.
The patient had worked as a dental technician since 2002. At the current work place, she had worked for 8 months, and she worked from 9 AM to 6 PM 6 days a week. As she recalled, she had witnessed that her colleagues had also suffered from intermittent cough and sputum. The company she worked for had recently been established, about a year earlier, and had 3 workers. The patient mainly worked in the trimming and ceramic build-up processes.


A 55-year-old female.
Swelling in the body after exposure to ignition coal fumes.
She had operated a second-floor bar 5 years. In 2011, she experienced body swelling, sputum, and muscular symptoms beginning when the meat roasting restaurant on the first floor of the same building replaced charcoal with ignition coal a few weeks before presentation to the division of rheumatology. She was diagnosed with FMS and treated. Her symptoms were improved when she took her prescribed FMS medicine. Her symptoms recurred after exposure to the fumes of ignition coal when she returned to work. Within a few weeks, she moved into a new house which used city gas (previously, she had lived in a house using an oil boiler). Her symptoms, such as whole body swelling (both arms and both calves were especially edematous), sputum, myalgia, and arthralgia worsened, and she had the new onset of headache after exposure to the smell of city gas. She vomited at night when she moved to a new house. To remove the city gas fumes, she began to use an air purifier. If symptoms recurred, she took FMS medication, but the symptoms did not improve. Eventually, 1 year later, she moved into a different house. Gradually, she developed a sensitivity to the smell of burning and exhaust gas. After exposure to the fumes, symptoms such as swelling, myalgia, arthralgia, poor concentration, and nausea occurred. The symptoms consistently occurred when she inhaled the fumes of ignition coal, so she finally quit her job 1 year later from first induction of symptoms. She underwent a pulmonary function test because she had difficulty breathing, whole body swelling, and a sore throat, but the laboratory tests results were normal. In 2013, a multiple allergen simultaneous test (MAST) was performed; the result was non-specific except for a weak positive outcome for house dust mites. There was no improvement in the symptoms, so she was treated for rheumatism at the hospital. Even though the treatment was conducted, her symptoms continued to be aggravated by city gas fumes. Hence, she plans to move to a new house where city gas is not used.
Atopy, hormonal treatment of menopause symptoms (5 years).
There is no family history of skin disease. Her daughter did not complain of symptoms similar to her when her daughter came home to visit.
No smoking. No drinking.
She operated a bar on the second floor above a restaurant. She quit her job 1 year later from first induction of symptoms.
In the past, she jogged almost every day for two hours. But during the last two years, she has usually exercised by walking.
The bar employees reported being aware of smelling ignition coal, but none experienced symptoms such as swelling, sputum, and myalgia like she did.
Results of one set of hospital tests: A clinical examination including a complete blood count (CBC), liver-renal function test (LRFT), electrolyte tests, rheumatoid factor (RF), uric acid, hepatitis B test, antinuclear antibody (ANA test), anti-cyclic citrullinated peptide antibody (anti-CCP antibody), thyroid-stimulating hormone (TSH), and urine analysis (UA) were performed, 2011. No specific findings were revealed except mild elevation of aspartate aminotransferase (AST) and alkaline phosphatase (ALP) and cholesterol. After that, 10 months later, she underwent a re-examination for CBC, LRFT, uric acid, and electrolytes but the results were within normal range again. Three more months later, pulmonary function tests and simple chest radiography and chest computed tomography did not show specific findings. The test results for MAST were low positive (1+) for total immunoglobulin E and house dust mites (mite-farinae, mite-pterony), and very low positive for Acacia, Timothy grass, dog, and cockroach mix.
Symptom changes before/after exposure to the city gas fumes: The most sensitive triggering substance was city gas. After becoming aware of the smell of city gas, she developed symptoms including dyspnea, chest discomfort, sputum, body swelling, myalgia, arthralgia, and headache within 5 minutes. When she was exposed to the smell of city gas, her dyspnea worsened. Therefore, she had to avoid the place of exposure. When she moved to a different location, her symptoms gradually disappeared within 2–3 hours.
First visit to our hospital: The other hospital examinations showed no abnormal findings from blood tests, urine tests, pulmonary function tests, and lung imaging tests. After medical examination by interview, a physical examination was performed according to her symptoms. We considered MCS, FMS, CFS, and somatization disorder, based on the diagnostic process and differential diagnosis proposed by Chae et al [].
Physical examination: The patient reported that her body weight was highly changeable after exposure to certain smells. Therefore, we measured her height and weight (148.1 cm, 53 kg). We examined the degree of swelling, but there were no abnormal findings. She also complained of joint pain including that of both metacarpal phalangeal joints (MCP joints), the right metatarsal joint of the thumb, and both the Achilles tendons, but there was no swelling, tenderness, or joint deformity. Although she complained of myalgia (including that of the right arm, left forearm, and both calves), there was no swelling or tenderness.
The symptom graph differed before and after exposure to ignition coal (Figure ). The symptom severity score was 25 points before exposure to ignition coal and it was 56 points after exposure to ignition coal. Before exposure to ignition coal fumes, the score of “skin related symptoms” and “genitourinary related symptoms” was high because of her medical problems with atopic dermatitis and menopause-related symptoms. After exposure to ignition coal fumes, her existing symptoms worsened and new symptoms occurred (including airway or mucous membrane symptoms, musculoskeletal symptoms, affective symptoms, and head-related symptoms). On the Korean Version of the QEESI, all of the area scores were high and very suggestive of MCS.
The symptom severity score was 56 points, chemical intolerance score was 53 points, other intolerance score was 60 points, life impact score was 77 points, and masking index score was 7 points.
Second visit to our hospital, differential diagnosis with FMS: We used the 2010 American College of Rheumatology (ACR) preliminary diagnostic criteria for FMS. We scored each situation, to compare before and after the exposure to the smell. Before exposure to ignition coal, the widespread pain index (WPI) score was 1 point and the symptom severity scale (SSS) score was 8 points. This results did not meet the criteria for a diagnosis of FMS. After exposure to ignition coal, the WPI score was 7 points and the SSS score was 9 points. These met the criteria for the diagnosis of FMS. However, she did not show common FMS clinical features. She had no symptoms of pain worsening with exercise, sleep disturbance (characterized by non-restorative sleep and increased awakenings), or cognitive difficulties including short-term memory problems. After exposure to ignition coal fumes, the diagnostic criteria for FMS was fulfilled because the symptoms are similar to MCS. Hence we excluded FMS.
We used the 1994 Centers for Disease Control and Prevention (CDC) diagnostic criteria for CFS. Before exposure to ignition coal fumes, the patient had no symptoms among the diagnostic criteria. However, after exposure to ignition coal, she showed six symptoms among the diagnostic criteria (poor mental function, sore throat, myalgia, multiple arthralgia, new onset headaches, and feeling unwell). However, when she was exposed to fresh air or avoided exposure to triggering substances, all of her symptoms disappeared. Because CFS symptoms are not improved by avoiding the triggers, we excluded CFS.
We used the Diagnostic and Statistical Manual of Mental Disorders IV (DSM-IV) in considering somatization disorder. She had social impairment with 4 or more pain symptoms and 2 or more gastrointestinal symptoms, and her symptoms did not start before the age of 30. She also had no sexual symptoms or pseudoneurological symptoms. Therefore, we excluded somatization disorder.
Her weight was 53.4 kg. She complained of more edema than before. Pitting edema was observed in both forearm regions (1+) and both anterior tibial regions (2+).
Pitting edema is an abnormal accumulation of fluid in the interstitium characterized by indentations persisting after the release of pressure []. Generally, pitting edema occurs because homeostasis of the interstitial fluid has broken. It can be caused by heart failure, liver failure, renal failure, pregnancy, or electrolyte imbalance. She has not observed any organ dysfunction. She was in menopause and her electrolytes were within normal range. No other diseases were suspected.
The patient had multi-organ symptoms when exposed to a triggering chemical that is normally considered to be safe. Her symptoms also occurred repeatedly with chemical exposure, and improved with removal of the chemical exposure. Her symptoms were chronic, and she was sensitive to many chemicals. Considering the diagnostic criteria for MCS agreed upon in Atlanta in 1999 and her results from the Korean version of the QEESI, our case fits the criteria well and the diagnosis is obvious. The patient was educated about MCS and avoiding exposure to triggering substances. She showed changing social behavior in that she moved house twice, reduced her hobbies, and quit her job. Therefore, we aimed to help her return to work and live in her new house by avoiding triggering substances in the environment. She was educated about air purifiers and air purification plants. We also explained long-term care and the necessity of moral support.


A 49-year-old man was admitted with two months persistent constipation, incoercible with medical therapy. He did not report any urinary symptom, only a mild erectile dysfunction. On digital rectal examination his prostate was regular in size and consistency but with an extrinsic compression on the right-posterior wall by a mass. Transabdominal ultrasonography showed a localized cystic mass of about 7,3 × 5 cm, probably of the right seminal vesicle, characterized by multiple endoluminal septa. Transrectal US confirmed the origin of the lesion from the right seminal vesicle without infiltration of rectum, bladder, or prostate ().
Routine blood exams, including PSA and prostatic acid phosphatase, were normal.
Magnetic resonance (MR) was performed and reported a 56 × 63 × 59 mm lesion of the right seminal vesicle with a fluid content and endoluminal central solid tissue caracterized by a sepimentated structure with enhancement after Gadolinium-DTPA. On T1 weighted images, some hyperintensities in small cavities, caused probably by past hemorrhages, were observed. Prostate and bladder were normal, without signs of infiltration. No local adenopathies were found ().
Colonoscopy confirmed a delayed fecal transit through the rectum and cystoscopy showed compression on the vesical neck without any alteration of the mucosa.
Computed tomography (CT) staging was performed without any abnormalities of thoracic and abdominal organs; lesion of the right seminal vesicle was confirmed ().
US guided needle biopsy was performed and it was negative for atypical cells.
The patient underwent right seminal vesiculectomy, with section of ipsilateral vas deferens and the saving of prostate, bladder, and rectum.
On gross examination the specimen consisted of a distorted and dilated seminal vesicle of 5 × 5 × 5 cm, with an intact and smooth external surface. On serial sectioning, in particular of the central mass of hard consistence, the lesion was characterized of necrotic-haemorragic areas with pseudocysts. Histologic examination demonstrated a biphasic tumor: one part with ischaemic necrosis, the other without necrosis characterized by an epithelial matrix mainly cystic delimited by addensed stroma (CD34+, vimentin +, actin ML +, estrogenic and progestinic receptors +) with atypic cells and a variable grade of mitosis. The proliferation index Ki 67 MIB1 clone was inhomogeneous with foci of high proliferation, in particular near the necrotic areas and associated with squamous metaplasia. The final histological diagnosis was cystosarcoma phyllodes of the right seminal vesicle.
Patient's recovery was uncomplicated, so after one week he was discharged.
Clinicians decided jointly for a strict clinical and CT follow up. Actually, after 14 months, the patient is still alive and makes his periodic clinical-strumental-laboratoristic controls without evidence of disease on chest and abdominal-pelvic CT.


A 29-year-old multiparous caucasian woman with an unplanned pregnancy has been exposed to varenicline 1 mg twice a day unintentionally 4 weeks from her last menstrual period (LMP). Limited rodent data regarding varenicline did not permit a definitive risk assessment; however, after the risk communication with the patient, she decided to continue pregnancy with perinatology follow-up. She received folic acid supplementation between weeks 4 and 12. Fetal ultrasound (USG) performed at each trimester showed normal fetal growth with no malformations. A detailed anomaly scan and fetal echocardiography were performed at the 22nd gestational week and revealed normal development of fetus. The patient delivered a healthy baby girl via cesarean section at the 38th week of gestation. First- and fifth-minute Apgar scores were 9 and 10, respectively. The infant was the second child of nonconsanguineous parents. The baby's weight was 3130 g, length was 49 cm, and head circumference was 34 cm with normal percentiles and the baby did not show any physical examination abnormalities postnatally. Neurological examination had normal findings including posture and muscle tone, newborn reflexes, and deep tendon reflexes.
In the follow-up controls, at the ages of 2, 4, and 6 months we saw an age-appropriate developed child with a normal internal and neurological status. Social contact, fine motor, language, and gross motor skill categories measured with Denver II Developmental Screening Test adapted and standardized for Turkish children at the fifth and sixth month were compatible with her age.


A 28-year-old Chinese man with good past health and no significant family history presented to the Accident and Emergency Department (AED) due to sudden onset bilateral lower limb paralysis (Medical Research Council power grading 2/5) on August 13, 2013. The patient reported no recent head, neck, or spine injury and remained conscious all along. Physical exam revealed a muscular young man with no features of dehydration, hyperthyroidism, or Cushing's syndrome. Urgent imaging including plain computer tomography (CT) of brain and X-ray spine showed no abnormality. ECG showed typical u-waves and serum biochemistries came back to show severe hypokalemia (1.6 mmol/L, RR 3.5–5.0 mmol/L) (). His serum creatinine, magnesium, thyroid stimulating hormone, and pH levels were all normal. He was therefore admitted to the ICU and was commenced on oral and intravenous potassium replacement. Spot urine for potassium (14 mmol/L) was only saved after the start of potassium replacement when the paired serum potassium was 2.2 mmol/L. In total, 20 mmol of intravenous and 10 mmol of oral potassium chloride were given, and his serum potassium was stabilized at 3.7–4 mmol/L eighteen hours after admission. Urine for toxicology screen was saved and the report was pending at that time.
Upon further questioning, the patient admitted to have taken some weight loss agents of unknown nature which he had purchased over the Internet until the date of his competition. He then disclosed that, in preparation for the bodybuilding competition, he had been taking heavy loads of carbohydrate for muscle building and restricting salt and fluid intake a few days prior to the presentation. He denied any recent use of anabolic steroid. The patient recovered fully from his paralysis a couple of days later and discharged himself from the hospital against medical advice on August 15, 2013. His latest serum potassium prior to discharge was 4.4 mmol/L.
The urine toxicology screen came back two weeks after the patient's presentation showing rhein, emodin, aloe-emodin, and physcion. Unexpectedly, mycophenolate mofetil (MMF) and diltiazem were also identified although the patient denied having any prescribed medications. The patient was phoned up and the urine toxicology results were explained to him, with emphasis on the potential adverse effects of taking drugs without prescription from doctors.


A 13-year-old premenarchal girl was admitted to the emergency department with a complaint of worsening lower abdominal pain during the past 4 months. Secondary sexual characteristics had developed normally; she had Tanner stage IV breasts and normal pubic and axillary hair development compatible with her age.
The absence of a hymeneal opening without any bulging was observed on examination of the external genitalia. The abdomen was tender and distended. At admission, her laboratory investigations were within normal limits with a leucocyte count of 5,800/mm3 and C-reactive proteins were low. An abdominopelvic ultrasound scan revealed a distended uterocervical cavity suggestive of hematometrocolpos compressing the urinary bladder without any other pathology of the genitourinary system. For a thorough evaluation, the patient was taken to the operating theater to be examined under anesthesia. After rectal examination, the presence of imperforate hymen was confirmed and a tender soft mass in the upper part of the vagina was detected. The imperforate hymen was excised and a short vaginal blind pouch of 2 cm bearing vaginal rugae was visualized. As the incision did not result in drainage of the collection, presence of a thick TVS was suspected. The patient was scheduled for a magnetic resonance imaging (MRI) scan. While waiting for her appointment for MRI scan at the hospital, the patient had a worsening abdominal pain with vomiting and her leucocyte count was elevated from 5800/mm3 first to 13,000/mm3 and within 6 hours reached to 15,000/mm3. There was only 24 hours between trial of hymenectomy and MRI scan. MRI scan revealed an air-fluid level representing pyometrocolpos within a distended vagina, endometrial cavity, and endocervical canal. Posterior vaginal extraperitoneal leakage was detected and it was interpreted as the sign of a fistula (long arrow) between the vagina and the rectovaginal space (). The level of the obstruction was suspected to be on the middle third of the vagina and the rectum was compressed by the leakage filling the rectovaginal space posterolaterally (). Although laparoscopic approach was planned, malodorous pus expelled after the insertion of the Veress needle, it was decided to proceed to laparotomy. At laparotomy, pus with peritoneal microabscess formations was observed. The pus was sampled for culture and antibiogram and drained followed by intra-abdominal washing. Escherichia coli grew from the culture; both taken during the operation from the pus found in the abdominal cavity and the specimen taken from the drained pus.
The uterus and bilateral tubes were normal in size and shape for the age of the patient; they were pink and fresh looking. A close observation of the uterus and cervix revealed no possible perforation sites that would explain intraabdominal spillage of the pus. A general surgeon was invited to the operation to rule out a probable rectal fistula but neither a fistula nor a perforation site was observed. As the probable communication or the tract between the abdomen and vagina was not observed, it could not be resected or repaired. In order to provide the drainage of pyocolpos, a 1 cm transverse incision at the superior part of the lower uterine segment was performed and with the guide of a number 8 Hegar dilator that was inserted from the incision site and then passed through the cervix into the vagina blindly, the septum was localized (). Then the TVS was excised vaginally, with the guidance of the Hegar dilator. Almost 200 mL of dense, brownish, malodorous pus was drained with suction. After total excision of the TVS, the cervix became clearly visible at the upper part of the vagina. A Foley sound was inserted into the cavity for the drainage of the collection and the uterine incision was repaired. A silicon drain was placed at the pouch of Douglas before the closure of the abdominal wall. The operation completed uneventfully. During the postoperative follow-up, pus drainage continued both from the silicon drain and via the posterior vaginal fornix. The patient received appropriate antibiotics regimens according to the culture results. The patient was scheduled for a control MRI after cessation of the drainage of the pus from the drains. Repeated MRI confirmed complete resolution of the pelvic collection. The patient was discharged uneventfully. She had normal regular menstrual cycles without any pain and complaints during the 8 months that she was followed after the surgery. The patient used a vaginal dilator to prevent stenosis for two months after the operation and clinical evaluations at 4, 6, and 8 months postoperatively confirmed an adequate vaginal space without any stricture.


An 11-year-old right-handed boy was born to non consanguineous parents, with no family history. At birth, he was diagnosed with cervical myelomeningocele () and operated at the age of 7 months with no immediate postoperative complication. Five months later, his parents noticed that voluntary movements of one hand was unintentionally reproduced by the other hand. Developmental milestones were normal.
At the age of 6 years, he complained of difficulties in bimanual activities. He was referred to our “Movement Disorders and Botulinum Toxin” consultation for evaluation of movement disorders in hands. Neurological examination showed bilateral involuntary synkinetic imitative movements in one hand, occurring with all voluntary movements of the opposite hand, concluding to hand’s MM. Amplitude of the intended movement was higher than that of the mirroring hand. He had also a right trapezium muscle atrophy and pyramidal syndrome in all four limbs. Sensory examination was normal.
Cerebral and spinal MRI at the age of 7 years was normal. EMG and NCV revealed mild neurogene changes in trapezium, supraspinatus and infraspinatus nerves, bilaterally.
Diffusion tensor imaging (DTI) of the brainstem and the cervical spine was performed. No obvious cortico-spinal tract decussation in lower medulla oblongata was observed. Cervical DTI was normal (,).
Coronal (a,b) and sagittal (c) sections of the brainstem. No evidence of cortico-spinal tract decussation in lower medulla oblongata is noticed. Cervical DTI is normal (c)


A 6-month-old boy with hydrocephalus was referred to our hospital in 2008. The hydrocephalus during the first admission was confirmed by computed tomography (CT) scan. Examination of cerebrospinal fluid (CSF) did not reveal any abnormality.
A VP shunt was inserted in June 2008 and the infant was discharged in acceptable condition, with good control of hydrocephalus confirmed by another CT scan on discharge day. Approximately 16 months after insertion of the VP shunt, the child was readmitted with a one-week history of fever (up to 38.5°C), and acute abdominal pain. The diagnosis was brucellosis based on serum agglutination test 1/640 and 1/1280 three days later. A titre of 1/320 measured in 2 mercaptoethanol (2ME) test. The patient was treated with trimethoprimsulfamethoxazole, rifampin, and gentamicin. His blood culture was negative.
The Ascitic fluid examination showed a glucose level of 89 mg/dL, a protein level of 5800 mg/dL, white blood cell (WBC) count was 480 (10% polymorphonuclear cells and 90% mononuclear), and the red blood cell (RBC) count was 4800.
After 4 months, he was again admitted to our hospital with drowsiness, vomiting and fever. Clinical diagnosis was suggestive for a VP shunt infection. Laboratory results were as follows: Hemoglobin, 9.1 g/dL; WBC count, 6.1×103 cells/mm3.
A sample of ventricular CSF analyzed was slightly xanthochromic and contained a total of 4000 cells (70% polymorphonuclear cells and 30% lymphocytes), 80 mg/ dL glucose, and 192 mg/dL protein. No organisms were observed on the direct gram-stained smear.
Blood culture was sterile and CSF serological test was positive (Brucella agglutinin titer was 1/80).
The patient responded well to a course of rifampin, gentamicin and trimethoprim-sulfamethoxazole daily. Three days later the shunt was extracted. He was well on three follow-up visits at the outpatient clinic and the latest follow-up visit was 12 months after the diagnosis has been made. and show Brain CT scan before and after extracting the VP shunt, respectively.


A 65-year-old Caucasian man with a history of PAF was referred to our hospital for further evaluation of a mass located in the apex of his left ventricle. The mass was discovered incidentally after a transthoracic echocardiographic (TTE) examination during hospitalization for paroxysmal atrial fibrillation.
Our patient’s medical history was unremarkable apart from an unsuccessful pulmonary vein ablation for PAF, which took place 12 months previously. On admission, our patient had a heart rate of 80 beats/minute in sinus rhythm, blood pressure of 130/68mmHg, normal carotid pulse, and absence of murmurs or extra-cardiac sounds. A physical examination was normal and our patient was asymptomatic.
A two-dimensional TTE was performed, which showed a large, pedunculated, mobile mass sized 15 × 18mm located at the apical interventricular septum of his left ventricle, with a normally contracting adjacent myocardium (Figure a,b). His left ventricular systolic function and the morphology and function of his cardiac valves were normal. His previous TTE performed 12 months previously at the same laboratory and by the same sonographer did not demonstrate any left ventricular mass (Figure a,b). Further evaluation with cardiac computed tomography (CT) confirmed the presence of a nodular pedunculated mass (14 × 10mm) with radiographic evidence of myxoma (Figure a,b) that was not evident in the examination performed 12 months earlier (Figure a,b). The previous TTE and chest CT were performed before our patient underwent pulmonary vein ablation. Magnetic resonance imaging showed a nodular mass of 15mm with a 5mm stalk located at the anterior apical segment of his intraventricular septum. The intensity of the mass was higher than that of the myocardium, with no signs of infiltration of the endocardium.
Our patient underwent urgent cardiac surgery. Under cardiopulmonary bypass and cardioplegic protection, a left apical 2.5cm longitudinal ventriculotomy was performed, which exposed the mass in full view (Figure ). The defect was closed in a linear fashion and reinforced with felt strips (Figure ).
Resection of the tumor was performed through the ventriculotomy with macroscopically uninvolved margins. A pathology examination demonstrated the typical features of a myxoma (Figure ). Apart from two self-terminated episodes of PAF, our patient had an uneventful postoperative course and was discharged eight days after the procedure.
Echocardiographic follow-up has not shown evidence of recurrence eight months after the resection of the myxoma.


A 68-year-old Asian man presented to our cardiologic clinic with chief complaints of long-term swelling of his ankles, intermittent palpitations, and a propensity for becoming easily fatigued. On arrival, he presented the following vital signs: blood pressure of 121/71mmHg, body temperature of 35.9°C, and a heart rate of 52/minute. A physical examination revealed a grade III/VI blowing pansystolic murmur, best heard low over the left lower sternal border, an irregular heartbeat, jugular vein engorgement, and bilateral grade III pitting edema of his lower legs. The electrocardiogram showed sinus rhythm with frequent premature ventricular contractions. Color flow Doppler echocardiography showed an enlarged, hypokinetic right ventricle with a paper-thin right ventricular free wall and dilatation of his tricuspid valve annulus compatible with tricuspid regurgitation. Given the evidence from a serial laboratory study and image examination, the cardiologist had a high level of suspicion for arrhythmogenic right ventricular dysplasia. An EMB was arranged subsequently for sampling of the patient’s right ventricular pathological tissue via his right internal jugular vein. Using Seldinger’s technique, a 9 French silicone-locked catheter introducer sheet was applied. The bioptome was introduced via the sheet and then directed toward his right atrium. However, the cardiologist felt kinking while introducing the bioptome within the sheet just above the level of the patient’s right subclavian vein. A metallic cup tip was lost when the bioptome was withdrawn. The silicone introducer sheet was later removed without incident.
Out of concern for the floating foreign body inside the vasculature, an angiography via a right femoral approach was performed promptly, but no visible radiopaque foreign body was found within the patient’s vasculature with fluoroscopic guidance. He did not experience any immediate neurological or cardiovascular catastrophe. The cardiologist immediately arranged a computed tomography (CT) scan, and detected a high density lesion (Figure a) within the patient’s prevertebral musculature.
The head-and-neck surgeons arranged neck exploration the next day. Out of consideration for safe surgical removal, we adopted a right lateral cervical approach. After developing superior and inferior subplatysmal flaps, the patient’s carotid sheath was opened by retracting the sternocleidomastoid muscle posteriorly. We explored between his internal jugular vein and his common carotid artery after identifying the vagus nerve, and then continued dissection deep down to the buccopharyngeal fascia. However, we were unable to approach the foreign body using physical digital examination, even after identifying the vertebral transverse process and sympathetic trunk. We then applied a mobile C-arm (X-ray image intensifier, Figure b) to check the exact three-dimensional relationship of the foreign body to the vertebral body. We then identified the missing metallic cup tip, just medial to a vertebral vein, and stuck within the deep prevertebral muscles (Figure a). The patient underwent surgery to remove the metallic cup tip (Figure b) uneventfully, with no immediate sequela. He was discharged 10 days later. The wound healed well during follow-up.


A 29-year-old man with acute lower abdominal pain and vomiting was admitted to the emergency department of Shariati hospital in Isfahan province. The physical examination revealed that there is a large tender and mobile mass in hpogastric region. An abdominal distension was noticed too ().
He was afebrile (36.9°C) with normal blood pressure (123/82 mm Hg) but with tachycardic (112 beats per minute). White blood cell count, hemoglobin level, platelets count and urinary analysis were normal. A plain abdominal radiographic examination showed marked colonic gaseous distension present in the left upper quadrant.
Sonographic examination showed a homogeneous 21 × 15 × 8 cm mass in the lower part of the abdomen and pelvis with echogenicity that was consistent with normal spleen tissue while the normal splenic bed in the left upper abdomen was empty. The left kidney was atrophic measuring 85 mm in length which is showing increased parenchymal echogenisity. Right kidney was also enlarged to 129 mm in length. The lower poles of both kidneys were deviated medially suggesting a horseshoe kidney. No sign of hydronephrosis or stone was detected in the kidneys. Laparotomy examination confirmed the clinical and ultrasound findings. The enlarged spleen mass in the lower part of the abdomen and pelvis had a 720 degree torsion around a 25-cm long splenic pedicle with a horseshoe kidney. The spleen had no holding ligaments. Although a detorsion of the spleen was attempted, there was no success since the pedicle had become fibrotic. There were varicose dilatations in the veins surrounding the pedicle of the spleen. The splenic artery was double tied and excised.Then the splenectomy completed by double tying and excising the vein as well. The excised spleen had 843 g weight. (, , ). The patient had no additional problems during the follow-up period and was discharged three days later.


A 27-year-old Hispanic man with no significant prior medical history presented to the emergency room with shortness of breath and a cough for three weeks. He also complained of mild precordial chest pain and generalized fatigue. His family history was contained type 2 diabetes mellitus in both parents but reported no history of cancer or genetic diseases. His social history revealed that he worked as a gardener, was married with two kids, was a non-smoker, occasionally consumed alcohol and had no history of drug abuse. He gave no history of any psychological illnesses. A physical exam revealed sinus tachycardia, distant heart sounds and elevated jugular venous pressure and a normal blood pressure. Chest X-ray showed an impressive mediastinal enlargement. Transthoracic echocardiogram (TTE) showed a large pericardial effusion (
) and collapse of the right atrium during diastole along with respirophasic changes consistent with cardiac tamponade physiology. The patient underwent an emergent therapeutic pericardiocentesis that yielded hemorrhagic fluid. Cytological analysis of the fluid revealed no malignant cells. Computed tomography (CT) of the chest showed persistent pericardial effusion and a left anterior mediastinal mass (
). Work-up for possible metastases performed using Gallium scintigraphy showed no evidence of involvement of any other organs. Left anterior thoracotomy, pericardial window and left anterior mediastinotomy were done, revealing a well-encapsulated gelatinous tumor originating from the pericardium. The tumor grew from the inner surface of the pericardium near the origin of the superior vena cava and ascending aorta (
). A portion of the pericardium where the tumor was attached was resected and the tumor was biopsied. Complete resection was not attempted due to the proximity and attachment to the great vessels.
Review of pathology on routine hematoxylin-eosin stain showed that the tumor was monophasic and was composed of numerous spindle to oval cells in a fascicular growth pattern. The cells showed scant cytoplasm, oval to spindle nuclei with granular chromatin and with inconspicuous nucleoli (
). The tumor was mitotically active and showed 3–4 mitosis per high power field (
). Some areas showed myxoid background (
) and others showed large areas of necrosis (
). Immunohistochemistry showed that the tumor cells were strongly positive for BCL-2 (
), CD 99 (
), Vimentin (
), FLI1 (
) and neuron-specific enolase (NSE) (
). Stain for epithelial membrane antigen (EMA) showed focal scattered positive cells (
). Muscle markers (smooth muscle actin, desmin and myogenin), neural and melanoma marker (S-100), epithelial markers (AE1/AE3), CD57, CK5/6 and p63 were negative. Based on morphology and immunostaining results, a diagnosis of monophasic synovial sarcoma was made. Fluorescent
in-situ hybridization (FISH) was done to confirm the diagnosis and to exclude Ewing sarcoma. It showed the presence of SS18 (SYT) gene rearrangement on chromosome 18q11, substantiating the diagnosis. Also the FISH assay for rearrangement or dosage abnormalities of the EWSR1 oncogene at Ewing sarcoma locus 22q12 was negative.
The patent was treated neoadjuvantly with high dose ifosfamide (2000 mg/m
2/day intravenous over 3 hours for 4 days) and mesna (1200 mg/m
2) repeated every 21 days between July and December 2012. On repeat CT imaging prior to planned surgical excision, the tumor had substantially decreased in size (> 50%) and the patient was noted to have elevation of left hemi-diaphragm indicating likely involvement of the left phrenic nerve.
A median sternotomy was performed and access to the heart and great vessels was obtained. The pericardium was opened and the tumor was seen abutting the bifurcation of the pulmonary artery and distal aortic arch, and involving a portion of the pericardium including the left phrenic nerve. The portion of the mass that was attached to the distal part of the pulmonary trunk and a lateral portion of the pericardium were resected. The tumor appeared to encase the left phrenic nerve consistent with the finding of elevation of left hemi-diaphragm on CT scan. This nerve was transected and its edges were controlled with hemoclips. The hemoclips also served as markers for possible future radiation treatment. The entire outline of aortic arch and the pulmonary artery were visible after removal of the thymus and the tumor, with no evidence of direct invasion. Once the tumor was mobilized laterally towards the left lung, it showed possible involvement of the wedge of the left upper lobe. The lung parenchyma was divided and a portion of the lung was transected from the left upper lobe. The entire tumor was removed en bloc with the surrounding tissue. The level 5 (subaortic) lymph node was also resected and sent for pathology.
The resected mediastinal mass (6.5 × 4 × 3 cm) was confirmed to be a monophasic synovial sarcoma on histology and immunohistochemistry. The adjacent lung was not involved by sarcoma. The three lymph nodes resected along with the mass were benign with non-necrotizing granulomas. The level 5 lymph node also showed non-necrotizing granulomas and was negative for malignancy. One of the resection margins was positive for malignancy. The patient recovered well after the repeat thoracotomy and EBRT was initiated 1-month post surgery. He had no recurrence of tumor on CT imaging at the first and second three-month intervals post surgery. Chest CT repeated at 9 months (third three-month interval) showed local recurrence. The patient was noted to have an anterior mediastinal mass extending to the left upper lobe of the lung and a left pleural effusion. There was no evidence for any distant metastases. The patient underwent resection of the mass and left upper lobectomy, left video-assisted thoracoscopy, drainage of pleural effusion and total decortication of left lung. Pathology confirmed the recurrence but the resection margins were free of malignancy. The pleural fluid was also negative for malignant cells. Chemotherapy was reinitiated with docetaxel (100mg/m
2) every 21 days along with steroid premedication with dexamethasone (4mg) for 5 days prior to treatment. This resulted in minor improvement on repeat CT imaging, but more importantly there was no further symptomatic worsening. The patient is clinically stable 18 months after diagnosis.


In June 2011, a 60 year old woman was admitted to our emergency room with complaints of shortness of breath, palpitation, chest pain and progressive dyspnea. She had history of dyspnoea on exertion and orthopnea too. She had no history of trauma or major illness. On her physical examination, arterial blood pressure was 135/90 mmHg, heart rate was 110 beats per minute, SpO2 94% on room air and respiratory rate was 24 breaths per minute. On auscultation, the lungs were normal and no cardiac murmur, no JVP raised or gallop rhythm was noted. Cardiac enzymes, biochemical analysis, and complete blood count were normal except for eosinophilia. Chest radiography noted that cardiomegaly with bulging left heart boarder at admission (). Electrocardiography (ECG) showed normal sinus rhythm with poor progression of R wave in lead v4, v5, and v6 and no changes in ST segment. Her myocardial-specific enzyme values were found in the normal range. Transthoracic echocardiographic investigation showed a large multicystic mass (size 77 × 57 mm) seen intrapericardially extending from left ventricle lateral wall to pericardial sac causing pressure effect on left ventricle. The abdominal ultrasonography revealed intracardiac hydatid and other cystic lesion either in the liver or other abdominal organs were not found. Contrast-enhanced CT confirmed the presence of a well-defined, thin-walled, homogeneous multiple cardiac hydatid cyst with internal trabeculae arising from pericardium with adjacent structure (). Results of serologic tests for hydatidosis (indirect hemagglutination tests) were positive for E. granulosus, and marked eosinophilia was present too. After diagnosis, the patient was scheduled for surgery with the diagnosis of cardiac hydatid cyst. Patient was taken into the operation theatre and monitoring including ECG, pulse oximetry and non-invasive blood pressure were established and general anesthesia was performed with opioid-muscle relaxant technique after that established left radial artery cannulation for beat to beat blood pressure monitoring and internal juglar vein cannulation for central venous pressure monitoring. The pericardium was accessed through left lateral thoracotomy, accessing to the left mediastinal area, a solitary cystic lesion with a well-defined border was visualized in the pericardium adjacent to the left ventricle and the pericardial cyst was surgically removed (). Patient’s trachea extubated and Intraoperative and postoperative hemodynamic status was maintained and within normal limits. Post-operative pain managed with inter costal block with 0.5% of bupivacain plain, inj. tramadol and diclofenac sodium I.M. Pathologic examination of the specimen was shown with hydatid cyst.


A 53-year-old female was admitted to the First Affiliated Hospital of Xi’an Jiaotong University, (Xi’an, China) after presenting with a mass in the left upper quadrant which had been there for three months. The patient did not present with any other clinical manifestations. The study was conducted in accordance with the Declaration of Helsinki and was performed with approval from the Ethics Committee of Xi’an Jiaotong University. Written informed consent was provided by the participant.
An abdominal B-ultrasound, performed prior to hospitalization, revealed multiple mixed cystic-solid lesions in the left upper quadrant and the lower abdominal region. A needle biopsy of the mass revealed hyperplasia of fibrous granulation tissue, infiltration of chronic inflammatory cells, focal shape necrosis, active growth and tumor-like hyperplasia in sections of the cells. The histological features prompted the consideration of a mesenchymal tissue tumor. Following admission, the patient had a cancer antigen (CA)-125 level of up to 4,712 U/ml (normal <35 U/ml). Subsequent abdominal and pelvic computed tomography (CT) scans revealed multiple mixed cystic-solid lesions and abdominal and pelvic cavity effusion, presumably due to an ovarian malignancy and multiple metastatic intraperitoneal tumors (, prior to chemotherapy). Pathological results of the needle biopsy on the pelvic mass revealed a level II papillary adenocarcinoma accompanied by necrosis.
The patient was diagnosed with ‘abdominal and pelvic cavity metastasis of ovarian carcinoma’ and administered six cycles of systemic chemotherapy (TP scheme). The patient received 100 mg docetaxel (day l) and 40 mg cisplatin (days 1–2) for 21 days in each cycle. During the course of therapy, the CA-125 level decreased from 4,712 U/ml to 39.42 U/ml. Follow-up CT reexamination demonstrated that the lesions had markedly diminished, thus, the therapeutic evaluation was partial remission (PR) (). Subsequently the patient underwent ovarian cytoreductive surgery in addition to a splenectomy. The final pathology results revealed OCCA (oxyphil cell type) with massive necrosis and splenic metastasis of OCCA (oxyphil cell type) with massive necrosis, which were in accordance with the chemotherapeutic results. The immunohistochemical results were positive for CA-125, cytokeratin (CK)7, CK19 and epithelial membrane antigen, but negative for CK20 and CD68 ().


The patient is a 29 year old lady, married and house keeper .she came to our office with an erythematous nodule on back of her right hand and multiple painless subcutaneous nodules that are in linear pattern spread toward the elbow. The patient had a precedent history of a penetrating injury When she was cleaning the fish tank in her house (one centimeter length) just at the site that first nodule appeared. One week later she developed local redness with linear spread toward the elbow at the same hand. She received 200 mg Doxicycllin per day for 14 days. Although redness decreased but nodules size did not have any changes and after that multiple subcutaneous nodules appeared (). In physical examination her general condition was good, vital signs were stable and had not any significant problem but in her right hand in 3rd metacarpofalingial region had a 1 cm superficial wound with an erythematous nodule and multiple satellite papules around it. There were 7 non tender approximately 1.5 cm nodules between wrist and elbow. There were no epitrochlear or axillary lymphadenopathy. She had complaint about itching at the wound site.
In past medical history she had cesarean section 7 month ago and hypothyroidism since one year ago that she was on levothyroxin. She had not significant diseases in her family history. On laboratory investigations, the blood, urine and stool were all within normal limits. Gram staining and smear for Leishman-Donovan bodies was negative. Hepatitis B surface antigen and HIV screening were negative and PPD test was positive (20 mm). Chest X-ray was normal. In Gram Stain Gram positive bacilli was seen with many PMN and in Ziehl Nellson Stain Few Acid fast Elements were seen. Microscopy and direct KOH test for fungal spores were also negative. Bacterial culture of blood and wound secretion also had not any significant data. Histopathology of skin revealed acanthosis of the epidermis with dermal lymphocytic infiltrate and few Langhan’s giant cells while fine needle aspiration cytology showed a tuberculous picture. Diagnosis was M. marinum lymphangitis and the patient were treated with the following medications; Ethambotul 800 mg/daily, Rifampicin 600 mg/daily and Doxicycllin 100 mg/bid. After four month patent had not any sign and symptom and treatment were discontinued after 6 months.