A 35-year-old woman presented to an outside medical clinic in Yazd, Iran, in January 2012 with low back pain. Her pain was started since several years ago. Physical examination had shown moderate splenomegaly without any evidence of lymphadenopathy or hepatomegaly. Routine blood test results were normal, except an elevated level of alkaline phosphatase (ALP=350U/ l, normal: 20–125 U/l). Radiographs had demonstrated numerous lesions throughout the lumbar vertebrae () and skull (not shown here), suggestive of osseous metastases. A bone scan had shown avid radioisotope uptake in the mentioned areas. Abdominal CT-scan with contrast had shown multiple well-defined low-attenuation mass lesions in spleen and liver without marked enhancement (). Biopsy of the posterior iliac crest was performed at that time reported as myelofibrosis probably secondary to osseous metastasis. Subsequently, the patient had undergone splenectomy in a private hospital. The pathology report of splenectomy material was malignant vascular tumor. As a result, the patient had received 6 cycles of chemotherapy. In order to severe pain in lumbar area and lower extremities that were unresponsive to chemotherapy, the patient referred to our center in February 2014. All the pathology slides were subsequently reviewed at our institution in conjunction with the radiographic studies. The pathologic features of the posterior iliac crest biopsy showed thin-walled vessels within the marrow spaces of cancellous bone with associated reactive fibrosis (). These vascular spaces were dilated, filled with blood, and lined by bland endothelial cells without nuclear pleomorphism or mitotic activity to suggest a malignant vascular neoplasm. The findings were felt to be, instead, characteristic of angiomatosis of bone. The slides related to splenectomy material revealed spleen tissue with tumor composed of larger vessels with cystically dilated lumina and thin walls. Foci of old hemorrhage, fibrosis, and calcium deposition were also present (). There was no evidence of malignancy. The final diagnosis was generalized angiomatosis.


The patient was born prematurely (34 weeks and 1 day of gestation) at an outside hospital to a 29-year-old woman by elective cesarean section. The pregnancy had been complicated by preterm premature rupture of the membranes at 28 weeks and 2 days of gestation as well as prenatal diagnoses of cystic hygroma, pericardial and pleural effusions, left renal artery duplication, and bilateral ventricular hypertrophy with worsening myocardial function. This conglomeration of symptoms was later confirmed to result from Noonan syndrome. Specifically, this patient was found to have a PTPN11 gene mutation (c.923A>C; p.N308T).
At 12 hours of life, the patient became hypotensive and did not respond well to fluid resuscitation. Given the constellation of complications, the decision was made to transfer her to the tertiary care children's hospital for genetic assessment and cardiologic consultation. Upon arrival, the patient remained in respiratory distress and was hemodynamically unstable, and the placement of an umbilical catheter was attempted. A portable chest radiograph demonstrated a caudal loop of the catheter prior to ascending to the superior abdomen, so the catheter was assumed to be within an umbilical artery. However, the tip of the line was ultimately located to the right of the vertebral column (). Given this unusual position of the catheter, a cross-table lateral radiograph was performed. This demonstrated the tip of the umbilical line projecting over the expected location of the right atrium, at the level of the lower thoracic spine, and anteriorly and to the right of the expected position of the aorta (). A subsequent portable radiograph was obtained the following morning, redemonstrating the atypical position.
An ultrasound with Doppler assessment was performed to accurately determine the positioning of the line. The aorta was found to be unremarkable and without an intraluminal catheter. Instead, the catheter was identified within the inferior vena cava, with the tip positioned at the lower cavoatrial junction (). Confirming previous radiographs, the course of the catheter involved a caudal loop inferiorly and to the left, before coursing superiorly and to the right.
Further Doppler ultrasound imaging demonstrated an umbilical vein, which coursed inferiorly into the left iliac vein (). Sonographic imaging was unable to identify the ductus venosus. The development of the portal system, however, was not assessed in this patient. The patient's obstetrical records were obtained from the outside healthcare system, and these indicated a suspicion of atypical umbilical venous anatomy seen during prenatal ultrasound (). The umbilical catheter had no complications, and the remainder of the patient's hospital course was unremarkable. The patient is now one year old.


A one-year-old male patient with Down syndrome was in a late postoperative period of congenital cardiac disease correction. The patient had hypothyroidism and severe chronic malnutrition and had been hospitalized in a pediatric intensive care unit (PICU) since he was two months old.
After surgical cardiac disease correction, the patient evolved with limited nutritional reestablishment and recovery because of intolerance to enteral feeding and multiple infections associated with mechanical ventilation and invasive devices. He received broadspectrum antibiotics in a recurrent and prolonged manner. Therefore, the use of probiotics (Saccharomycesboulardii - 200 mg capsules) was indicated for four days at a dose of 200 mg, 12/12 h, via a nasogastric tube.
Two days after having finished receiving probiotics, the patient developed septic shock, resulting in the collection of cultures and the expansion of the antibiotic spectrum for vancomycin, ciprofloxacin, and amphotericin B. Initially, the central venous catheter was not replaced because of the small numbers of sites available (multiple strokes and previous phlebotomy). After five days of expanding antimicrobial therapy, and with the child stable from a clinical standpoint, the blood cultures (central and peripheral) identified yeast in a partial result, without the growth of aerobic bacteria. At that moment, Vancomycin and Ciprofloxacin were suspended and Amphotericin B was replaced by Fluconazole, with the hypothesis of Candida infection sensitive to Fluconazole.
After changing the therapeutic regimen, the patient’s clinical condition deteriorated, leading to an empirical extension of Amphotericin B, Vancomycin and carbapenem, in the absence of new blood culture results. Due to this extension, there was clinical and laboratory improvement of the infectious condition. The final identification of blood cultures, which were taken from central and peripheral sites, occurred on the seventh day of treatment and showed the presence of Saccharomyces cerevisiae, without available antifungigram. This result led to the discontinuation of antibiotics on the tenth day of prescription and the maintenance of Amphotericin B for up to 14 days after obtaining negative blood cultures taken at the beginning of the extended antimicrobial therapy. The patient had a central venous catheter and nasogastric tube, which were replaced after the identification of Saccharomyces cerevisiae. The peritoneal dialysis catheter and the tracheostomy tube were not replaced. It is worth noting that, at that moment, no other patient in the inpatient unit presented a Saccharomyces cerevisiae infection.
The patient remained hemodynamically stable, in nutritional rehabilitation therapy. The patient’s guardian signed the informed consent form and the institution’s Research Ethics Committee approved the report.


An 18-year-old Native American female with a past medical history significant for a bicycle versus motor vehicle accident presented to urgent care for evaluation of low back pain. The patient reported that she had waxing and waning low back pain ever since her bicycle collision four months prior, but for the last six weeks, it had insidiously increased to an unbearable intensity. On presentation, she described her pain as a dull, throbbing pain lasting all day, which was localized to the lower back region without radiation elsewhere. She rated her pain as “10/10” and noted that it worsened with prolonged immobility; however, the pain was not exacerbated by movement. She was previously treated with acetaminophen, ibuprofen, hydrocodone, and tramadol with little effect. The patient did not have any recent sick contacts or travels, and she did not believe her pain was related to her bicycle accident. She denied fever, chills, night sweats, weight loss, bladder and bowel incontinence, weakness, numbness, and saddle anesthesia. Except for obesity and her previously mentioned bicycle accident, her medical history was otherwise unremarkable. The patient was evaluated in the emergency department (ED) at the time of her accident and reported that she sustained no fractures or significant soft tissue injury. She had no prior surgeries, and her family history was also unremarkable. She was a student at a local community college and lived at home with her parents, boyfriend, and 16-month old son. The patient denied smoking or illicit drug use, but she endorsed the occasional alcohol use.
On presentation, her vital signs were as follows: blood pressure; 124/76 mmHg, heart rate; 84, respiratory rate; 16, temperature (oral), 36.6°C, and oxygen saturation (room air), 97%. Her body mass index (BMI) was 38.0. On physical examination, she was obese, alert and oriented, and in obvious pain. She had no gait abnormalities. There were no visible lesions, rashes, ulcers, or ecchymosis throughout her body, and there was no visible evidence of scoliosis, kyphosis, or lordosis. There was midline point tenderness along the spine in the L4-L5 region without significant para spinal muscle tenderness. No pulsatile masses were appreciated on the abdominal exam, and there was no costovertebral angle tenderness. The patient was completely neurovascularly intact and had full strength in all major muscle groups throughout her bilateral upper and lower extremities. All deep tendon reflexes were 2+ and symmetric, and the Babinski reflex was absent bilaterally. Forward flexion and extension of the spine were limited due to pain, but there was a full, active range of motion with lateral flexion. Straight leg raise was negative bilaterally. The remainder of her physical exam was unremarkable.
A complete blood count with differential showed no leukocytosis or other abnormalities. Because of point tenderness along the patient’s lower lumbar spine, lumbar radiographs were obtained. These radiographs showed normal bone density, normal alignment, and curvature of the spine, no evidence of acute fractures and overall no significant degenerative changes. However, there was a small Schmorl’s node noted in the inferior endplate of L5 best seen on the lateral lumbar view (Figure ). The anteroposterior (AP) view showed unremarkable findings (Figure ). Given the history of previous trauma, lower lumbar point tenderness on physical exam and radiographic findings consistent with an SNs, the patient was diagnosed with low back pain secondary to a trauma-induced SNs. The patient was then referred to an orthopedic spine surgeon who recommended physical therapy, weight loss, ice packs, and non-steroidal anti-inflammatory drugs (NSAIDs) as initial management. She was informed that surgical treatment existed if her symptoms persisted despite conservative management. The patient agreed with conservative management, and her pain improved over the next several months.


A 31-year-old female was referred to the outpatient Plastic Surgery Clinic due to an abnormal contour deformity of the right thigh. She had previously sustained injury as a pedestrian two years before referral, after being struck by a motor vehicle, and while down her right thigh, she was ran over by a second motor vehicle. Her chief complaint was a right thigh mass/deformity, which she noted to be chronically painful. On physical examination, she was noted to have a soft palpable enlarged area on the lateral right thigh measuring 15 × 15 cm; however, there were no local or systemic signs of infectious process ().
Magnetic Resonance Imaging (MRI) revealed a 1.7 × 0.7 × 5.5 cm fluid-filled structure along the iliotibial tract with tethering of the adjacent skin ().
The patient underwent suction-assisted lipectomy with utilization of tumescent fluid of the right thigh. The soft-tissue area was circumferentially marked preoperatively. General anesthesia was given and the patient was placed in the left lateral decubitus position. Three-millimeter incisions were made at the anterior border of the deformity and at the posterior border. Tumescent fluid was infused into the soft tissues and then three-millimeter cannulas were utilized for liposuction until satisfactory intraoperative contour. Incisions were closed with simple interrupted 5-0 polypropylene sutures and then wrapped with an ACE wrap for compression. The patient was discharged home the same day.
A follow-up appointment was conducted in the office one week postoperatively. There was a moderate amount of ecchymosis along the lateral right thigh; however, the previously noted contour abnormality was markedly improved. The patient had mild residual postsurgical pain but was satisfied with the early results.
Seven weeks postoperatively, another follow-up appointment occurred. The previously noted ecchymosis had completely resolved, the contour deformity remained considerably improved, and she was exceptionally pleased with her outcome (). Patient was again seen for follow-up at five months postoperatively. She remains very satisfied with the aesthetic appearance of the contour, with her only complaint being residual neuropathy, unchanged from preoperative symptoms.


A 69-year-old lady was referred from the dermatology service in May 2016 for assessment of thrombocytopenia and rash. The rash was predominantly localised to the right leg and lower back and developed 3 weeks after ipsilateral total knee replacement surgery. She had normochromic, normocytic anaemia (Hb: 9.4 g/l), thrombocytopenia (105 × 109/l) with prominent rouleaux on peripheral blood film, and moderate renal impairment without hypercalcaemia, with a normal erythrocyte sedimentation rate (ESR). Serum protein electrophoresis demonstrated a biclonal phenotype with a total IgG of 29.2 g/l, IgM of 0.44 g/l, and IgA of 33.8 g/l, with an IgA kappa band of 30.3 g/l and a small unquantifiable IgG kappa band. The absolute kappa free light chains were 259.08 mg/l, lambda free light chains were 48.72 mg/l, and the involved : uninvolved free light chain ratio was 5.32 (normal reference ranges: IgG 7–16 g/l, IgM 0.4–2.3 g/l, IgA 0.7–4.3 g/l, kappa free light chains 3.3–19.4 mg/l, lambda chain 5.71–26.3 mg/l, and ratio 0.26–1.65). Bence-Jones protein analysis was negative and beta-2-microglobulin was significantly elevated, at 10.48 mg/l. The X-ray skeletal survey was negative for lytic lesions.
Initial bone marrow examination demonstrated fewer than 10% plasma cells with no light chain restriction to suggest clonality. A repeat sample obtained from the contralateral iliac crest also contained fewer than 10% plasma cells, but these were kappa-restricted. Skin biopsy demonstrated changes consistent with the diagnosis of vasculitis. A fluorodeoxyglucose-PET-CT (FDG-PET-CT) was performed to localise the paraprotein-producing tumour, demonstrating bilateral FDG-avid cervical and axillary nodes, with a single mediastinal and portacaval node (Figures , , and ). The largest axillary node (7 mm) was biopsied revealing replacement of the nodal architecture by a CD138 positive, kappa-restricted plasma cell infiltrate ().
The patient was started on CyBorD chemotherapy (cyclophosphamide, bortezomib, and dexamethasone) with symptomatic improvement and resolution of the vasculitic rash. The paraprotein present in the beta region reduced to 7.2 g/l and the IgA kappa band became undetectable. Free light chain ratio normalised to 1.40 with an absolute kappa of 75.53 mg/l and lambda of 54.11 mg/l. Her anaemia significantly lessened, and thrombocytopenia resolved between chemotherapy cycles. A very good partial response (VGPR) was achieved according to the International Myeloma Working Group (IMWG) criteria. Moreover, repeat FDG-PET-CT following 4 cycles of treatment showed a complete response (Figures , , and ).


A 63-year-old woman, housewife, with 4 years of education, was evaluated at the
outpatient neurological unit due to gait disorder, cognitive decline and urinary
incontinence in September 2008. Her history had begun about 19 years earlier, when
she developed headache and fever. At that time, cerebrospinal fluid (CSF) revealed
neurocysticercosis and she was treated with improvement of symptoms. Full
information on the medications she received at that time was unavailable, but she
received an anti-parasitic drug and corticosteroids. Three years later, she had
another episode of severe headache with nausea and vomits, which was successfully
treated with steroids, but since then she had had repeated episodes of headache and
vomits every two to three years. She had to be hospitalized several times and since
2003 had been taking steroids orally (Deflazacort 6 mg to 30 mg) almost
continuously. She also reported improvement of the headaches when submitted to
lumbar punctures for CSF analysis.
In 2005, she started to show progressive gait disturbance and complain of
forgetfulness. Her husband confirmed the memory decline. These disorders worsened
and about 2 years ago she started to have urinary incontinence. Due to gait
disturbances and memory decline she was unable to perform her usual house
chores.
She had been operated for benign thyroid cysts in 2003 and treated with thyroxine
since then.
At first interview, neurologic examination revealed an unsteady gait with widened
base. She was unable to walk in tandem. There was lack of body balance with slight
tendency for retropulsion. Fundoscopy revealed bilateral papilledema in regression.
She scored 24 on the Mini-mental State Examination (MMSE). Her scores on the verbal
fluency tests were very low (7 animals in one minute and 7 words starting with the
letter "p" in one minute). The remainder of the neurologic examination was
unremarkable.
A CSF analysis done in July 2008 disclosed 35 leukocytes (predominantly
lymphomononuclear cells with 5% eosinophils), 77 mg/dL of proteins, with high
concentration of gammaglobulin (27%; normal 7-14%), normal glucose and a positive
test for neurocysticercosis on antibody-enzyme linked immunosorbent assay
(Ag-ELISA). Serological tests for toxoplasmosis, cytomegalovirus and Epstein-Barr
virus (both IgG and IgM), and HIV were all negative. In October 2008, MRI of the
brain (not shown) had disclosed mild communicating hydrocephalus. At this time she
was submitted to a ventricular shunt with a ventriculoperitoneal catheter and an
almost immediate improvement was observed by the neurosurgeon (ECS). Also, a CT scan
performed after the derivation showed resolution in hydrocephalus, and a slight
increase in the right Sylvian fissure was depicted ().
The patient did not return for consultation until May 2010 when she informed that
after the ventricular shunt she had a rapid improvement of her urinary incontinence,
gait disorder and memory problems and had resumed her normal activities. However,
episodes of headache had reappeared together with somnolence, mental confusion and
urinary incontinency about 8 months earlier. At examination, no papilledema was
evident but a broad-based gait was observed. She scored 23 on the MMSE and was able
to say only 6 animals in one minute. CT scan revealed increased ventricular
enlargement (dilatation) (). Brain
MRI disclosed moderate hydrocephalus and the catheter tip of the ventricular
peritoneal shunt into the right lateral ventricle. There were multiple cysts in the
right Sylvian fissure, without scolex, and peripheral enhancement, whose appearance
resembled a "bunch of grapes" (,
 and ).
Dexchlorpheniramine 6 mg daily (2 mg t.i.d) and dexamethasone were introduced.
Dexamethasone was prescribed, in a tapering dose schedule, starting with 12 mg daily
and ending after twenty days.
There was a slight improvement but in September 2010 she was operated to insert a new
ventriculoperitoneal shunt. The patient experienced clinical improvement, with
improvement of gait disorders, urinary incontinence and remission of papilledema.
After three years of observation, the patient remains stable, in use of
dexchlorpheniramine at 4 mg daily. Follow-up brain MRI was performed and showed
resolution of hydrocephalus, remarkable reduction in the number of cysts in the
Sylvian fissure, with persistent slight peripheral enhancement after gadolinium and
high signal intensity on T2 / Flair (,
,
and ).
In the last examination in March 2012, she had resumed her normal activities, without
gait, cognitive or urinary problems and scored 30 on the MMSE.


A 77-year-old gentleman first presented to our institution four years ago for a second opinion and treatment options. His history dated back three months prior to presentation when he began to notice calf pain after biking. This was followed, a month later, by a mechanical fall after which he started experiencing constant mild back pain. During this time, he also noticed that his right testicle was enlarged. The testicular enlargement did not respond to antibiotics and he subsequently underwent an ultrasound which showed solid masses in both testes. PET scan demonstrated increased metabolic activity in the right testicle (SUV 6), a portion of the left testicle (SUV 5), and a flare in a 6 mm retroperitoneal lymph node (SUV 4). There was also increased metabolic activity observed in the lytic lesions involving the left pubic symphysis (SUV of 7.2) and L5 vertebral body (SUV 6.2). The patient underwent a bilateral orchiectomy and, soon after, presented to our institution. The pathology specimen was reviewed and showed lymphomatous involvement with sheets of medium-sized cells with blastoid morphology characterized by fine chromatin, occasional distinct nucleoli, and scant cytoplasm. Immunohistochemical stains revealed the neoplastic cells to be positive for CD10, CD34, CD43, BCL-2, and TdT and negative for CD3, CD5, and CD20. Flow cytometric immunophenotypic analysis showed an aberrant population of lymphoblasts that were positive for CD10, CD19, CD34, HLA-DR, and dim CD4 and negative for surface immunoglobulin light chains, consistent with B-LBL. A bone marrow biopsy performed at our institution showed only 2% blasts with no morphologic support for acute leukemia. Flow cytometry analysis of the bone marrow aspirate specimen revealed a very small population of B-lymphoblasts (0.006%), consistent with minimal disseminated disease (MDD) by B-ALL. Cytogenetic analysis performed on the bone marrow showed a normal male diploid karyotype, 46, XY[20]. Fluorescence in situ hybridization (FISH) using a probe specific for BCR-ABL1 rearrangement was negative. However, real-time PCR testing for BCR-ABL1 rearrangement, a part of routine panel testing at our institution, detected a low-level e1a2 BCR-ABL1 fusion transcript coding for the 190 kDa BCR-ABL1 protein (BCR-ABL1/ABL1, 0.48%). We subsequently performed a core needle biopsy of the patient's L5 vertebral lesion which was also consistent with B-LBL (). Given the presence of low-level BCR-ABL1 fusion in the bone marrow detected by PCR, we performed FISH analysis for BCR/ABL1 rearrangement in both the testicular biopsy and the L5 vertebral biopsy, which showed that 90% (testes) and 80% (L5 vertebral body) were positive for BCR/ABL1 rearrangement (). Meanwhile, PCR performed on a peripheral blood sample also showed a low-level of BCR-ABL1 gene rearrangement (1.46%). Based on these findings, the patient was diagnosed to have Ph+ B-LBL. He received rituximab, hyper-CVAD chemotherapy, and dasatinib, along with intrathecal prophylactic chemotherapy. He achieved a complete remission, documented by repeat marrow and PET imaging. He developed pleural effusion and fever with dasatinib, and thus dasatinib was switched to imatinib, and later nilotinib (for possible better antileukemia activity). Repeat PCR on bone marrow and peripheral blood samples showed no evidence of BCR-ABL1 gene rearrangement. However, a routine yearly PET scan performed 4 years after the initial diagnosis showed a new soft tissue mass below the right kidney. A biopsy revealed relapsed B-LBL (). Therapy with blinatumomab and ponatinib was planned. However, the patient developed aspiration pneumonia and died before therapy could be instituted.


A 65-year-old male, a farmer, from a subtropical area south of the department of Cauca, located in the Colombian Massif, with an average environmental temperature of 19° C. He works in continuous contact with poultry and has no previous history of importance. He consulted for intense holocranial headache of four months of evolution that yielded partially with NSAIDs. Forty-eight hours before admission he was exacerbated with nausea and generalized clonic seizures of the subintrant type. He was treated with a dose of intravenous loading of Valproic Acid, continued with 500 mg every 8 hours, which controlled the seizures during the time of hospitalization.
A neurological examination revealed an obscure, confused patient with normal fundoscopy, no signs of endocranial hypertension, nor meningeal, normal force in all four limbs. A determination of Reactive Protein C and Globular Sedimentation Velocity was performed, a hemogram with leukocytosis and neutrophilia. The ELISA test for HIV was negative. With a diagnostic impression of epileptic syndrome, a computerized axial tomography (CT) scan of the brain was requested and showed no signs of endocranial hypertension, expansive lesions, or other relevant lesions. A lumbar puncture was performed demonstrating hypoglycemia and hyperproteinorraquia (), so it was decided to study as meningitis. On the third day of hospitalization, the patient persisted with altered state of consciousness, and presented dysmetria, dysarthria, dysdiadochokinesia, a positive Stewart-Holmes sign and a right hand drive without nystagmus, nor intentional tremor. For persisting without clinical improvement, another lumbar puncture was performed, which found that he found a high opening pressure, pleocytosis and hypoglycemia. The Chinese ink staining allowed to identify encapsulated blastoconidides compatible with Cryptococcus spp that were confirmed in cultures in blood agar, chocolate and Sabouraud, where C. neoformans var. Grubii NIV in the Laboratory of the National Institute of Health of Colombia. Antifungal therapy with Amphotericin B at 50 mg daily and Fluconazole at 400 mg every 12 hours was initiated, with improved mental status, decreased febrile episodes, headache, ataxia and dysmetria. On the ninth day of hospitalization, the results of the MRI were reported: in the T1 segment diffuse hypointense cerebellar lesion with contrast enhancement without evidence of nodule or abscess; In the T2 Flair sequence, a predominantly right bilateral diffuse hyperintense cerebellar lesion corresponding to inflammatory changes was evidenced. (A, B, C). The definitive diagnosis of Cryptococcal Cerebellum was made. The same antifungal therapy was continued for one week until epileptic status was reported for a period of approximately 12 min and died.


A 22-year-old woman (height 160 cm; weight 50 kg; BMI 19.5 kg/m2), gravida 1 para 0, at 16 weeks of gestation (WG) presented to the emergency department complaining of lack of ability to walk and severe weakness of the lower limbs. She had no history of symptoms similar to GS, and there was no family history of neuromuscular diseases. She previously had been in excellent health apart from mild hyperemesis gravidarum, which resolved spontaneously at approximately 10 WG without medication. She had no diarrhea and denied taking herbal medicine, diuretics, laxatives, or glucocorticoids prior to her illness.
The patient was in her usual state of health when 5 days back she noticed progressive fatigue and bilateral muscle cramping predominating in lower extremities. The patient noticed knee buckling with frequent falls. Rapidly, within that span, the muscle weakness worsened, and she was confined to bed and unable to mobilize her legs, when she decided to consult in the hospital.
Her vital signs on examination were heart rate 130/min, blood pressure 100/65 mmHg, respiratory rate 22/min, oxygen saturation 99% on room air, and temperature 37°C. The patient's review of systems was found negative for shortness of breath, cough, and swallowing difficulties. On neurological examination, the patient was alert and well-oriented. Her motor strength in upper limbs was 4/5. Hip flexors, quadriceps, and hamstrings were 1/5 bilaterally; dorsiflexors and plantar flexors were 0/5 bilaterally. Her cranial nerve examination was intact, and the sensation was diminished to touch and pinprick on lower extremities. Cranial and neck muscles were normal, but examination revealed deep tendon reflex to be absent on knees and ankles. The examination of other systems was unremarkable.
Table shows the initial laboratory data of the patient. Serum level of potassium was 2.2 mmol/L, serum magnesium 0.57 mmol/L, creatinine 68 μmol/L, and urea 2.8 mmol/L. Serum levels of sodium, total calcium, glucose, cortisol, thyroid, and liver function tests were found to be in normal range. Other blood investigations showed the following: hemoglobin 12.4 g/dL, white cell count 7.5 × 103/μL with a neutrophil count 5.9 × 103/μL, and C-reactive protein 5 mg/dL. Serum creatine phosphokinase level was 55 UI/L.
Her electrocardiogram (EKG) exhibited sinus tachycardia with inversion of T wave in leads D III, V1 to V4 suggesting hypokalemia.
She was assessed by a neurologist, and a diagnosis of Guillain–Barré Syndrome (GBS) was suspected. Her time nerve conduction studies (Table ) revealed increased distal motor and sensitive latencies and reduced conduction velocities predominating in low members suggestive of acute demyelinating polyradiculoneuropathy, in keeping with GBS. Her cerebrospinal fluid (CSF) analysis revealed normal white cell count, protein, and glucose levels. The infectious and autoimmune screen was nonrevealing, and a fetal ultrasound scan showed an ongoing pregnancy at the age of 16 weeks and 6 days.
She was then shifted to ICU, and the next day, a course of IV immunoglobulins (0.4 g/kg per day for 5 days) was started, but this therapy resulted in a very poor effect. She was also given a continuous potassium supplementation in form of potassium chloride infusion (130 mmol/day) and intravenous magnesium chloride (40 mmol/day) under cardiac monitoring. Her muscle weakness worsened over 5 days of the treatment and spread to the upper limbs, trunk, and neck muscles. There was no associated difficulty in breathing or swallowing at this time. Serial biochemical evaluations during her hospital stay revealed persistent hypokalemia and hypomagnesemia despite parenteral supplements (Fig. ). This was further investigated by urine analysis, which showed the potassium to be 51.5 mmol/L (normal range 16–83), chloride: 91 mmol/L (normal range 73–167), calcium: 29 mg/L (normal range 66–200 mg/L), and creatinine: 11.5 mmol/L (normal range 3.5–23). Arterial blood gas analysis was performed with the patient breathing room air, which showed metabolic alkalosis (pH 7.48, HCO3 37.3, PaCO2 43.5). She also had increased plasma renin activity (2.6 ng/mL/h; normal range 0.12–1.59) and serum plasma aldosterone (720 ng/dL; normal range 80–365). Under the presenting circumstances, a tubulopathy causing renal potassium loss was considered. Based on the findings of hypokalemic metabolic alkalosis without hypertension, severe hypomagnesemia, and hypocalciuria, a diagnosis of GS was suspected. This diagnosis was ascertained with the sequencing of the implicated gene, which confirmed GS as a heterozygous mutation in SLC12A3.
It was decided to reinforce ion supplementation, so potassium and magnesium supplements were increased to respectively 195 and 60 mmol/day.
On day 7 of her hospitalization, her condition deteriorated, and her tidal volume and minute volume decreased with swallowing difficulty. Oxygen saturation was 90% at 10 L/min delivered via a mask. To prevent hypoxemia and avoid emergency intubation, she was intubated and ventilated on controlled mandatory ventilation mode, and her vital parameters (electrocardiogram, invasive blood pressure, oxygen saturation, and temperature) were monitored. At this point, hypokalemia of 1.9 mmol/L was evident along with magnesium 0.43 mmol/L. Periodic fetal evaluation documented fetal well-being and normal size fetus with normal liquor and Doppler.
On day 8, the hemodynamic status worsened and the patient experienced prolonged hypotension and intermittent episodes of bradycardia, which occurred spontaneously, without relation to vagal maneuvers, such as endotracheal suctioning. She required vigorous volume replacement with normal saline and the use of vasopressor (Norepinephrine infusion) to maintain her mean arterial blood pressure up to 65 mmHg. EKG examination showed sinus bradycardia with the rate of 45 and prolonged QT interval. Echocardiographic and Doppler assessment failed to reveal any abnormalities in myocardial morphology or function.
On day 9, spontaneously fetal demise was observed, and the patient developed ventricular fibrillation (VF). She underwent electrical cardioversion (200 J biphasic), which restored sinus rhythm. Serum level of potassium was 1.5 mmol/L, and serum magnesium was 0.4 mmol/L. In view of the recent clinical deterioration, the use of a potassium-sparing diuretic or aldosterone antagonist was discussed, but this approach could result in hypovolemia and possibly worsen the hemodynamic status. Spironolactone was finally administered to this patient (100 mg/day). Subsequently, she continued to present repeated VF on the same day, which was complicated by a refractory cardiac arrest despite advanced cardiac life support.


A 49-year-old female attending the National Severe Insulin Resistance Service had originally presented at age 32 years with gestational diabetes, which persisted postpartum. She developed central adiposity during puberty. She has a positive family history for diabetes (). Prior to referral, her glycemia had been managed with metformin (stopped due to gastrointestinal intolerance) and gliclazide, followed by insulin therapy and exenatide. Examination and DEXA imaging () confirmed central adiposity with peripheral lipodystrophy. Biochemical tests were consistent with those typical of patients with partial lipodystrophy. Therefore, having excluded mutations in LMNA (lamin A/C) and PPARG (peroxisone proliferator activated receptor γ), a diagnosis of FPLD1 was made. A low-calorie diet was introduced, but glycemic control remained suboptimal despite large insulin doses. Triglyceride levels were modestly elevated (>2 mmol/L) (). Referral for gastric bypass surgery was made at the patient’s request on clinical grounds at age 53. At this time, her body mass index (BMI) was 27.4 kg/m2. Blood glucose readings fell within days of surgery, requiring the cessation of all medication (), with a corresponding fall in hemoglobin A1c. Oral glucose tolerance testing was undertaken before and 3 months after surgery (). Postoperatively, glucose tolerance was greatly improved, and this was associated with dramatically increased L-cell–derived satiety hormones glucagonlike peptide-1 (GLP-1) and peptide YY (PYY). Glucose-dependent insulinotropic polypeptide levels were unchanged. Consistent with the reduction in adiposity and fasting glucose, fasting insulin levels were markedly reduced, whereas the incremental insulin response to the glucose load was similar to that found preoperatively, despite the much lower postprandial glucose levels.


This is a 53-year-old African female patient who was brought in by emergency medical services (EMS) after the patient got trapped in the elevator. At that moment, she started feeling anxious and scared following which she activated EMS. EMS arrived at the scene after 20 minutes. During their evaluation, the patient stated she had retrosternal chest pain which was dull in quality, nonradiating, and 7/10 in severity. The chest pain had resolved at the time EMS arrived. At the time the patient arrived at our hospital, she was asymptomatic. Physical examination revealed a blood pressure of 146/77 mmHg, heart rate of 42 bpm, and respiratory rate of 16 per minute. Initial EKG as shown in demonstrated a 2nd-degree Mobitz type 2 atrial-ventricular (AV) block 2 : 1 with a heart rate of 40 bpm. Laboratory data were remarkable for troponin I 0.03 ng/ml (normal range is <0.02 ng/ml), pro-Brain Natriuretic Peptide 271 pg/ml, potassium 4.1 mmol/L, creatinine 0.8 mg/dl, and hemoglobin 12.9 g/dl. The patient had normal hepatic function test and thyroid stimulating hormone (3.49 mU/mL). Chest X-ray showed normal cardiac silhouette and no sign of fluid overload.
Patient was admitted to coronary care unit (CCU) and a transcutaneous pacemaker was placed on demand. Labs were repeated 6 hours later after admission and troponin I trended up to 0.93 ng/ml, suggesting cardiac injury. Repeat EKG demonstrated T wave inversion in the anterolateral leads (). Patient was started on heparin drip and loading doses of Plavix and aspirin were given. Our patient did not have β-blockers because of her presentation with bradycardia. Transthoracic 2D-echocardiogram revealed left ventricular ejection fraction (LVEF) of 45% as well as apical inferior, anterior, lateral, and septal hypokinesia.
Coronary angiogram was done in the view of new AV block, T wave inversion on EKG, and elevated troponin. Coronary angiogram demonstrated nonobstructive coronary artery disease. Ventriculogram demonstrated anterolateral and apical hypokinesia consistent with takotsubo cardiomyopathy (). On further workup electrophysiology study revealed high-degree suprahisian AV block with intermittent 1 : 1 AV conduction, as well as 2 : 1. Recommendations were event monitor and follow-up.
Cardiac MRI was carried out just before discharge and showed normal LV ejection fraction (EF) of 66 and right ventricle EF of 66%. There was no evidence of myocardial scar, necrosis, or edema. LV apex appeared to be hypertrabeculated. Cardiac MRI findings were similar to those of the repeated transthoracic echocardiogram upon discharge which also showed LVEF of 60% with no wall motion abnormalities.


This is a previously healthy 49-year-old male who had a 3-month history of cough and worsening fatigue. The cough was productive for yellow brown sputum and was associated with a 30-pound weight loss. The patient also noted excessive lower extremity edema. He denied any smoking history and denied any respiratory conditions. Patient denied any industrial work or exposure to asbestos or other carcinogenic materials. Patient denied any personal history of cancer. He denied any prior history of diabetes or high blood sugars. He had no known medical problems and did not see a physician regularly. Upon presentation, he was acutely hypoxic saturating 93% on 4 L of O2 nasal cannula and tachycardic. On physical examination, his entire right lung sounds were diminished, with mild crackles noted bilaterally. Lower extremities showed 2 to 3+ pitting edema bilaterally without obvious erythema or weeping. Laboratory findings included an elevated white count of 23.8 × 10(9)/L, sodium of 128 mM/L, potassium of 4.4 mmol/L, chloride of 79 mmol/L, CO2 of 20 mmol/L, BUN of 21 mg/dL, creatinine of 0.57 mg/dL, and glucose of 453 mg/dL. Liver function tests were within normal limits. Lactic acid was 1.7 mg/dl. Hemoglobin A1c was 14.7%. Hemoglobin was 11.4 g/dL. Platelets were 476 × 10(9)/L. HIV test was negative. CT scan of the chest was done and showed a large right sided pleural effusion containing pockets of gas along with focal airspace disease within the lingula and left lower lobe (). Chest X-ray performed after thoracentesis showed a large right pneumothorax ().
He was placed on medical therapy for likely mild to moderate DKA along with broad spectrum antibiotic coverage with piperacillin-tazobactam (Zosyn), levofloxacin, and vancomycin. He underwent a right sided VATS procedure for drainage of the empyema and two chest tubes were placed. The right chest was entered through a more posterior incision where a large abscess cavity draining approximately 1300 mL of gray foul-smelling material was encountered. Pleural biopsy and cultures were taken. Initial sputum culture from admission demonstrated Candida species and many Gram-positive rods resembling Lactobacilli. Cultures from the pleural fluid obtained during the VATS procedure grew Lactobacillus gasseri. Antibiotics were deescalated to piperacillin-tazobactam alone. Postoperatively his course was complicated by acute respiratory distress syndrome (ARDS) and repeated CT chest imaging showed left sided empyema and left lower lobe collapse () and a left chest tube was placed for drainage on hospital day 8. Left lung pleural fluid cultures also grew L. gasseri. The laboratory methods for the sputum culture and pleural fluid aerobic culture (from both right and left lung pleural fluid) were a send-out test to a larger teaching hospital that our community hospital is affiliated with. Both cultures were identified as GP bacilli, alpha colony, Lactobacillus gasseri susceptible to penicillin []. Pleural biopsy revealed reactive mesothelial cells and acute and chronic inflammation.
On hospital day 10 he was weaned off of pressor support and underwent a tracheostomy and PEG placement. Patient continued to improve and was transitioned to tracheostomy collar by hospital day 13. Antibiotics were changed to Ampicillin/Sulbactam for the remainder of his hospital stay. Patient was discharged on hospital day 25 on Amoxicillin-clavulanate 875 mg/125 mg. On discharge he had one remaining chest tube on the right side, and there was a persistent bronchopleural fistula. Seven weeks after his initial presentation his chest tube was removed. His oral antibiotics were discontinued shortly thereafter. Chest X-ray was obtained four months after his hospital discharge ().


A 28-year-old male presented to the surgery outpatient department with the complaint of swelling in the scrotum for a period of four years. The swelling gradually progressed in size over four years and was not associated with pain or fever. There was no history of prior surgery or trauma to the scrotum. On examination, a 12 x 7 cm non-tender swelling was noted in the left hemiscrotum, and it was soft and fluctuant. The left testis was not separately palpable and we could get above the swelling. The testis on the right side was normal. A diagnosis of left hydrocele was made, and a left-sided eversion of sac under local anesthesia was planned.
On scrotal exploration under the spermatic cord block, a large 12 x 7 cm pink fleshy mass was seen in the left hemiscrotum with the left testis densely adherent to the upper pole of the mass (Figure ).
The mass was dissected from the testis and removed in toto (Figures -) and was sent for histopathological examination.
The patient made an uneventful recovery. Histology of the mass showed a tumor composed of spindle-shaped cells with blood vessels admixed with chronic inflammation (Figure ).
Immunohistochemistry with smooth muscle actin (SMA) showed focal positivity (Figure ) and with Ki67 showed low proliferation of the tumor cells (Figure ).
Based on the histology and immunohistochemistry features, the tumor was reported as a fibrous pseudotumor of the spermatic cord.


A 43-year-old female was admitted for perirectal abscess. Her comorbid conditions included hypertension, obstructive sleep apnea, type 2 diabetes, atrial fibrillation, morbid obesity, and rheumatoid arthritis (treated with chronic steroid therapy). The patient’s current presentation began with 1 month of reddish drainage from the right buttock. She reported no trauma, fever, or other systemic symptoms. On physical examination, she had a large perirectal abscess sized 12.24 × 8.56 cm with overlying necrosis and extensive erythema up to the lower back with discharge of brown-red material. Her creatinine was 3.7 mg/dL, which increased from her baseline of 0.8 mg/dL. She was started on intravenous cefoxitin and clindamycin. The findings revealed 10 – 12 cm of necrosis and left perirectal abscess involving the ischiorectal fossa but not extending to the rectum. Postoperatively, she was transferred to the ICU as she was hypotensive, requiring vasopressor infusion. The patient was taken to the operating room multiple times for debridement and split-thickness skin grafts from her left posterior thigh. Her condition continued to improve, and she was able to be moved out of the ICU. Her creatinine came back to 1 mg/dL.
On hospital day 17, she developed areas of numerous severely sharp, painful erythematous lesions with central dusky necrosis and subsequently ulcerated lesions ranging in size from 1 to 5 cm on the bilateral lower extremities (). Punch biopsy from these lesions was done. The final pathologic diagnosis was small-vessel vasculitis. It was thought that the lesions were pyoderma gangrenosum related to her underlying rheumatoid arthritis. Her steroid dose was increased to prednisone 30 mg daily for lesion treatment. The labs were sent off for the genetic hypercoagulable panel, including protein C, protein S, cryofibrinogen, reptilase time, DRVVT screen ratio, prothrombin G20210A mutation, lupus anticoagulant, and antithrombin III, which were all negative. The patient was discharged to a short-term rehab facility as her condition had improved. Three days later, she was readmitted again for sepsis syndrome due to infected ulcers on the bilateral lower extremities. She developed fever up to 38.2 °C and was hypotensive. Broad-spectrum antibiotic, vancomycin, and piperacillin-tazobactam were initiated again. Since the patient had numerous non-healing bilateral lesions on her lower extremities, which did not respond to steroids, skin biopsy of the same lesion was performed again and sent to a pathology laboratory in a tertiary facility. Final pathology showed extensive fat necrosis and lymphohistiocytic inflammatory cell infiltrate. Medial calcification of vessel walls was seen focally, and some pockets of neutrophils were visible. These findings were consistent with calciphylaxis. On review of the record, the patient also had secondary hyperparathyroidism due to vitamin D deficiency (). Her calcium and phosphorus were in the normal range. The intact parathyroid hormone (PTH) was mildly elevated at 88 pg/mL (14 – 72 pg/mL). Total vitamin D level was 11 ng/mL (31 – 100 ng/mL). Consequently, she was started on treatment with 15 L of oxygen by facemask for 2 hours a day, sodium thiosulfate 25 g intravenously 5 days per week, and sodium thiosulfate intralesional injections every 7 days. Daily prednisone 30 mg was maintained for rheumatoid arthritis treatment. Oxycodone, oral morphine, gabapentin, and acetaminophen were used to control the ulcer pain. Vitamin D level was repleted with cholecalciferol 1,000 µg per day.
On 6-month follow-up, the ulcerated lesions had satisfactorily improved (). Two out of four ulcer regions on her lower extremities were healed. The two remaining were in the process of healing. She has made good improvement in physical function.


A 30-year-old male with no history of gastroesophageal disease and with known allergic rhinitis due to HDM (Dermatophagoides pteronyssinus specific IgE 11 × 103 IU/L) diagnosed using a radioallergosorbent test, and asthma in childhood, received the first tablet (ACARIZAX 12 SQ-HDM, Dermatophagoides pteronyssinus and Dermatophagoides farinae 50%/50% ALK-Abelló, Hørsholm, Denmark) at an outpatient facility. The SLIT dosage-regimen was initiated as one tablet a day for a planned three years. The dose was held sublingually for one minute before any swallowing was permitted. The treatment was performed in the morning. During the first few minutes only discrete oropharyngeal itching was present. After 30 minutes the itching had subsided; however, concomitantly a debilitating reflux of stomach acid started. The symptoms included severe pyrosis, regurgitation, and nausea, which subsided a little during the following hours but worsened in relation to any meals and ingestions of any type of liquid. The patient continued on the medication at the prescribed dose in spite of these side effects. While recumbent or supine and during the night, the gastroesophageal reflux intensified, hence the symptoms of pyrosis and regurgitation. On these grounds the patient consulted his general practitioner (GP) after five days. He was prescribed 40 mg of esomeprazole daily. Furthermore, the GP added ranitidine 150 mg before bedtime. The treatment did, however, not resolve the problem and the pyrosis and regurgitation continued relentlessly at the same intensity. After two weeks of treatment the patient decided to stop the SLIT treatment after consulting the prescribing allergologist. The day after the termination of the SLIT, the patient was symptom-free. After six months, no further episodes of symptomatic gastroesophageal reflux have been reported. The SLIT therapy has been abandoned and the patient continues on subcutaneously administered immunotherapy (100,000 SQ-U Alutard® SQ,, ALK-Abelló, Hørsholm, Denmark) with good effect. As the symptoms went away concomitantly with the termination of SLIT, no further investigations were carried out, for example, gastroscopy, 24-hour pH measurement with impedance, and high-resolution oesophageal manometry.


A 47-year-old previously healthy female presented with abdominal pain and generalized discomfort over a span of 4 to 6 months. The patient had no exacerbating or alleviating factors. The respiratory, cardiovascular, and CNS system review was essentially normal.
The abdomen was grossly soft, with no rigidity or guarding. Upon auscultation, bowel sounds were audible in all four quadrants. On deep palpation, there was a mass in the right upper and right mid quadrant with overlying mild tenderness.
Her past medical history included nephrolithiasis and gastroesophageal reflux disease. Previous surgical history was significant for bilateral tubal ligation. Her family history was notable for bladder cancer in her mother. The patient denied taking medications or having any allergies. She was married, lived with her 3 children, and denied smoking, alcohol, and illicit drug use.
Due to persistent discomfort, she was evaluated by gastroenterology by an upper endoscopy, ultrasound of the abdomen, and colonoscopy which were all unremarkable. The preliminary labs included a CBC and a Basic Metabolic Panel which were essentially within normal limits.
Radiology work up included a CT scan of the abdomen and pelvis which revealed a 4.6 × 3.6 × 3.9 cm right paracaval mass extending to the right renal vein and artery alongside effacement of the inferior vena cava (). In addition, an enlarged and lobulated uterus was visualized in the pelvis.
A CT guided biopsy was performed of the retroperitoneal/pericaval abdominal mass, and multiple core samples were obtained. Initial light microscopy evaluation of the sample demonstrated evidence of atypical spindle cells however, the precise type of cancer could not be determined on this exam. The core samples were sent to pathology for additional evaluation.
A PET imaging scan was performed from the skull base to the mid-thigh with concurrently acquired low-dose CT for attenuation correction and anatomical localization. The scan showed a heterogeneous area of increased radiotracer activity in the upper right retroperitoneum in the region of the IVC. A large focus of increased radiotracer activity was noted along the right side of the uterus, more intense than usual for a benign fibroid. There was otherwise normal, physiological distribution of radiotracer activity throughout the visualized portion of the body. No other focal areas of abnormally increased activity were identified.
After obtaining sufficient evidence that there were no pulmonary or hepatic metastases, the patient was cleared as a candidate for surgery. The small intestine and large colon were mobilized and lifted out of the way. The large mass was identified in the retroperitoneal space. The liver was mobilized, and dense attachments to the gallbladder were taken down.
The inferior vena cava was completely mobilized and encircled near the iliac bifurcation giving us distal control of the inferior vena cava (). The aortic caval space was opened and para-aortic lymph nodes were removed all the way up to the level of the SMA. A vessel loop was used on the inferior vena cava above the level of the renal vessels, giving us proximal control. The kidney was mobilized off the retroperitoneum. The tumor was mobilized off the posterior spine. The right renal vein was visualized which was posterior to and encased in the tumor. After ligating the renal artery, the tumor was completely mobilized off the inferior vena cava using sharp and blunt dissection. The left renal vein was dissected out and preserved as was the suprarenal as well as the infrarenal vena cava just above the level of the bifurcation.
The tumor, as well as the right kidney was resected off the IVC en bloc using a bevelling technique to preserve the orifice of the left renal vein within the confluence of the suprarenal cava and then transecting the infrarenal vena cava just above the bifurcation. With the tumor removed, a 20 mm Gore-Tex graft was then doubled approximately and an end-to-end anastomosis was completed using running 4-0 Prolene (). This anastomosis, which included the renal vein on the left and the suprarenal cava, was tested, and flow was restored from the renal vein into the vena cava. After appropriate flushing maneuvers were performed, flow was restored and excellent flow was noted. Frozen section margins of the specimen were negative. Due to the concern for a secondary malignancy, a total abdominal hysterectomy was performed.
The closest margin was confirmed to be less than 1 mm and consisted of multiple mitotic figures per high-power field. The patient was subsequently classified as T1b N0 M0 AJCC stage IIA.
A radically resected retroperitoneal/Inferior vena caval specimen was submitted to pathology for evaluation. The soft tissue was fresh, 251 grams in weight, and 11.5 × 6.5 × 4.0 cm in dimensions. On sectioning the IVC, the cut surface showed a 4.5 × 4.2 × 4.0 cm firm pink-tan to white lobulated mass (). The mass had a whorled bulging cut surface and was well encapsulated. The mass extended into the wall along the posterior, superior, inferior, and medial aspect of the IVC (). The tumor measured 2.0 cm from the renal sinus. Representative sections of the superior, inferior and retroperitoneal margins of the IVC and mass were submitted for frozen section diagnosis. No areas of ulceration were grossly noted. On sectioning the kidney, the cut surfaces showed unremarkable cortical medullary junctions with a cortical thickness averaging 0.6 cm. The renal capsule was intact and unremarkable. No enlarged or palpable lymph nodes were noted in the renal hilum nor surrounding the IVC.
A uterus was also submitted to pathology which was 229 grams and 6.5 × 6.0 × 5.5 cm in dimensions. On sectioning, four firm irregular intramural masses were noted ranging from 1.2 cm up to 3.2 cm in greatest dimension. The cut surface of each was homogenous pink-tan to white whorled and bulging, grossly consistent with leiomyomata. No areas of calcification, hemorrhage, necrosis, or cystic degeneration were noted.
Microscopic evaluation of the tumor revealed malignant spindle cells with marked nuclear atypia. The malignant spindle cells had elongated eosinophilic cytoplasm and elongated nuclei with rounded ends. The mitotic rate was 7/10 high power fields, with focal microscopic necrosis. The cells were arranged in fascicles ().
The patient had an unremarkable postoperative course and was discharged the following week. A limited non diagnostic post surgical CT scan was performed for placement of radiation therapy. No radiological evidence of lung metastases or lymphadenopathy was noted.
Radiation therapy began with 10 mV photons being prescribed, 5400 cGy was delivered in 30 equal fractions and inverse treatment planning techniques were utilized to maximize dose to the target and minimize dose to the normal surrounding tissues, particularly the left kidney. Daily image-guided radiation therapy was utilized to facilitate precision of beam delivery.
The patient tolerated adjuvant radiation therapy well. She is presently without clinical evidence of disease and is scheduled for repeat CT of the chest, abdomen and pelvis for restaging purposes and treatment course.


We describe herein a case of a 53-year-old male who presented with a painless neck mass that he first noticed 30 years ago. The initially small mass has progressively grown and is now accompanied by night sweats and dizziness. The patient denies blurry vision, parasthesia, paralysis, palpitations, difficulty swallowing, or changes in voice.
Physical examination showed a right neck mass about 4 x 3 cm. It was non-tender, and does not move with swallowing. No lymphadenopathy was appreciated. Cranial nerves I-XII were grossly intact except for decreased sensation in his right lower mandible and the superior right aspect of his neck.
CT angiography of the neck demonstrated an avidly enhancing mass splaying the right external carotid artery (ECA) and the internal carotid artery (ICA). The mass begins 1.6 cm below the right CCA bifurcation with the bulk of the tumor measuring 4.3 x 3.4 x 5.8 cm. There was additional tumor projecting approximately 1.6 cm above the dominant mass. The right internal jugular vein is compressed ().
B-mode sonography showed a solid, well-defined, and hypoechoic tumor splaying the ICA and ECA (). Color flow Doppler sonography showed also splaying of the internal and external carotid arteries by the mass with intratumoral vascularity ().


A 22-year-old male with a history of ongoing IV heroin abuse presented to the hospital with a cough productive of reddish sputum and shortness of breath of 4 days duration. He denied fever, chills or other systemic symptoms. Past medical history was significant for a recent episode of left arm cellulitis around 2 months earlier which had resolved after treatment with amoxicillin-clavulanate. He had been seen several times in the outpatient and inpatient setting in the past for substance abuse where attempts at detoxification and rehabilitation had been unsuccessful.
The patient was in respiratory distress on presentation, and his vital signs revealed oxygen saturation of 85%, blood pressure of 170/95 mmHg, pulse rate of 99/min and respiratory rate of 25/min. Physical exam showed track marks in the left antecubital fossa and bilateral coarse rhonchi in all lung fields. The patient was emergently intubated and admitted to the intensive care unit for management of respiratory failure.
A chest X-ray () was obtained and showed the presence of multifocal rounded airspace opacities within both lungs. Subsequently, a CT scan of the chest (, ) was obtained which demonstrated bilateral pulmonary emboli along with bilateral conglomerate pulmonary nodules in both lower lungs.
Based on patient’s history of IV drug abuse, recent episode of cellulitis, concomitant lower back pain and consistent imaging findings, the diagnosis of right-sided infective endocarditis with septic pulmonary embolism was initially considered and patient was empirically started on IV antibiotics after drawing blood cultures. However, a transthoracic echocardiogram obtained the next day did not reveal any vegetations and the patient continued to remain afebrile with all blood cultures coming back negative. This prompted a reconsideration of the diagnosis. All radiographic images were reviewed again with radiology and were notable for the lack of necrosis in the pulmonary nodules. A follow-up abdominal CT also revealed the presence of retroperitoneal lymphadenopathy indicating a possible neoplastic process.
A thorough physical exam was performed again which revealed the presence of a small left testicular mass. Ultrasound of the testes was obtained which showed two complex left testicular masses containing cystic and solid components as well as microcalcifications. His serum alpha fetoprotein, lactate dehydrogenase and beta-human chorionic gonadotropin were all found to be elevated.
The patient was diagnosed with metastatic non-seminomatous germ cell tumor of the testis. Because of the large tumor burden, he was started on chemotherapy with cisplatin, etoposide and iphosphamide, and orchiectomy was deferred for a later stage when he was more stable. He was extubated after receiving his first cycle of chemotherapy, and underwent left orchiectomy and retroperitoneal lymph node dissection soon after, with biopsy confirming the diagnosis of germ cell tumor with residual viable teratoma. He continues to receive medical care for malignancy as well as for substance use disorder as an outpatient.


A 53-year-old woman was admitted to the hospital because of abdominal pain, fatigue and anorexia for 2 days. The patient was in her usual state of health until approximately 2 days prior to the admission, when fatigue and anorexia developed, followed by abdominal pain. She reported a low grade fever, night sweats, and about 11pounds weight loss over the past year. She had some shortness of breath, but no cough.
Splenomegaly with multiple splenic infarcts had been diagnosed by CT scan approximately a year earlier after an episode of left upper quadrant pain developed and had been treated by Coumadin for 3 months. Transesophageal echocardipgram and extensive coagulation studies did not reveal any abnormality. The etiology for the splenomegaly was unclear at that time. CT scan done 5 months ago demonstrated a new splenic infarct. The patient resumed Coumadin. The patient had hypothyroidism and hyperlipidemia. She was a middle school teacher, never smoked, and did not use alcohol or illicit drugs. She did not have any recent history of travel or pets exposure. Her mother had coronary artery disease. Her father had diabetes mellitus. Her brother had hypothyroidism. Medications included Synthroid, Coumadin, and Rosuvastatin.
On examination, the patient appeared anxious. Her temperature was 36.2 °C, blood pressure 108/59 mmHg, pulse rate 97 beats per minute, respiration rate 14 breaths per minutes, and oxygen saturation 97% while she was breathing ambient air. There was no scleral icterus. The abdomen was soft, with tenderness in the epigastrium. The spleen was firm and nontender and extended to the left lower quadrant and the supraumbilical region. There were several palpable lymph nodes in the left neck with the largest one in the left submandibular region. The remainder of the examination was normal. CT scan of the abdomen, pelvic, and thorax with oral and intravenous contrast after admission showed multiple small or borderline enlarged lymph nodes in the left axilla, mediastinum, and retroperitoneum. The spleen was markedly enlarged 22.8 cm in length with peripheral wedge-shaped hypodensities, suggesting infarcts (). A test for human immnunodeficiency virus was negative. Epstein Barr Virus antibodies, cytomegalovirus antibodies and toxoplasma antibodies were negative. Direct antiglobulin test and test for cold agglutinins were negative. Electrocardiogram was normal. Serum protein electrophoresis did not reveal an Ig M kappa paraprotein. Tests for hepatitis B virus surface antibody and antigen and hepatitis C virus antibody were negative.
The left submandibular lymph node excisional biopsy and bone marrow biopsy were done. Pathological examination of the biopsy specimen of the lymph node revealed monotonous nodular infiltrate of small to medium sized lymphocytes with irregular nuclear contours, condensed chromatin, inconspicuous nucleoli and small to moderate amounts of cytoplasm, with noted follicular colonization (). Flow cytometry showed an expanded population of kappa-restricted CD19+ CD20+ CD23+CD5-CD10- B cells with unquantified CD5+CD10+ T cells. FISH analysis was negative for Ig H/BCL1 translocation, IgH/BCL2 translocation and for a MALT1 gene rearrangement. Pathological examination of the bone marrow revealed that hypercellular marrow (80%), and lymphoid aggregates that made up approximately 20% of the marrow cellularity and interstitial lymphoid aggregates. Flow cytometry performed on the bone marrow aspirate demonstrated an expanded population (10%) of kappa-restricted B cells that expressed CD19, CD20, CD45, HLA-DR but not CD5, CD10, CD103, CD11c. FISH analysis performed on bone marrow was positive for IgH rearrangement, while negative for IgH/BCL1 and IgH/BCL2 translocations and for ALK1 and BCL6 rearrangements.
Splenic marginal zone B-cell lymphoma with involvement of lymph node and bone marrow was diagnosed. Position Emission Tomography (PET) scan for staging after the bone marrow biopsy showed hypermetabolic activity was limited to the spleen and goiter, which was consistent with the history of thyroiditis. The grossly enlarged spleen had nearly homogenous increased metabolic activity, measuring at least 22 cm in cross-section with extension of the spleen below the iliac crest. There were some peripheral wedge-shaped areas of decreased activity, reflecting areas of splenic infarction. After obtaining the second opinion from physicians in University of Pennsylvania, four cycles of Rituxan chemotherapy were given. The patient tolerated the chemotherapy well.
There was no clinical evidence of recurrence of the lymphoma and no new lymphadenopathy until 9 months after initial diagnosis. She presented to the oncology clinic with left upper extremity swelling. Deep venous thrombosis work-up was negative. Repeated PET scan demonstrated multiple left axillary lymph nodes extending to left retroclavicular region and a new lymph node posterior to the left scapula. The spleen was 4 cm smaller compared to the previous PET scan. The wedge shaped defect in the spleen had resolved. Excisional biopsy of the left axillary lymph node was done. Pathology examination demonstrated marginal zone B-cell lymphoma with follicular colonization pattern and increased numbers of large cells. The histological findings were not those of outright diffuse large B-cell lymphoma. Decision was made to resume Rituxan.
On follow-up after finishing second round of 4 cycles of Rituxan, the white cell count was elevated at 148 × 109/L with 95% lymphocytosis. Restaging PET scan showed 3 new left anterior cervical lymph nodes, about 8 mm in diameters in each of them. One new 2.5 cm lymph node was found in the right axilla. The spleen had enlarged compared with the PET scan 4 months ago. New areas of hypodensities within the lateral periphery of the spleen were consistent with new small infarcts. R-CHOP (Rituxan- Cyclophosphamide, Hydroxydaunorubicin, Oncovin, Prednisone) chemotherapy regimen was started after a port-A- catheter was placed. White cell count significantly decreased after 1st cycle of R-CHOP from 144 × 109/L to 61.2 × 109/L.
After two cycles of R-CHOP, patient developed more fatigue, right facial numbness and right ptosis, being diagnosed with idiopathic right-sided Bell’s palsy. PET scan after 4 cycles of R-CHOP demonstrated interval resolution of previously described metabolically active cervical and axillary lymphadenopathy. New metabolic activity within right inguinal lymph nodes and abdominal lymphadenopathy were found with stable splenomegaly. Patient received 1 cycle of Bendamustine (Treanda). She developed a possible “hematoma” on right medial elbow (). MRI of the right upper extremity without contrast revealed the mass was not of vascular origin, likely representing a deposit of the lymphoma (). During this hospitalization, patient needs blood transfusion consistently and she deteriorated quickly. Decision was made by family for comfort measures.


A 22-month-old Caucasian female presented to Louisiana State University (LSU) hospital with symptoms of a low-grade fever, cough, decreased activity and oral intake, and an associated bilateral swelling under the jaw line, as reported by her parents. She was born at term with no issues during pregnancy or delivery. She did not have any significant family history of immunodeficiency, although her maternal aunt had lupus, antiphospholipid antibody, autoimmune thyroid, and celiac diseases. She had an incomplete vaccination record and did not receive her 12-month-old vaccinations including Measles, Mumps, Rubella (MMR), Varicella, and Diphtheria, Tetanus, Pertussis (DTaP) #4. She had a history of recurrent otitis media with tympanostomy tube placement and eczema. The patient was tested for Mumps virus due to an incomplete vaccination history and a concern over parotid swelling. Her IgM was positive for the aforementioned virus. Her immunoglobulin levels were all elevated, including IgM, IgG, IgA, and IgE (Immunoglobulin M, G, A, E Flex® Reagent Cartridges). Her IgG subclass levels including those of IgG1, IgG2, IgG3, and IgG4 were all increased. Her respiratory panel for rhinovirus (FilmArray Respiratory Panel) and enterovirus (Cepheid Xpert EV Assay) was positive. Her cytomegalovirus (CMV) (COBAS® AmpliPrep/COBAS TaqMan® CMV Test), human immunodeficiency virus (HIV) (Clearview® COMPLETE HIV 1/2 Assay), and hepatitis panels (COBAS AmpliPrep/COBAS TaqMan HCV Test, v2.0) were negative. The child was leukopenic and was found to have cold agglutinin associated autoimmune hemolytic anemia. On physical exam, there was mild hepatosplenomegaly and mild bilateral cervical lymphadenopathy with 0.5–1 cm mobile lymph nodes. Her EBV viral capsid antigen (VCA) antibodies IgM and IgG were positive at 1.3 and >8.0, respectively; and her early antigen antibody, nuclear antigen antibody, and heterophile antibody were all negative, indicating acute primary infection (BioPlex 2200 EBV IgM and IgG Kits). Her plasma EBV viral loads by quantitative RT-PCR were between 9,000 and 20,600 copy numbers per microliter in serial testing (Viracor Eurofins' Assay, Viracor Laboratories, Lee's Summit, MO). Low levels of CD19+ B-cells (J3-119, Beckman Coulter, Brea, CA), CD3+ (UCHT1, Beckman Coulter), CD4+ (SFCI12T4D11, Beckman Coulter), and CD8+ (SFCI21Thy2D3, Beckman Coulter) T-cells and CD16+ (3G8, Beckman Coulter) and CD56+ (N901, Beckman Coulter) natural killer cells were found by flow cytometric analysis of her peripheral blood. She was given Doxycycline, Vancomycin, and Ceftriaxone. Her blood and urine cultures were negative. During her inpatient hospital stay, her respiratory status deteriorated, which required oxygen infusion and admission to pediatric intensive care unit. Chest X-ray (CXR) and chest computerized tomography (CT) scans found that the patient had bilateral perihilar pulmonary infiltrates with right middle lobe consolidation which were initially interpreted as pneumonia. She was started on Gentamicin and Azithromycin. Her inflammatory markers trended down with her plasma EBV viral load at 9100, and her clinical symptoms improved, although her pulmonary infiltrates persisted per CXR. She was discharged to home temporarily to finish 7 days of Amoxicillin for a total 10-day course of antibiotics for pneumonia. Allergy and immunology service was consulted. She was assessed to possibly have impaired immune function due to her hypergammaglobulinemia and decreased circulating lymphocytes and their subsets. Her EBV load increased to 24,500 a week later and stayed at about 15,000. She was given Vancomycin and Cefepime.
Due to her febrile neutropenia and associated skin rash, she was readmitted to LSU hospital a month later. CXR showed bilateral pulmonary alveolar and interstitial infiltrates. Her soluble interleukin-2 level was elevated at 3051 U/ml. She did not meet 5 of 8 criteria of hemophagocytic lymphohistiocytosis. A normal neutrophil oxidative burst was observed in the patient's blood sample following phytohaemagglutinin (PHA) stimulation (Dihydrorhodamine Flow Cytometric Test, Mayo Clinic Medical Laboratories, Rochester, MN). Significantly decreased CD45+ (J33, Beckman Coulter) total lymphocyte and CD3+ T-cell proliferative responses were found with PHA stimulation; but normal and robust lymphocyte proliferative response to pokeweed mitogen (PWM) was noted (Invitrogen Click-iT EdU Assay for lymphocyte proliferation to mitogens, Mayo Clinic Medical Laboratories). Three lung biopsy specimens were obtained and all revealed a diffuse proliferation of large atypical cells lacking any significant architectural pattern (). Many of these cells had large irregular nuclei with occasional prominent nucleoli (). Angioinvasion of the blood vessels was present () along with areas of necrosis (). There was a background of small lymphocytes scattered amid the larger more atypical cells. Background alveolar lung parenchyma was present, which was histologically unremarkable. The sheets of large B-cells were CD20-immunopositive (L26, Ventana, Tucson, AZ) (). The large atypical lymphoid cells were positive for CD10 (SP67, Ventana) () and MUM1 (MRQ-43, Cell Marque) and negative for BCL-6 (GI191E/A8, Cell Marque) and had a high 90% proliferation index by Ki-67 (30-9, Ventana) immunostain. EBV by in situ hybridization using Epstein-Barr virus-encoded small RNA (EBER) probe (EBER 1 DNP Probe, Ventana) was diffusely positive in about 80% of tumor cells (). The lymphoma cells were also positive for CD30 (Ber-H2, Ventana), Epstein-Barr virus latent membrane protein 1 (LMP1) (CS1-4, DAKO, Santa Clara, CA) (performed at Center for Cancer Research, National Institutes of Health, Bethesda, MD), and EBV nuclear antigen 2 (EBNA2) (PE2, Novocastra) (), but they were CD15-immunonegative (MMA, Ventana). Special stains for acid-fast bacillus (AFB), AFB-Fite, and Grocott's methenamine silver (GMS) were negative for microorganisms. Flow cytometric analysis of lung biopsy specimens showed a monoclonal large B-cell population with a kappa restriction comprising about 2.8% of total cells (Figures –). Molecular diagnostic studies for immunoglobulin heavy chain frameworks I, II, and III and kappa light chain V-J and V-Kde gene arrangements by PCR were all positive (Invivoscribe Technologies, Inc., San Diego, CA). The FISH assay for MYC gene rearrangement (Abbott Molecular, LSI MYC Dual Color Break Apart Probe) was negative. The patient was subsequently transferred to St. Jude Children's Research Hospital. She was unresponsive to various therapeutic regimens including chemotherapy and cellular therapy (detailed treatment protocols and information were not available for release at the time of manuscript submission) and further developed EBV+ DLBCL lymphoma in the central nervous system and later expired.


An 81-year-old woman presented with erosions and crusted papules on the scalp and patchy areas of alopecia which had been progressive over many years. The largest of these was excised, as it was hyperkeratotic and ulcerated and therefore mistaken clinically for a squamous cell carcinoma. The rest of the scalp was covered with erosions and crusted plaques (). Her past clinical history was significant only for numerous nonmelanoma skin carcinomas, especially squamous cell carcinomas on her scalp. Of note, there was no known history of hyperuricemia or gout. The patient presented with numerous actinic keratosis like areas, and in this case with a larger area that had ulcerated, and within the context of her history being one of actinic damaged scalp skin and multiple actinic keratoses, a diagnosis of squamous cell cancer was made.
Excision revealed a variably atrophic epidermis with an impressive crust containing neutrophilic debris (). High power examination of the horn-like crust revealed numerous neutrophils, bacterial (coccal) colonies, and strikingly pale eosinophilic material (). On close examination, this material was revealed to have birefringent filamentous areas and a crystalline appearance (). Gram stain revealed gram-positive cocci in the crusted areas. PAS stain revealed rare pityrosporum species. In the epidermis, there was neutrophilic infiltration with accumulation in the superficial layers. The dermis showed a mixed chronic inflammatory response and some dermal fibrosis ().


A 38-year-old male presenting with excruciating posterior left sided neck pain for four days, dysphagia and neck stiffness. The emergency department staff was initially concerned for abscess of the deep spaces of the neck. The CT scan of the neck with contrast was done and it revealed a prevertebral retropharyngeal low attenuation collection spanning from C1 to C5 (Figure ). It also revealed dystrophic calcifications anterior to C1 and C2 (Figure ). There was no enhancement of the fluid collection. Furthermore, there was no evidence of neck adenopathy or bone destruction to suggest a retropharyngeal abscess. Magnetic resonance imaging (MR) of the neck was done in an attempt to better characterize the disease process. It consolidated the CT findings (Figure ) by demonstrating the retropharyngeal effusion. The patient’s symptoms improved significantly with few doses of Ketorolac and Acetaminophen after a brief period of hospitalization.


A 53-year-old female presenting with neck pain and dysphagia for five days. The patient had normal lateral neck radiograph from 2007 (Figure ). The CT scan of the neck without contrast was done and it demonstrated amorphous calcifications just inferior to the anterior arch of C1 (Figure ). It also revealed retropharyngeal effusion (Figure ). There was no evidence of neck adenopathy or bone destruction to suggest a retropharyngeal abscess. The patient symptoms improved drastically with nonsteroidal anti-inflammatory drugs (NSAIDs) and heat therapy.


A 31-year-old man underwent living nonrelated kidney transplantation at age of 16 years for ESRD secondary to biopsy-proved immune-complex mediated membranoproliferative glomerulonephritis. The patient was maintained on cyclosporine A, prednisone, and azathioprine immunosuppression regimen. His early posttransplant course was complicated with lymphocele, which was percutaneously drained and pseudomonas aeruginosa urinary tract infection, which was treated by meropenem. The patient was noncompliant with his immunosuppression therapy and the clinical and radiological follow-up was not optimal due to patient's poor compliance. He experienced multiple episodes of acute cellular graft rejection, which resulted in severe transplant glomerulopathy, global glomerulosclerosis, severe interstitial fibrosis, and tubular atrophy and severe chronic allograft arteriopathy. Radiological studies during the follow-up period showed no evidence of tumour neither in native kidneys nor in renal allograft. Haemodialysis was resumed 11 years after transplantation due to failed renal allograft. Four weeks after resuming haemodialysis, the patient presented to the hospital emergency with a history of watery diarrhea, vomiting, and fever associated with rigor and mild diffuse abdominal pain for 3 days. In addition, he reported an episode of gross haematuria. The patient's vital signs were as follows: body temperature 37.9°C, respiratory rate 20/min, heart rate 101/min, and blood pressure 138/93 mmHg. His oxygen saturation was 100% on room air. Physical examination revealed mild generalised abdominal and graft tenderness. The rest of the physical examination was unremarkable. Urine analysis showed white blood cells (WBC) 7/HPF, red blood cells (RBC) 1/HPF, and protein 600 mg/dl. Laboratory tests showed haemoglobin level of 143 g/l, white blood cell count 3.9 × 109/L, platelet 150 × 109/L, normal electrolytes, blood urea nitrogen 6.2 mmol/L, and serum creatinine 801 μmol/L. Abdominal ultrasonography (US) demonstrated 14.3 cm renal allograft with well-defined, round lesion at the mid zone of the kidney, measuring 1.8 cm in maximum dimension, highly suspicious for malignancy. Both native kidneys were atrophic with no evidence of cystic changes or focal lesion. Abdominal computed tomography (CT) scan showed 1.8 cm enhancing lesion in the middle third of renal allograft (). The patient was diagnosed with acute gastroenteritis and mass suspicious for malignancy in the renal allograft. He was treated with cefazolin and gentamycin, which resolved the gastroenteritis. Later, the patient was admitted electively and underwent embolization of the renal artery of the renal allograft () and graft nephrectomy.
Gross examination of the graft nephrectomy specimen showed well-defined, solitary, round, mahogany corticomedullary tumour in the mid zone of the renal allograft, measuring 1.6 cm in maximum dimension. No tumour central scar, haemorrhage, or necrosis was seen. The tumour was confined to the kidney and showed no extracapsular extension. Sections from the tumour were fixed in 10% buffered formalin, paraffin-embedded, sectioned at 5 μm, mounted on coated glass-slides, and stained by routine haematoxylin and eosin stain (H&E). A panel of diagnostic immunohistochemical (IHC) stains was performed (). All immunoassays were performed according to the manufacturer's guidelines, using automated platform (Ventana Benchmark XT, Tucson, AZ, USA) and heat antigen retrieval by ultracell condition solution PH 8.4. Ultraview universal DAB detection kit was used for reaction visualization. Proper positive and negative controls were utilized for all IHC stains.
Histopathological examination showed well-circumscribed eosinophilic variant of ChRCC. The tumour consisted of medium to large, round, and polygonal neoplastic cells that exhibited mild degree of nuclear pleomorphism and arranged predominantly in variably sized nests, cords, and poorly formed acini and had hyperchromatic nuclei, indistinct nucleoli, irregular “raisinoid” or round nuclear cell membranes, abundant amount of dense eosinophilic cytoplasm, and poorly defined cell membranes (Figures –). Scattered binucleated cells and occasional neoplastic cells with pale cytoplasm and perinuclear cytoplasmic clearing were present (). Mitotic activity was inconspicuous. No papillary growth pattern, foamy histiocytes, psammoma bodies, or background of extensive network of thin-walled “chicken-wire” vasculature was identified and no tumour necrosis was seen. There was no sarcomatoid differentiation. Lymphovascular invasion was not identified. Immunohistochemically, the neoplastic cells exhibited diffuse positive cytoplasmic staining for pancytokeratin (CK), low-molecular weight CK (CK8/18), CK7, and epithelial membrane antigen (EMA), and membranous staining for E-cadherin (Figures –), while IHC staining for vimentin, CD10, CD117, and renal cell carcinoma (RCC) marker was negative (Figures –). The remaining kidney showed severe segmental and global glomerular sclerosis, severe interstitial fibrosis and tubular atrophy, severe, widespread arteriolar hyalinosis, and severe chronic allograft arteriopathy.
The patient had an uneventful postoperative clinical course. On follow-up, the patient remains clinically stable on haemodialysis and without clinical or radiological evidence of locoregional recurrence or distant metastasis at 55 months after graft nephrectomy.


A 4.5-month-old caucasian female infant was presented in April 2016 to the Department of Hematology of the Institute for Childhood Diseases with an extremely low platelet count. The finding was a component of the evaluation prior to surgical treatment of the tumor on her neck, which seemed to be a congenital hemangioma.
She was the third child of non-consanguineous parents, from third healthy pregnancy, born at term by cesarean section: birth weight (BW) 4260 gr, birth length (BL) 57 cm, head circumference (HC) 37 cm, and Appearance, Pulse, Grimace, Activity and Respiration (APGAR) score 9/9. She was fed on adapted milk formula from the beginning. Her family history is unremarkable.
In the first hours after birth, a tissue mass in the cervical thoracic part of her vertebral column, which looked like a hematoma, measuring 1.5 × 1.5 cm was detected. In the further course of treatment the mass was defined as a tumor, 2 × 2 cm in size, which seemed to be a hemangioma (Fig. ).
The child had been taken to regular check-ups until the indication for operative treatment was established. Her blood parameters (leukocyte formula and platelets) were within the normal range prior to preoperative evaluation.
On admission, the child was conscious, body mass (BM) 5900 gr, with adequate spontaneous and provoked activity, afebrile with a body temperature (BT) of 36.7 °C, and with normal respiratory and heart rate; she was acyanotic, anicteric, and had normal osteomuscular status. Her vital signs were in the normal ranges. In her neck, in suboccipital region and medially, existed a tumor of hemangioma type, size 6 × 6 cm, of firm consistency. The skin above the tumor was cyanotic, with petechiae, without signs of devitalization. Other physical findings were normal.
The values of some laboratory parameters were: white blood cells (WBC) 9.21 × 109/l; red blood cells (RBC) 3.06 × 1012/l; hemoglobin (Hgb) 87 g/l; platelets 7 × 109/l; partial thromboplastin time (PTT) 12.6 seconds; international normalized ratio (INR) 0.98 seconds; activated partial thromboplastin time (APTT) 41.6 seconds; fibrinogen 1.0 g/l; and D-dimer 26.9 mg/ml.
Doppler ultrasound of the soft tissue of the posterior aspect of her neck revealed an extensive, soft tissue heterogeneous mass, predominantly hyperechogenic, with areas of calcification and enhanced color Doppler (CD) signal, which may correspond to a hemangioma.
A magnetic resonance imaging (MRI) examination revealed cutis and subcutaneous tissue of the posterior aspect of her neck, and a soft tissue, irregular, lobular mass, size 58 × 53 × 35 mm (craniocaudal × laterolateral × anteroposterior; Cc × LL × AP), which was completely hyperintense on T2, with single isointense to hypointense areas and isointense signal on T1, compared to surrounding structures. It was a completely nonhomogeneous structure, clearly bounded, not propagating toward the spinal canal with intensive inhomogeneous contrast enhancement. In conclusion, on MRI the mass had characteristics of benign soft tissue mass (Figs. and ).
As a result of our patient’s low platelet count and laboratory signs of consumptive coagulopathy, substitution with fresh frozen plasma (FFP) 15 ml/kg, along with intravenously administered corticosteroid (methylprednisolone) 2 mg/kg, were carried out daily during 7 days. The laboratory parameters improved: platelets 30 × 109/l; fibrinogen 1.5 g/l; and D-dimer 25 mg/ml. The child was discharged home in good general condition. Her parents were advised to take her for a check-up at Hematology out-patient clinic in 7 days with complete blood count (CBC), fibrinogen, and D-dimer. The re-planning of surgical management was also indicated.
Six days after discharge, the child was readmitted. Her parents noticed enlargement of the tumor in her neck and laboratory tests revealed a low platelet count. Compared with previous clinical examination, the tumor was larger and was 8 × 8 cm in size. The values of some laboratory parameters were: WBC 9.5 × 109/l; RBC 3.2 × 1012/l; Hgb 90 g/l; platelets 9 × 109/l; PTT 13.5 seconds; INR 1.05 seconds; APTT 36.4 seconds; fibrinogen 2.1 g/l; and D-dimer 12.7 mg/ml. Urine culture was sterile, while Staphylococcus aureus was isolated from chemo-culture. A cardiologist was consulted. Her electrocardiogram and echocardiographic findings were normal.
Intravenously administered corticosteroid therapy (methylprednisolone) 2 mg/kg, FFP 15 mg/kg, and symptomatic therapy were administered. Ten days after admission, a rise in her body temperature occurred. Her inflammatory parameters such as C-reactive protein (CRP) 6.8 mg/l were monitored and the symptomatic treatment was continued. The child also received RBC transfusion because of low Hgb values. The values of some laboratory parameters, 7 days after discontinuation of therapy, were: WBC 8.5 × 109/l; RBC 4.2 × 1012/l; Hgb 112 g/l; platelets 25 × 109/l; PTT 13.5 seconds; INR 1.05 seconds; APTT 36.4 seconds; fibrinogen 2.4 g/l; and D-dimer 10.9 mg/ml.
After 14 days of the second hospitalization, in consultation with a pediatric surgeon, treatment with propranolol was initiated at a total daily dose of 0.5 mg/kg divided into three doses. Her blood pressure, heart rate, and blood glucose were regularly monitored. On the second day, the dose was increased to 1 mg/kg, then to 2 mg/kg and on the fourth day, to 3 mg/kg. The values of monitored parameters were in physiological ranges. The control platelet counts were continuously increasing (from 5 × 109/l to 100 × 109/l) within 2 weeks, along with an improvement of coagulation status and reduction of hemangioma size to 5 × 5 cm. After 30 days of hospitalization, she was transferred to Department of Pediatric Surgery for further treatment (Fig. ).
After 3 weeks of propranolol therapy, the child underwent the surgical extirpation of the tumor. The tumor tissue was approached through an elliptical incision in normal skin area. By careful preparation, it was separated from the normal subcutaneous tissue. Among the thrombosed vasculature, the nutrient blood vessel was identified (Fig. ). The tumor was dissected down to the prevertebral fascia and removed. Hemostasis was achieved and a drain was placed in the tumor bed. Her early postoperative period was uneventful. The tumor was sent for histopathological (HP) examination which revealed well-circumscribed lobules of closely packed capillaries composed of plump endothelial cells and pericytes, separated by normal-appearing dermal stromal elements (hematoxylin and eosin stain; Fig. ). Immunohistochemical analysis of tissue was as follows: CD34 positive (Fig. ); Ki67 labeling index = 1% (Fig. ); and cells were positive for vimentin and negative for epithelial membrane antigen (EMA) and pancytokeratin (CK). Based on clinical and HP features, a definite diagnosis of hemangioma capillare cutis was made.
Dual antibiotic therapy (ceftriaxone, amikacin) was given postoperatively. The wound was cleaned and the dressing changed on a regular basis. The wound was clean and dry, and was healing by first intention. Sutures were removed on 7th postoperative day. The laboratory tests on the 3rd postoperative day showed normalization of CBC parameters and coagulation status.
She was discharged in good general condition on the 7th postoperative day with follow-up appointments with hematologist every 7 days for the first month, and then once a month for up to 12 months, along with the results of laboratory tests, platelet counts, and coagulation status parameters, which were within physiological ranges (Fig. ).


This is a case of a 54-year-old male who was treated at 13 years of age with a combination of chemotherapy, bone marrow transplant, and whole brain and spinal axis radiation (cranial 24 Gray) spinal (12 Gray-Gy) for acute lymphocytic leukemia (ALL). At the of age 46 years, while the patient was being evaluated with MRI scans for intermittent headaches, it was discovered that the patient had developed two intracranial tumors radiologically consistent with meningiomas. The tumors were a right cavernous sinus tumor of 13 x 10 millimeters and a larger multilobulated left tentorial tumor of 13 x 11 x 12 mm with direct compression on the left pons. The repeat MRI scans over a period of five years demonstrated the continued growth of the tumors, especially in the left tentorial notch. At the age 51 years, although the patient had no neurologic symptoms, because of continued tumor growth, he was referred for radiosurgical treatment. At this time, the right cavernous sinus tumor was 19 x 14 mm with a tumor volume of 792 mm3 and contiguous to the right anterior brainstem, while the left tentorial tumor was more lobulated and 24 x 14 mm with a tumor volume of 2002 mm3. There was marked direct indentation and pressure on the left lateral pons, including the root entry zone of the left trigeminal nerve (Figure ).
Both the tumors were treated in three sessions of radiosurgery using the CyberknifeR (AccurayR, Sunnyvale, California, USA) with the right cavernous sinus/posterior-clinoid tumor receiving 12 Gy to the 77% isodose line and the left tentorial tumor receiving 18 Gy to the 85% isodose line in three fractions of 600 cGy each (Figure ).
Nine months after radiosurgery to the tumors, the patient started developing lancinating left facial pain without numbness. He initially responded to medical management with carbamazepine 100 mg and gabapentin 300 mg daily. However, gradually over the next 15 months, his pain recurred and it was unresponsive to the medical treatment, despite increasing doses of carbamazepine to 600 mg daily and gabapentin 1800 mg daily. With high doses of carbamazepine and gabapentin needed to control his facial pain, he developed side effects which left him unsteady and somnolent. The repeat MRI scans showed the left tentorial tumor had reduced to 11.86 mm. The MRI scan, 24 months after original tumor treatment revealed 3.13 mm residual tumor adherent to the left fifth cranial nerve root entry zone with no tumor in the trigeminal cistern. He did not want to consider balloon compression and underwent another radiosurgery treatment of 70 Gy to the left Gasserian ganglion. He responded with loss of pain and was on baseline medication of gabapentin 300 mg two times daily and carbamazepine 100 mg daily (Figure 3).


A 76-year-old male had undergone an emergency coronary angiography for acute chest pain 1-year prior demonstrating a noncritical stenosis at his proximal right coronary artery (RCA) (Fig. ), which resulted in a conservative treatment. The patient received an optimal medical therapy including aspirin and statin along with antihypertensive and antidiabetic agents. However, a follow-up coronary computed tomography angiography at 1 year demonstrated an eccentric tight stenosis with a “Napkin-ring sign” which is known as an independent risk for an occurrence of acute coronary syndrome (ACS) (Fig. ).[ Given these findings and the fact that we were only dealing with a single vessel, it was decided to treat this lesion using PCI. During the procedure, coronary angiography demonstrated a critical stenosis at the proximal RCA, which was evaluated as tighter than previous with no stenosis on his left coronary system (Fig. ). Optical frequency domain imaging (OFDI, FastView; TERUMO, Tokyo, Japan) delineated a TCFA proximally with red thrombus distally, both of which suggested that this lesion was a high risk for embolic complications during PCI (Fig. A1, A2). To avoid these risks, ELCA was applied combined with intracoronary aspiration before DES implantation. Laser atheroablation was performed using a 1.4 mm Vitesse-Cos RX catheter (Spectranetics, Colorado Springs, CO) with an incremental energy setting, starting with 40 mJ/25 Hz and completing with 60 mJ/40 Hz, followed by intracoronary aspiration using a 7F Eliminate catheter (TERUMO, Japan), which resulted in unique OFDI appearances (Fig. B1,2–D1,2). The red thrombus was vaporized by ELCA in an energy-intensity dependent manner and was subsequently removed by intracoronary aspiration (Fig. B1–D1). The fibrous cap of TCFA was dissected with the material beneath the cap ablated by ELCA and extensively removed by intracoronary aspiration (Fig. B2–D2). After treating the stenosis with intracoronary aspiration, D1 (Fig. ) lumen had an area of 14.3 mm2 with 7.0 mm2 that of D2 (Fig. ), indicating greater than 50% residual plaque in the lesion which has been shown to be an independent risk factor for restenosis.[ To reduce this risk of restenosis, implantation of a 4.0 mm × 18 mm Resolute Integrity stent (Medtronic, Minneapolis, MN) was performed, followed by postdilatation with 4.5 mm × 15 mm Hiryu plus balloon (TERUMO, Japan) at 20 ATM, resulting in an optimal angiographic result without flow compromise (Fig. E). The final OFDI delineated that a large and clear lumen was obtained with minimal inter-strut protruding tissues observed (Fig. E1 and E2). The aspirated material was macroscopically yellowish white and the microscopic findings were consistent with an atherosclerotic plaque component (Fig. ). This combined synergistic strategy of ELCA-aspiration-DES yielded a successful outcome.


A 25-year-old male with a known diagnosis of autosomal dominant polycystic kidney disease (ADPKD), with preserved renal function, presented with sudden onset severe left flank, following a rugby game. The loin pain was associated with frank haematuria. On examination, he had severe tenderness to his left hypochondrium and renal angle. He was tachycardic and blood pressure was elevated at an average of 160/90 from a baseline of 130/80. A CT abdomen (Figures , , and ) revealed a large left sided haemorrhage surrounding the polycystic kidney which extended into the retroperitoneum. The renal lengths and volumes were 15.7 cm and 571 mL, respectively, on the right and 16.0 cm and 732 mL, respectively, on the left. He was managed supportively with intravenous fluids (0.9% sodium chloride solution, 125 mLs/h), analgesia (including paracetamol and codeine), and haemodynamic monitoring. Twelve hours after presentation his haemoglobin dropped from 132 g/L to 60 g/L with an associated fall in haematocrit, but with maintenance of blood pressure. This was treated with a blood transfusion (3 units packed red cells) and radiological embolization of the bleeding source (Figures , , and ). Renal function remained stable and the patient improved without the need for further intervention. He was advised to avoid contact sports in future and his haematuria and pain resolved within two weeks.