A 75-year-old man (Asian, Japanese) was admitted to our hospital for examination of a heart murmur and haemolytic anemia. The patient had a history of mitral valve annuloplasty with a Carpentier-Edwards Physio II Annuloplasty Ring, 30-mm (Edwards Lifesciences Corp., Irvine, CA, USA), TAP with a rigid prosthetic ring (Edwards MC3 Tricuspid Annuloplasty Ring, 32 mm; Edwards Lifesciences Corp, Irvine, CA, USA), and coronary artery bypass 3 months previously. At the initial operation, no debridement of the mitral annulus had been performed in the posteromedial commissure or vicinity. Postoperative echocardiography performed 1 month after the operation showed no mitral regurgitation and trace tricuspid regurgitation.On admission, a high-pitched pansystolic murmur was audible along the left sternal border. Laboratory studies showed decreased haemoglobin (6.7 g/dL), elevated serum lactate dehydrogenase (2,211I U/L), and fragmentation of red blood cells. Echocardiography showed a high-velocity jet originating from the LV outflow tract and extending to the RA along the tricuspid valve leaflet through a small defect (Figure
). However, no leaks were detected in the mitral position. Cardiac catheterization and left ventriculography confirmed LV-RA communication. We judged that surgical repair of the LV-RA communication was essential to treat mechanical haemolysis, regardless shunt amount.At operation, the patient was noted to have a communication just beneath the detached prosthetic ring at the anteroseptal commissure of the tricuspid valve, and a jet of bright red blood entering the right atrium through the defect (Figure
). Two previous TAP mattress sutures were removed, and the detached rigid TAP ring was then cut and removed 1 cm from left-side edge of the ring. A 3-mm defect was found in the atrial septum adjacent to the tricuspid annulus. The defect was repaired with a pledgeted mattress suture of 2-0 polyester (Nespolene, Alfresa Pharma Co., Tokyo, Japan) by passing the needle from the right ventricle to the right atrium posterior to the tricuspid valve. In addition, the uncovered cut edge of the TAP ring was covered with a pledgeted mattress suture of 4-0 polypropylene (Prolene™, Ethicon Endo-Surgery, Inc., Blue Ash, OH, USA). The remaining section of the ring was left in place. Intraoperative transesophageal echocardiography showed no residual LV-RA shunt. The patient has been asymptomatic for more than 10 months after the operation, with trivial tricuspid valve regurgitation and no signs of residual left-to-right shunt or haemolysis.
LV-RA communication has been noted to occur rarely after aortic or mitral valve replacement
[-]. While these procedures, extended debridement of annular calcifications in the posteromedial commissure and its vicinity may lead to membranous septum injury and LV-RA communication
[,]. However, no annular debridement in these areas had been performed in our patient. Furthermore, the location of the communication in this case differed from that of LV-RA communication following mitral surgery, in which case the location of the defect is usually at the base of the septal tricuspid leaflet near the coronary sinus.
Iatrogenic LV-RA communication caused by TAP suturing has been also reported, but it is extremely rare
[,]. Anatomically, the line of attachment of the septal leaflet of the tricuspid valve crosses the membranous septum, dividing it into an anterior interventricular portion, which lies between the ventricles, and a posterior atrioventricular portion, which lies between the left ventricle and right atrium. In addition, the position of the mitral annulus is slightly more cephalad than that of the tricuspid valve. Trauma to the membranous septum could therefore result in either a ventricular septal defect or an LV-RA communication. According to Aoyagi et al., if a mattress suture for TAP is inserted into the atrioventricular portion of the membranous septum rather than the tricuspid annulus at the anteroseptal commissure of the tricuspid valve, dehiscence of the ring and creation of a tear of the atrioventricular membranous septum may occur
[]. We speculated that a similar phenomenon had occurred in our case. Interestingly, all previously reported LV-RA communication after TAP, including the present case, used a rigid ring
[,], suggesting that strong tension around the tricuspid annulus associated with a rigid ring may lead to increased risk for LV-RA communication.


In June 2011, a 16-year-old male presented to The Second Affiliated Hospital, Zhejiang University School of Medicine (Hangzhou, China) with pain and swelling in the right zygomatic facial region for the previous two months. Upon physical examination, a 4.5×2.5-cm firm, fixed mass with a local sensation of warmth was identified in the right zygomatic facial region. Computed tomography (CT) of the head and neck revealed that the right maxillofacial tumor had caused cortical destruction of the wall of the right maxillary sinus and a sunburst-like periosteal reaction (). Furthermore, the soft tissue mass of the right maxillary sinus was found to be heterogeneous with a low-density necrotic area (). Magnetic resonance imaging (MRI) of the head and neck revealed a soft tissue mass arising from the right maxilla, which was occupying the right maxillary sinus. The solid section of the tumor was isointense to the normal muscle on the T1-weighted images (T1WI; ) and heterogeneous hyperintense on the T2-WI (T2WI; ). On the contrast-enhanced T1WI, a marked heterogeneous enhancement with a necrotic area was identified following the intravenous administration of gadolinium (). An ultrasonography-guided percutaneous core needle biopsy using an 18G coaxial cutting needle was performed. The biopsy specimen showed poorly differentiated tumor cells with small, blue, round or oval nuclei and scant cytoplasm (). Cluster of differentiation (CD)99 (also termed Mic2), vimentin, neuron-specific enolase (NSE), glycoprotein hormones, α-polypeptide (CgA) and S-100 were positive. Creatine kinase (CK), epithelial membrane antigen, myogenin, myogenic differentiation D1, leucocyte common antigen (LCA), desmin and glial fibrillary acidic protein were negative. Based on these histopathological features and the immunohistochemical pattern, the tumor was diagnosed as a pPNET. With a diagnosis of localized pPNET of the right maxilla, the patient commenced treatment with multiagent combination chemotherapy followed by definitive radiation without en bloc resection. This procedure was not performed due to the unfavorable prognosis that is associated with the disease even following en bloc resection. In addition, the extensive cosmetic and functional destruction resulting from resection of the involved section of the maxilla is considered to be unacceptable. The patient demonstrated remission during the 21-month follow-up period.


In October 2011, a 16-year-old male was transferred to The Second Affiliated Hospital, Zhejiang University School of Medicine with progressive painless swelling of the right mandible, a sensation of numbness and difficulty opening the mouth for one month. The patient was initially admitted to a local hospital with facial cellulitis due to the patient’s prolonged problem with dental caries and swelling of the right side of the chin. The patient was treated with empirical intravenous antibiotic treatment for a presumed infection of dental origin. However, the painless facial swelling became aggravated and the seventh and eighth right mandibular molars were subsequently extracted. The painless facial swelling continued for approximately one week, following which the patient was transferred to The Second Affiliated Hospital, Zhejiang University School of Medicine. Upon examination, the patient showed a firm non-tender fixed mass (size, 6.9×6.9 cm) with a local sensation of warmth on the right lateral aspect of the chin, wrapped around the angle of the mandible. A sensation of numbness was identified in the right lower lip and in the skin of the chin. Following admission to hospital, further imaging analyses of the head and neck were performed. MRI of the head and neck revealed a soft tissue mass arising from the right mandibular ramus, which was occupying the right masseter compartment. The tumor was isointense to the normal muscle on the T1WI () and hyperintense on the T2WI (). On the contrast-enhanced T1WI, the mass enhanced heterogeneously following the intravenous administration of gadolinium (). Bony cortex erosion and the involvement of the right mandibular canal were also evident, as well as bone marrow replacement, which was observed with isointense signal change on the T1WI and the T2WI. CT of the head and neck revealed cortical destruction and a sunburst-like periosteal reaction of the right mandibular ramus ().
An ultrasonography-guided percutaneous core needle biopsy using an 18G coaxial cutting needle was performed. Histopathologic analysis of the biopsy specimen showed small cells with a diffuse distribution, which occupied the majority of the view (). CD99, synaptophysin, NSE and CgA were identified to be positive. CK, S-100, LCA and desmin were negative. Based on these histopathological features and the immunohistochemical pattern, the tumor was diagnosed as a pPNET. Oral surgeons were consulted for en bloc resection; however, the tumor was unresectable as it involved the right mandible, parapharyngeal space and right masseter. En bloc resection of the involved portion of the right mandibular ramus would have caused unacceptable extensive cosmetic and functional destruction. Therefore, the patient was transferred to the radiotherapy department for concurrent chemotherapy and radiotherapy. After three cycles of systemic vein chemotherapy, followed by local treatment in the form of radiation therapy, the right mandibular tumor shrunk significantly. The patient received a further three cycles of treatment of systemic vein chemotherapy for nearly two months. Despite the treatment, the patient exhibited skull and meninx metastases and succumbed 18 months after diagnosis.


A 46-year-old male with a 20-year history of gout presented with nontender nodules in the subcutaneous tissue of the abdomen, groin, and right thigh. The nodules had been present for two years; however, over the past three weeks, some of the nodules became erythematous and eventually ulcerated. In addition, the patient had recently been admitted for congestive heart failure and acute renal failure 3.5 weeks prior to presentation. His gout had never been treated. Furthermore, the patient's medical history was complicated by type II diabetes and morbid obesity with a BMI of 40.6.
Physical examination revealed multiple, whitish subcutaneous nodules and 4 skin ulcers in the lower half of the abdomen, right groin, and right thigh. All ulcers measured between 1 and 3 cm; fibrinous, necrotic, and chalky whitish material covered poorly formed granulation tissue. The nonulcerated pale, white nodules with focal erythema could be palpated subcutaneously and averaged from 1.0 to 1.5 cm in diameter (). Clinically, there was also significant swelling of the right elbow and the MCP joints on the patient's right hand.
Results of laboratory tests from the patient's most recent admission revealed the following: uric acid 14.3 mg/dL (reference range 4–7 mg/dL), blood glucose 255 mg/dL (reference range 70–99 mg/dL), BUN 47 mg/dL (reference range 5–20 mg/dL), creatinine 1.61 mg/dL (reference range 0.8–1.4 mg/dL), and estimated GFR 46 mL/min/1.73 m2 (reference range ≥59). His uric acid levels were 10.8 mg/dL and 11.2 mg/dL at the time of surgical removal of the ulcerated nodules, 3 and 4 weeks later. At that time also, his renal function and blood glucose levels were still abnormal: BUN 38 mg/dL, creatinine 1.65 mg/dL, GFR 45 mL/min/1.73 m2, and blood glucose 147 mg/dL.
The patient underwent 3 excisional biopsies, including 2 from an abdominal skin lesion and 1 from the right thigh. All 3 specimens were submitted separately, over a 3-week period, for histological evaluation. Sectioning of the specimens revealed multifocal areas filled with white, chalky material. Touch imprint slides were prepared before tissue fixation. Microscopic examination under polarized light showed multiple needle-shaped crystals displaying negative birefringence (). Permanent histology slides showed pools of pale and slightly eosinophilic amorphous as well as feathery material in the dermis and subcutaneous tissue (Figures and ). The lesions demonstrated different stages of development. The smaller lesions had a central area of feathery crystalline material surrounded by palisading histiocytes and multinucleated giant cells. The larger lesions consisted of disorganized, laminated material surrounded by collagen fibers and chronic inflammation. The lesions eroded outward to the skin surface leading to ulceration. Based on the clinical, laboratory, and histologic findings, a diagnosis of multifocal gouty tophi was made.


A 59-year-old female initially presented to the neurosurgery clinic with complaints of insidious onset of right leg radicular pain. She has no history of numbness, tingling, weakness, or bowel or bladder incontinency. She has never experienced any similar pain episodes. Physical examination had significant right side straight leg test positive at 45 degrees and left lower extremity at 70 degrees. Plain lumbosacral X-ray was inconclusive. The patient's lumbar MRI reported right L4-L5 lateral disc herniation with extrusions and the patient had partial response to conservative management.
She underwent a minimally invasive hemilaminotomy of right side L4-L5 and removal of the extruded disc particles. The patient had an uneventful postoperative course. She had immediate pain relief after surgery and was able to ambulate on postoperative day one. She was discharged from the hospital the following day after surgery.
The patient reported a new pain experience on the left buttock that started insidiously two weeks after discharge. The pain was constant burning at the left pelvic region, with sharp shooting pain in the left leg. The patient reported pain radiating in multiple trajectories but on clinical assessment had no specific dermatomal distribution. Pain was not aggravated by coughing or sneezing but is more during a sitting position.
On a new physical examination, a healed surgical scar of posterior approach was seen over the midline lower lumbar area. There was no sign of infection at the surgical scar. The patient walked with an antalgic and waddling gait. There was a significant Tinel sign on the left gluteal region over sciatic nerve. Straight leg rising test was negative in supine, sitting, and prone position. Detailed physical examination included spine mobility with great range of motion with no pain, manual muscle testing with normal muscle strength, and deep tendon reflexes were normal. Piriformis stretch test was significantly positive and could aggravate the patient's pain.
The patient had an MRI of lumbosacral, which showed postoperative changes with no disc herniations at the lower lumbar spine.
Throughout the patient's multiple pain clinic visits, we began a multimodality treatment, which included antiepileptic and antidepressant antalgic medication management, muscle relaxant, short acting narcotic medication, and physical therapy.
We decided to perform a diagnostic piriformis muscle block, when the combination of muscle relaxants, antidepressants, and antiepileptic medications did not provide significant relief.
The patient had a piriformis muscle injection under ultrasound guidance where 5 cc of 1% lidocaine solution was injected in the targeted muscle. The patient demonstrated immediate pain relief.
Repeated piriformis muscle injection was able to provide short course pain relief. We decided to do a pelvic MRI to visualize suspected pathologies. An MRI of the pelvis, without contrast, including coronal T1, STIR, axial T1, T2 fat-sat, and STIR sequences, was done. MRI imaging revealed left side sciatic neuritis (see ).
With the diagnosis of sciatic neuritis at the left pelvic region, we continued on our multidisciplinary aggressive pain management with a reasonable time frame. The patient was very motivated and followed all steps of the recommendations with significant efforts. She did not respond to the treatment plan after four months of continued aggressive management. We decided to proceed to neuromodulation modality by placing a temporary percutaneous thoracic spinal cord stimulator (SCS). The patient reported a complete response with almost 100% pain relief during one week of the SCS trial. We were able to decrease the patient's medications dramatically during the trial period. The patient then underwent a permanent implant of SCS. On a one-year followup, the patient had remained pain-free without using medication.


A 73-year-old Hispanic male with a history of coronary artery disease, hypothyroidism, and arthritis underwent a CT scan of the abdomen and pelvis for an unrelated complaint and was incidentally found to have a hypervascular left renal mass suspicious for malignancy. He was referred to an outpatient percutaneous transcatheter renal artery embolization, in preparation for a subsequent radical left nephrectomy with a view to decrease intraoperative bleeding at surgery. Physical examination prior to the procedure was essentially normal and did not reveal any cardiopulmonary abnormalities. The patient received intravenous conscious sedation over a period of 60 minutes consisting of 100 mcg fentanyl and 1.0 mg midazolam, administered in intermittent doses, while undergoing continuous physiologic monitoring. Per protocol [], the procedure was performed using a total of 40 mL of pure ethanol divided into three doses and administered in slow pushes at 15-minute intervals. This dose was derived based on a weight of roughly 80 kg, using the lower end of the recommended dose of 0.5–1 mL/kg. The result was a significant reduction in tumor vascularity, with approximately 10% residual renal artery tumor perfusion.
After the third ethanol injection, the patient developed cough, agitation, and acute respiratory distress with hypoxemia (SpO2 of 80%; normal >92%). He required sedation and emergent intubation for airway protection. Given the concern for pulmonary embolism, pulmonary angiography was immediately performed and showed patent pulmonary arteries, without central or segmental pulmonary artery embolus or vessel occlusion. The procedure confirmed the presence of PAH with a mean pulmonary artery pressure (mPAP) of 70 mmHg (normal <25 mmHg). Acute pulmonary vasospasm due to venous shunting from the systemic intra-arterial ethanol administration was suspected. A Fogarty balloon was inflated in the renal artery to prevent further dissemination of ethanol into the systemic circulation. Nitroglycerin was administered directly into the pulmonary artery circulation at a rate of 5 mcg/min with a total dose of 1400 mcg; this resulted in reduction of mPAP from 70 mmHg to 30 mmHg. A pulmonary artery catheter was left in place for continued pulmonary artery pressure monitoring.
The patient was admitted to the intensive care unit for close monitoring. He was started on intravenous dexmedetomidine for sedation and analgesia. On physical examination, his vital signs were within normal limits, with no focal findings. Laboratory workup revealed mild leukocytosis (white blood cell count 11.4 k/mm cu; normal 4–11 k/mm cu), anemia (hemoglobin 8.3 g/dL; normal 13.5–17 g/dL), and elevated blood alcohol level (80 mg/dL; normal 0–20 mg/dL). There was no evidence of hemolysis, as the hemoglobin level was close to baseline and the bilirubin was normal. Electrocardiogram and cardiac enzymes were negative for acute ischemia; chest X-ray (CXR) did not show any pleural effusion, consolidation, or acute pathology; transthoracic echocardiogram (TTE) showed normal cardiac function; lower extremity ultrasound Doppler ruled out deep venous thrombosis (DVT), and recent pulmonary functions tests (PFT) showed no abnormalities in baseline respiratory function. Arterial blood gases were within normal limits and the patient was successfully weaned and extubated the following day. Pulmonary artery pressure throughout his stay stabilized at a mean of 30 mmHg before the pulmonary artery catheter was discontinued. His respiratory status remained stable and he was gradually weaned off supplemental oxygen altogether. There was no evidence of new cardiopulmonary abnormalities at the time of discharge.


A 28-year-old Chinese male presented to our clinic with a 3-year history of condylomata acuminata of the penis. His lesions had been previously treated several times with liquid nitrogen and electrodessication without causing any pigmentary changes, but his problem had relapsed half a month before presentation and now he was presenting with five new warts. The patient was treated with electrodessication to remove his condylomata acuminata. When his wound healed 12 days later, he was advised to use imiquimod 5% cream for relapse prevention. He applied the cream for three nights weekly and washed it off in the morning. Before long, he noticed some irritation from erythema and excoriation in the treated areas, but he persisted in the application. After about 12 weeks of continuous use, he again presented to the clinic complaining of vitiligo-like depigmentation of the macules on his penis. He was instructed to stop using the imiquimod, but the macules in the treated areas gradually enlarged and asymptomatically merged; a vitiligo-like, depigmented patch also appeared on his scrotum during the following 10 days. After 4 weeks of applying tacrolimus 0.1% ointment, the lesions became slightly repigmented, and the area of vitiligo lesions stabilized. Unfortunately, no further repigmentation occurred after 3 months of follow-up.Laboratory examination and a skin biopsy were recommended. He and his family members had no history of vitiligo, other depigmented dermatoses, or autoimmune disorders. He denied use of any other topical treatment. Physical examination showed vitiligo patches involving the glans penis, the shaft of the penis, and the scrotum, along with some remaining pigmented areas within the vitiligo plaques (Figure
). Wood’s light accentuated the depigmented areas. He was not tired or irritable. EKG, chest X-ray, and thyroid and abdominal ultrasonic scans indicated no cardiac, pulmonary, bilateral thyroid, hepatic, splenic, nephritic, or other organ involvement.
Additional laboratory analysis revealed normal counts of white blood cells, red blood cells and blood platelets; normal elevated erythrocyte sedimentation rate; normal thyroid function tests; negative antistreptolysin O titer and rheumatoid factor; normal serum immunoglobulins G, M, and A, E levels; normal liver enzyme level; normal blood urea nitrogen and creatinine level; normal blood glucose level; negative antinuclear and antineutrophil cytoplasmic antibodies; negative HIV and syphilis antibodies; normal hepatitis A, B, and C serology; normal urine and stool analysis.A skin biopsy was performed on the dorsal surface of the penis, which showed a complete absence of melanocytes and melanin granules in the basal layer but with a normal dermis (Figure
). Loss of melanocytes and melanin granules in the epidermis was highlighted by Fontana-Masson staining (Figure
). Melanocytes and melanin granules exist in the epidermal basal-cell layer of normal skin (Figure
). He was diagnosed, clinically and pathologically, with imiquimod-induced localized vitiligo.


A 3.5 years old female child was brought to department of oral medicine and radiology with a complaint of mobility of right lower back tooth of 1 week duration. There was no history of toothache, trauma or associated symptoms. Extraoral examination revealed an irregular lobulated, nontender bony hard swelling of size 3 × 3.5 cm with uniformly blending borders on buccal aspect of right side body and angle of mandible (). Lower border of the mandible showed a discontinuity without considerable expansion. It extends to midline through submandibular region with a size of 4 × 5 cm. Local rise in temperature noted on overlying skin and is not fixed to swelling.
Intraorally, right lower gingivobuccal sulcus and floor of mouth obliterated due to swelling from 84 region to retromolar region (). An irregular lobulated swelling of variable consistency with bilateral cortical plate expansion was seen on premolar-molar region. Hard tissue examination showed full complement of healthy teeth except grade III mobile nontender, 85.
Occlusal and panoramic radiographs revealed ill-defined lytic lesions involving premolar-molar region of right side body of mandible, extending from 83 to angle of mandible. Cortex and periosteum was characterized by erosion, thinning and discontinuity on buccal cortex; radiating spicules on lingual cortex; irregular thinning of lower cortical plate seen without considerable expansion. Multiple irregular patchy radiolucent areas in internal structure of permeative and moth eaten pattern with size varying from 0.5 to 2 cm was noted in internal structure (). Considerable root resorption was seen on 84, 85. Other effects on surrounding structures include posterior superior displacement and mesial tipping of developed crown of dental follicles of 46, 47 and missing dental follicle of 45. Loss of alveolar canal outlines medial to angle of mandible (). Computed tomographic (CT) axial section showed multilocular expansile lytic lesion in body of mandible right side with significant enhancing soft tissue matrix ().
Trucut needle biopsy showed spicules of bone with a cellular cytoplasm composed of round cells with scanty cytoplasm and pleomorphic round or oval nuclei in sheets and sinusoidal pattern (). Immunohistochemistry showed diffuse strong membrane positivity for MIC2 (), focal positivity for synaptophysin and negativity for desmin and diagnosis came compatible with ES or primitive neuroectodermal tumor (PNET).
Patient underwent radiotherapy and adjuvant chemotherapy treatment with vincristine, cyclophosphamide, etoposide and mesna in Regional Cancer Centre, Thiruvananthapuram, Kerala. One year follow-up clinical review showed bony hard nontender diffuse expansion of right side mandible without apparent soft tissue swelling extraorally (). Intraorally missing 85 was noted due to exfoliation, 6 months after onset of treatment ().
Radiological follow-up using occlusal and panoramic radiography after 1 year of treatment showed mixed radiodensity on premolar-molar region and anterior border of ramus of right side of mandible involving 82 to 47. Intact and visible uniform expansion with cortical regeneration was seen throughout the lower border of the mandible near the lesion. Cortical break in buccal aspect remodeled with overzealous cancellous bone apposition resulted in an increased buccolingual width. Periosteal reactions including vertical spiculations completely resolved and cortical out line re-established in lingual aspect (). Considerable reduction in permeative and moth eaten pattern and replacement with accentuated multiple linear and granular trabeculae with a multiseptated pattern was noted in internal structure. Enamel formation completed and dentine formation started 44 and 47. Relative developmental delay was observed on 46 (). Radiological features suggestive of cessation of malignant tissue growth and active destruction, followed by vigorous regeneration and reparative reactions of healthy osseous and dental tissues and arrested growth of dental follicle near epicenter of the lesion.


A 56-year-old African American man who initially presented with diarrhea, weight loss and painless jaundice, was subsequently found to have adenocarcinoma of the head of pancreas. An initial workup revealed localized cancer with no evidence of distant metastases. He then underwent pancreaticoduodenectomy with complete surgical resection of a 5cm moderately differentiated pancreatic adenocarcinoma. Although our patient underwent complete surgical resection, a pathological examination revealed a neoplastic invasion of the resected adjacent organs, and one out of the seven resected lymph nodes contained cancer (T3N1M0). At that point, the oncology department recommended to proceed with adjuvant chemotherapy with gemcitabine (1000mg/m2 IV on days 1, 8, 15 on a 28-day cycle for six cycles) to try to reduce the likelihood of recurrence.
However, after completing two cycles (a total of six doses) of chemotherapy, he presented to the emergency department with worsening exertional dyspnea, three-pillow orthopnea, paroxysmal nocturnal dyspnea and fatigue. His physical examination revealed an elevated jugular venous pressure (JVP) (10cm above the sternal angle), bibasilar rales and +2 pitting edema of both lower extremities. Cardiac auscultation revealed a gallop rhythm with an S3 and a grade 3 holosystolic murmur over precordium. A chest X-ray showed cardiomegaly with mild to moderate-sized right-sided pleural effusion. It was thought that his presentation was consistent with fluid overload secondary to congestive heart failure (CHF) and he was started on intravenous (IV) furosemide with partial improvement in his symptoms.
The next day, a two-dimensional echocardiography (2D Echo) was performed, which showed left ventricular ejection fraction (LVEF) of 15 to 20 percent with global hypokinesia along with moderate mitral regurgitation. Given the findings of 2D Echo and the absence of significant risk factors for coronary artery disease (CAD) and ischemic cardiomyopathy (CMP), it was concluded that our patient’s CMP was related to the recent use of gemcitabine. Our patient was then started on carvedilol and an angiotensin-converting enzyme inhibitor in addition to diuretics and he was discharged from the hospital two days later in a euvolemic state.
At that point, the cardiology department recommended stopping further chemotherapy with gemcitabine. The oncology department advised further testing to rule out ischemia as a cause of CMP as, in their opinion, chemotherapy with gemcitabine was the only option to reduce the risk of recurrence in this patient. Two weeks later, our patient underwent myocardial perfusion imaging (MPI), which showed a fixed small- to moderate-sized inferior wall defect without any evidence of active ischemia. The ejection fraction (EF) on MPI was calculated to be around 17 to 20 percent with severe global hypokinesia. Our patient was continued on standard heart failure therapy with one more admission to the hospital for CHF exacerbation about two months later. He responded well to IV furosemide and adjustment of heart failure therapy. A 2D Echo was repeated a few months later and it showed improvement in systolic function with an LVEF of 40 percent.
Due to his poor functional status and underlying CMP, further gemcitabine chemotherapy was stopped. Later, our patient developed a recurrence of his pancreatic cancer; he refused further chemotherapy and decided to proceed with palliative care.
Although the exact etiology of our patient’s dilated cardiomyopathy remains unclear, gemcitabine remains the most likely culprit. The temporal relationship of his symptoms to the initiation of gemcitabine chemotherapy; the lack of risk factors for ischemic CMP and prior history of CAD, finding of global hypokinesia on 2D Echo, absence of ischemia on MPI; and improvement in his systolic function after discontinuation of gemcitabine were all consistent with gemcitabine-induced cardiomyopathy.


A 45-year-old Turkish woman was admitted to our emergency department with complaints of abdominal pain, constipation, nausea and vomiting. She had undergone abdominal radical hysterectomy for cervical carcinoma three years earlier. A physical examination revealed a serious abdominal distention, generalized abdominal tenderness and a rebound sign with a metallic bowel sound. Her blood pressure was 70/40mmHg, with a heart rate of 118 beats/min, a body temperature of 38.2°C and oxygen saturation of 92%. Laboratory tests showed a raised white cell count of 29.0×103/mmc, and a hemoglobin value of 14.0g/dL with a hematocrit level of 41.1%. The positive laboratory findings were C-reactive protein (CRP): 289mg/L, blood urea nitrogen (BUN): 56mg/dL, creatinine (Cr): 2.36mg/dL, sodium (Na):112mmol/L, potassium (K):2.7mmol/L. The arterial blood gas analysis revealed a lactic metabolic acidosis with a pH value of 6.99 and lactate level of 6.7mmol/L. In the historical examination, she had not been able to pass either gas or stool for three days. In her medical history, she had been admitted to the emergency room (ER) with a complaint of lower left abdominal quadrant pain but she had been discharged with a urinary tract infection diagnosis based on a urinalysis, which revealed pyuria with 56 leucocytes per mm3. An abdominal ultrasonography (US) scan was performed, followed by a computed tomography (CT) scan of her abdomen/pelvis. The abdominal US scan detected a swollen and immobile small bowel. The CT scan was performed for a better evaluation of our patient’s symptoms and confirmed the suspicion of mechanic bowel obstruction. The CT scan revealed intestinal dilatation, a large amount of free fluid in the abdominal cavity and an area suspicious for jejunal perforation. Moreover, the CT scan showed a group of poorly enhanced and thickened bowel walls (Figure ).
An aggressive fluid-electrolyte resuscitation (20ml/kg/hr) and antibiotics (ceftriaxone 2 gr/ 24h and metronidazole 500mg/6h intravenous) administration was started immediately in the intensive care unit. Because these radiological findings were suggestive for obstruction and bowel ischemia, our patient underwent emergency surgery six hours after admission.The prior midline incision was used to inspect the abdomen. The small intestines were dilated and there was a large amount of an exudative fluid in the abdomen. A jejunal segment was turned around a fibrous band and the surgeons realized that the ‘band’ was the right ureter (Figure ). A 45cm jejenum resection and side-to-side jejunojejunostomy was performed. A suction drain was placed into the Douglas cavity (Figure ).
She stayed in the intensive care unit until the postoperative second day. On the fourth day, she passed gas and started oral feeding with a high-calorie and high-protein diet. On the seventh day, she was given a normal diet. She developed a high body temperature (38.9°C). An abdominal CT scan was performed and a small amount of free fluid collection was detected between the small intestine loops. The fluid was aspirated at the interventional radiology unit. The fluid was clear and no microorganisms could be isolated in the fluid. She was discharged on the tenth postoperative day with normal oral feeding and excellent mobilization.


An 11-year-old prepubertal Caucasian girl was admitted to our emergency room with complaints of abdominal pain in the right lower quadrant, as well as nausea and vomiting. Her symptoms had started 7 days prior to her visit, but her pain had intensified the evening before she presented to our emergency room. She described her pain as moderate and colicky in nature.
Upon presentation, she was found to be afebrile, and her laboratory work-up revealed that her white blood cell count was 11,500/ml with 84% neutrophils. The other laboratory examination findings were within the reference ranges. During her physical examination, we detected right lower-quadrant pain without abdominal guarding. An ultrasound examination revealed a hypoechogenic cyst in the right side of the pouch of Douglas measuring 9cm in diameter. Doppler ultrasonography revealed blood flow on the edges of the structure and absence in the central part. The finding was misinterpreted as torsion of the cystically altered right ovary.After a Pfannenstiel incision was made, surgical findings revealed that the uterus, right and left round ligaments, ovaries, cecum and appendix were all normal. The right fallopian tube was twisted and large, distended, cystic, convoluted and filled with fluid that was tapering as it approached the ampular tube. The remaining proximal portion of the tube appeared viable. The contralateral fallopian tube was normal. After we performed a meticulous detorsion (Figure ), the viable part of the fallopian tube and the cystic, convoluted portion of the hydrosalpinx were clearly exposed (Figure ). We resected the cystically altered fimbrial part of the fallopian tube with a stainless steel probe and assessed the conductivity of the remnant, which consisted of the ampullary and isthmic parts of the tube (Figure ), and then we placed a nylon suture which anchored the tube orifice and thus maintained its permeability. Subsequently, we created a neostomy with five radially placed 6-0 Vicryl sutures (Figure ). The right ovary, uterus and left adnexa were left unchanged.
The pathologic findings confirmed that the excised structure was a cystically altered fallopian tube with a thinned wall and preserved parietal cells. The post-operative period was uneventful. The patient was discharged on the third post-operative day. She is in being followed closely in regular monthly post-period intervals by our gynecologist according to the protocol at our institution.


A twenty-two years old girl presented with gradually increasing left-sided neck swelling for 2 months. FNAC from the lymph node revealed necrosis only, without any AFB. Despite that picture, family physician started antitubercular treatment with 2HREZ/4HR. When we examined, the swelling was tender, mobile, without any sinus or fever. Clinical examination did not reveal any abnormality. Her routine blood examination, chest x-ray, mantoux test, HIV reports, and connective tissue profile were normal. Excision biopsy of the left cervical lymph node revealed lymphoid follicles with germinal centres. Areas of necrosis containing karyorrhectic debris were present. Necrotic areas were surrounded by histiocytes, lymphocytosis, and plasma cells. Entire picture is suggestive of Kikuchi's disease (). Z-N stain and Mycobacterium tuberculosis culture (MGIT 960) of the biopsy tissue were negative. The patient was better without any specific therapy.


A 71-year-old man developed progressive dyspnea, orthopnea, general malaise, and edema in both the lower limbs. He was admitted to a local hospital for congestive heart failure, categorized as New York Heart Association functional class III, and atrial fibrillation with a rapid ventricular response. He had no history of hypertension, diabetes, or familial cardiac disease. An electrocardiogram showed atrial fibrillation with a rapid ventricular response and a complete right bundle branch block (). Low voltage, a typical finding in cardiac amyloidosis, was not observed. Laboratory findings revealed highly elevated levels of B-type natriuretic peptide (1272.6 pg/mL; normal range, <18.4 pg/mL) and troponin I (0.491 ng/mL; normal range, <0.045 ng/mL). The white blood cell (WBC) count was 5,830/μL, with 1.5% eosinophils, and the C-reactive protein level was 0.48 mg/dL. Transthoracic echocardiography revealed an extensive, thin-walled akinetic region and thrombus at the apex, hypokinesis of the basal and midportion of the LV, and a decreased LV ejection fraction of 37% (). The LV diastolic and systolic diameters, LV posterior and septal wall thicknesses, and right ventricle (RV) free wall thickness were 49 mm, 39 mm, 12 mm, 17 mm, and 10 mm, respectively. There was no evidence of left ventricular outflow tract obstruction, systolic anterior motion of the mitral valve, or aortic valve disease.
The long-axis (), four-chamber (), and short axis views () of the CMR images revealed a thin-walled, apical aneurysm, with subendocardial LGE in an extensive area of the mid-to-apex region of the LV and a smaller area in the RV. The LGE involved the majority of the basal lateral wall. Smaller, midmyocardial LGE was observed in the basal interventricular septum, of which the wall was markedly thickened.
Regional subendocardial fibrosis is frequently observed in patients with subendocardial infarction. However, in the present case, coronary angiography did not show coronary stenosis or myocardial bridging (). Three endomyocardial biopsy specimens were collected from the right ventricular septum and indicated that, in each sample, the thickened fibrous endomyocardium extended through all layers (). We considered performing a left ventricular examination, but an apical thrombus in the LV was recognized as the source of embolic material and left ventricular biopsy and pressure measurement were not performed. The patient was treated with diuretics, bisoprolol, losartan, warfarin, and spironolactone, resulting in improvements in the heart failure symptoms.


A 23-year-old woman presented to our hospital with a two-day history of fevers and vomiting, epigastric pain, malaise, and headache. Her past history included polycystic ovaries, gastritis with previous helicobacter eradication, and hypothyroidism. She had recently travelled to her country of birth, Pakistan, for eight weeks and had returned to Australia two days prior to admission. Two weeks after arriving in Pakistan, she developed nausea, vomiting, headache, anorexia, and myalgia. Abdominal ultrasound was normal and barium swallow demonstrated gastritis. She was managed with esomeprazole, zinc, and atorvastatin. On returning to Australia, in addition to ongoing nausea and headache, she developed fevers and epigastric pain. On presentation to our metropolitan hospital she was febrile with 39.9°C, pulse rate 96/min, blood pressure 108/72 mmHg, and respiratory rate 20/min. Physical exam revealed a soft abdomen with mild suprapubic tenderness. Rose spots were present on her anterior chest and no jaundice was noted. Her white cell count was normal, platelet count was mildly low, her C-reactive protein elevated, and she had mild transaminitis (see ).
No abnormalities were detected on chest X-ray. The blood cultures grew Salmonella Typhi with resistance to ciprofloxacin and sensitivity to amoxicillin, ceftriaxone, and cotrimoxazole. Investigations for malaria, HIV, dengue, and viral hepatitis were all negative. Stool cultures were negative.
She was treated with intravenous ceftriaxone 2 g daily. On the third day of her admission she developed severe right upper quadrant pain with localised peritonism despite her fever settling. Her LFTs were further deranged and abdominal ultrasound showed thickening of the gallbladder with pericholecystic fluid and probe tenderness, consistent with acalculous cholecystitis (see ).
She underwent laparoscopic cholecystectomy on the dame day. During the operation, a swollen, oedematous, and nonperforated gall bladder without stones was noted. The liver was noted to be normal, and there was a small amount of free fluid in the pelvis. Culture of the bile was not performed.
The pathology of the resected gall bladder revealed chronic inflammatory cell infiltration and mild chronic cholecystitis (see Figures and ).
She became febrile two days after the operation (temperature 38.8°C). Intravenous azithromycin was added to the antibiotic regime. A CT abdomen was unremarkable, and a chest X-ray revealed right lower lobe atelectasis. Intravenous meropenem was subsequently administered for the following two days. Her fever settled and she was discharged with oral azithromycin on day 10 of her admission.


The patient is a 37 year old woman, born in Austria. She had two children (13 and 15 years) before her GBM diagnosis. She was diagnosed with GBM in the right frontal lobe in April 2008, and treated according to a current treatment standard consisting of a gross total resection of the tumor, fractionated confocal radiotherapy up to 60 Gy (2 Gy/fraction) and concomitant and adjuvant chemotherapy with temozolomide
. Diagnosis and treatment were performed at the Medical University of Vienna (MUV). Following the concomitant therapy, she experienced amenorrhea for 6 months, followed by irregular menstrual cycles with oligomenorrhea. Two years later, a local recurrence of GBM was diagnosed. She underwent a second resection and followed 6 cycles of dose dense temozolomide, 100 mg/m
2 orally for five days per week, with a drug holiday over every weekend. She became pregnant less than three weeks after the last intake of the sixth cycle of temozolomide, after a total dose of temozolomide of 20.9 g/m
2 (
).
Both parents wanted to carry the child to term. They were offered intensified pregnancy monitoring and genetic counselling, but no genetic tests were performed. During pregnancy the child developed normally as followed by close meshed ultrasound controls and the mother was doing well until week 27, when she developed signs of increased intracranial pressure as well as weakness of the left leg. She was admitted to the local general hospital for observance and anti edematous treatment. As the mother’s condition worsened, she received corticosteroids to induce lung maturation of the fetus, and the child was delivered after 32 weeks and 6 days of pregnancy by caesarean section using the Misgav Ladach method
. The child showed Apgar scores of 8/9/9, one, five and ten minutes after birth, respectively, weighed 1876 g, cried spontaneously and was neurologically inconspicuous. The infant did not need further respiratory assistance after the third day of life, and was discharged from the neonatal intensive care unit after an uncomplicated stay without any signs of neurological or any other organ deficit, weighing 2.5 kg. The child has shown normal growth and development ever since.
The mother underwent her third neurosurgical procedure two weeks after delivery, followed by chemotherapy with fotemustine 100 mg/m
2 every three weeks for six cycles combined with bevacizumab 10 mg/kg every two weeks 6 weeks later. Eight months after the caesarean section, distal progression in the brain was diagnosed on MRI and she was referred for radiotherapy of the distant recurrence.


A previously normotensive 9-year-old boy was admitted to the Pediatric Emergency Department with bilateral periorbital edema and gross hematuria for 24 hours.
20 days before, he had experienced an episode of mild sore throat spontaneously recovered.
On admission general conditions, neurological and visual examinations were normal except for periorbital edemas. Axillary temperature was 36.9°C, oxygen saturation 98%, heart rate 88 beats per minute, body weight 35 kg (two more than the previous week), and body height 1.350 m. Blood pressure was increased with systolic values ranging between 125 and 130 mmHg and diastolic values ranging between 80 and 85 mmHg (stage 1 hypertension: 95th percentile to the 99th percentile for gender, age, and height plus 5 mmHg
[]). Blood and urine investigations were consistent for acute renal insufficiency due to a nephritic-nephrotic syndrome (Table
).
Since the 2nd day of hospitalization, the patient was treated with one bolus of methylprednisolone (15 mg/Kg) per day associated with strict blood pressure monitoring.
On the 4th day of hospitalization, the patient developed severe headache associated with increased blood pressure up to 150/120 mmHg (stage 2 hypertension: > 99th percentile for gender, age and height plus 5 mmHg
[]). Alterations of conscious level, visual symptoms and vomiting were not present. Furthermore, cognitive dysfunctions, visual field defects, sensory abnormalities and ataxia were absent on the neurological examination.A brain MRI showed in fluid attenuated inversion recovery imaging (T2 coronal) high signal intensities in cerebellar white matter (Figure
A and C) and in parasagittal subcortical parietal regions (Figure
A and C). Diffusion weighted images did not show any restriction of diffusivity.
Methylprednisolone was discontinued. Candesartan was initiated and on 6th day of hospitalization, blood pressure normalized and both headache and periorbital edemas resolved. On 9th day of hospitalization, a control MRI showed complete regression of all the abnormalities (Figures
B, D and
B, D). Arterial hypertension, MRI findings and clinical outcome were consistent with the diagnosis of PRES.
The child was discharged in good clinical condition without any further treatment.
3 weeks later, clinical examination was unremarkable and blood pressure 105/70 mmHg. Complement C3 and serum creatinine normalized and urinalysis revealed only persistent isolated microscopic hematuria (7 cells per high-power field).


A 51-year-old woman presented to our neurosurgery center with a two-month long blurry of the right eye, which gradually worsened, eventually losing vision in the eye 20 days before admission. A neurological examination revealed that the sight of the right eye was 0.03, with bilateral temporal hemianopsia. There were no other remarkable signs. An endocrinological evaluation showed only a low value of blood cortisol. T1-weighted (T1-w) MRI scans revealed an iso-signal intra-sella tumor sized 3.0 × 2.5 × 2.5 cm, close to the medial part of the right cavernous sinus, with distinctive partial enhancement after a gadolinium injection, which arose from the right cavernous sinus, flame-like in shape, with bush-like edge (Figure
B). T2-fluid-attenuated inversion recovery-weighted sequences (T2-FLAIR) showed a homogeneous hyper-signal tumor (Figure
A). The sella fossa was obviously enlarged and the tumor had invaded into the sphenoid sinus. It was considered to be a non-functional pituitary tumor.
A microscopic endonasal transsphenoidal approach was used for surgical resection. After dural opening, a solid, tenacious, purple mass was found, sticking firmly with surrounding structures, with abundant blood supply. The tumor was extremely difficult to manipulate and had to be excised en bloc to avoid bleeding. It was completely excised after hours of careful separation between the tumor capsule and the cavernous sinus. The removed tissue was dark purple, shrinking, sponge-like, and approximately 2.5 cm in size. After common hemostasis, the wound was sealed with gel foam and glue. Microscopically, the lesion was essentially composed of spindle shaped smooth muscle cells and blood vessels with thick walls devoid of elastic lamina (Figure
A). The vessel lumen was slit-like and the inner wall was lined with monolayer endothelial cells, and the smooth muscles of the vascular wall intermingled with the smooth muscle cells between the vessels. No mitoses were found. Immunohistochemistry revealed that the spindle cells were positive for vimentin (Vim) and smooth muscle actin (SMA) (Figure
B), and negative for glial fibrillary acidic protein (GFAP) and neurofilament (NF). The endothelial cells were positive for CD34. The tumor was identified as ALM. The patient had no complaints postoperatively, except for a slight headache. The sight of her right eye promoted obviously two days after surgery. An MRI scan demonstrated a total resection (Figure
C) without any bleeding. At 11 days after the operation, she complained of a decreasing of hearing in her left ear and a little nose discharge, although the headache diminished. An ear-nose-throat inspection was recommended. However, an emergent incident happened while she was under nasal endoscope, as the surgical area suffered a sudden high pressure expulsion of blood. After 10 minutes, 1200 ml blood had been lost and she lost consciousness due to acute shock. Her nasal cavity was packed with gauze and the bleeding temporarily stopped. A cerebral DSA (Digital Subtraction Angiography) was immediately performed. A pseudo-aneurysm of the left internal carotid artery (ICA) cavernous segment was revealed (Figure
A arrow). When we tried to embolize it, a second bleeding occurred (Figure
B), invading into cranial cavity, and she fell into deep coma. We immediately embolized the left ICA and performed vital resuscitation. Although supported by mechanical ventilation and circulation, she died one day later.


A 49-year-old man had a one-year history of weakness in the lower limbs, walking instability, sporadic falling down, dizziness, nausea, and vomiting. His neurological examination revealed a tongue slant to right, a positive Romberg sign, and muscle strength grade IV in both lower extremities.An MRI scan revealed a large sub-tentorium lesion in the posterior fossa. The mass was sized 4.2 × 4.6 × 5.7 cm, affixed to the straight sinus and torcular, compressing forward. There was great displacement of the cerebellum, brain stem, and the fourth ventricle. The cerebella tonsillar had herniated into the foramen magnum. Supra-tentorial ventricular dilation was presented due to obstructive hydrocephalus. A T1-w MRI showed a slightly hypo-signal homogeneous tumor (Figure
D), and T2-w and FLAIR(fluid-attenuated inversion recovery) scans revealed even hyper-signal. With gadolinium contrast, the lesion was obviously unevenly or partially enhanced, the enhancing was progressive from posterior to anterior, flame-like, with an irregular flocky twiggy edge, and the front part of the lesion was hardly enhanced (Figure
E). The differential diagnosis included hemangioblastoma and meningioma.
A midline suboccipital craniotomy was performed. The tumor was found to be affixed to the torcular and the posterior part of the straight sinus, closely adhered with the dura. It was a purple color, tough, had a clear boundary, and an abundant blood supply. The tumor was removed piece by piece under a microscope. Most of the lesion was freed from the dura, except a minimal piece that could not be removed safely from the torcular, therefore a subtotal resection was made. Approximately 620 ml blood was lost altogether. The pathology features mimic those in case 1 (Figure
C). The smooth muscle cells were positive for SMA (Figure
D) and Vim, and the inner-vessel endothelial cells were positive for CD34, negative for epithelial membrane antigen (EMA), and negative for inhibin-a. The patient recovered without incident and an MRI scan showed a satisfying resection (Figure
B). On a follow-up CT scan five months later and a further follow-up MRI scan one year later (Figure
F), no recurrence was demonstrated. The patient’s legs resumed normal strength and recovered well, except for some walking instability.


A 77-year-old man suffered from an intermittent headache for five months. His neurological physical examination was unremarkable. T1-w MRI scans revealed an iso-signal tumor arising from the diploic space of the left temporal skull vault, invading inward to temporal lobe, sized 1.6 × 3.1 × 3.9 cm. T2-w and FLAIR scans showed a hyper-signal lesion (Figure
G). Obvious enhancement was seen after a gadolinium injection (Figure
H). There was no enhancement of the dura or ‘dura tail’. The diploic angioma, meningioma, fibrosarcoma, and aneurysmal bone cyst were considered.
A craniotomy was performed. The cranial outer table was intact, the inner lamina and diploe were eroded with hyperostosis around. The tumor was a dark reddish hue with a rich blood supply, adhered to the dura. The whole tumor was removed. After autoclaving, the bone flap was returned and fixed in the original place. A histological analysis demonstrated typical elongated smooth muscle cells and dilated irregular vascular channels (Figure
E). The smooth muscle cells were immunoreactive for SMA, actin, and Vim. The intra-vessel endothelial cells were positive for CD34 (Figure
F). The patient recovered rapidly. At 12-month follow-up, MRI scans were performed, there was no tumor recurrence (Figure
I).


A 56-year old male patient presented with backache that had persisted for more than seven months, with exacerbation of the pain for half a month. Six months prior to this, the patient felt pain in the back with no clear predisposing cause. In October 2011, the patient sought medical advice from the Affiliated Hospital of Inner Mongolia Medical University (Hohhot, China). A lumbar computed tomography (CT) scan revealed a lumbar disc bulging between the third and fourth lumbar vertebrae. The patient received Chinese medicinal pills (dosage and composition unknown) and plaster external treatment; however, the results were not effective. In January 2012, half a month prior to the current admittance to hospital, the patient felt lumbar acid, with exacerbated pain, weakness of the double lower limbs, activity limitations and dark brown urine. The patient consulted The Second Affiliated Hospital of Inner Mongolia Medical College (Hohhot, China), and CT revealed some specific findings ().
In February 2012, in order for further diagnosis and treatment, the patient was hospitalized in the Department of Orthopedics of the Affiliated Hospital of Inner Mongolia Medical University. Since the onset of the symptoms, the patient had experienced alternating diarrhea and constipation, and had lost ~30 pounds in weight. The patient’s family history was not contributory, and physical and library examinations revealed no abnormalities.
Lumbar vertebrae magnetic resonance imaging (MRI) () and thoracic MRI () scans showed abnormal signals. The CT and MRI findings revealed multiple myeloma and multiple bone metastases. A Bence-Jones protein test and biopsy were performed, with negative results. However, the pathological diagnosis was positive (). Based on these findings and the patient history, the patient was diagnosed with malignant papillary mucinous cystadenocarcinoma.
When the imaging and pathological diagnoses were combined, the source of the spinal multiple malignant tumors was not clear. Malignant papillary mucinous cystadenocarcinoma is primarily derived from glands, including those of the respiratory and gastrointestinal tracts. Based on analyses of the respiratory and digestive tracts, and other laboratory examinations and imaging, the tumor was located in the body of the pancreas ().
In June 2013, the patient succumbed to the condition. Retrospective analyses suggested that if the pancreatic lesions had been detected and treated earlier, the prognosis may have been improved.


A 62-year-old right-handed white male with a history of hypertension and hyperlipidemia was treated at the emergency room. He complained of two similar episodes within the previous 48 hours, consisting in repetitive questions to his wife with face of strangeness: “what time is it?” and other similar questions related to the activities that they had been doing the same day, for every 10 minutes for 2 hours with expressions of confusion. Subsequently, the patient did not remember anything about what happened during the episode. A similar episode happened the following day, and he returned to the emergency room. There was no history of migraine, psychiatric illness, head trauma, epilepsy, or alcohol abuse. There were no other pathological findings in his medical history. He asked the same questions over and over again, showing great difficulties in grasping simple information. All symptoms disappeared after a period of 120 minutes. Immediate and remote memories were preserved but he could not remember what had happened since the beginning of the symptoms and retrograde amnesia was present covering a period of several hours. Other high cerebral functions were preserved. On examination the patient was alert with a blood pressure of 130/80 mm/Hg, regular heart beats of 70 beat/min, and a temperature of 36.8°C. General and neurological examination showed no pathological findings (mRS = 0; NIHSS = 0). A neuropsychological test for memory function performed after the amnesic episode was within normal limits. The Minimental State Examination score was 30/30. The complete hematological screening, routine biochemical profile, urinalysis, thrombophilic, and autoimmunologic blood test, chest roentgenography, 12-lead electrocardiography, Doppler ultrasonography of the supra-aortic trunks, and 24 h Holter monitoring were normal. Brain MRI study on day 3 after TGA showed a 14 mm T2-hyperintense lesion on diffusion-weighted image (DWI) suggesting an acute ischemic infarct on right cingulate gyrus () with mild chronic ischemic periventricular white matter hyperintensities. The hippocampus, thalamus, and basal ganglia appeared normal. Intracranial MR angiography was also normal.
At the 6th and 12th month followup controls the patient remained entirely assymptomatic.


A 23-year-old female presented with nasal obstruction since childhood. She had symptoms of obstructive sleep apnoea with snoring and daytime somnolence. She did not have constitutional symptoms. Initial fibreoptic nasal endoscopy examination revealed a mass arising from the posterior nasal space and filling the nasopharynx.
MRI demonstrated a large mucosally based mass in the nasopharynx (Figures –). Based on the imaging findings, the diagnoses of lymphoma versus minor salivary gland carcinoma were considered. The patient subsequently underwent endoscopically assisted excision of the mass.
Histopathologic features were consistent with hyaline vascular CD. Lymphoid follicles with germinal centres were seen just below the epithelium. Some germinal centres were penetrated by hyalinised vessels and there was an increase in interfollicular vasculature (). Flow cytometry and IgH gene rearrangement studies revealed a polyclonal reactive population of lymphocytes.
The patient has remained well and asymptomatic at 2-year followup.


A 32-year-old parous woman visited our clinic complaining of gross hematuria. Before 4 months, her second pregnancy normally went to term and a healthy infant was delivered in the 39th week of gestation via vaginal delivery. Urinalysis revealed a urinary-specific gravity of 1.037, 100 mg/dL proteins, and numerous red blood cells per high power field. The result of urine cytology was class 1. Laboratory tests revealed no abnormal findings. Urethrocystoscopic examination failed to show hematuria coming from the ureteral orifice. Ultrasonography revealed no abnormal findings in the ureters and kidneys. Enhanced computed tomography (CT) demonstrated the compression of the LRV between the aorta and SMA with marked dilation of LRV and the venous collaterals (). Angiography to measure the pressure gradient was not performed. The diagnosis of nutcracker syndrome was made on the basis of these findings. Therefore, the patient was conservatively observed. The macrohematuria disappeared by itself after 1 month.


A 23-year-old Chinese woman presented with cough, expectoration, wheeze and dyspnea for 3 months. A physical examination indicated weakened respiratory sounds in her right lower lobe. A chest computed tomography (CT; Figure ) showed partial atelectasis of her right lower lobe. Her intermedial segment bronchus was in severe stenosis, with a diameter of approximately 3mm (Figure ). Our final diagnosis was pulmonary TB combined with bronchial TB based on the detection of tubercle bacilli from her sputum. She took isoniazid (0.3g/day), rifampicin (0.45g/day), ethambutol (0.75g/day) and pyrazinamide (0.5 three times daily) for 2 months.After attaining her signed informed consent and the permission of the Ethics Committee of our hospital, we performed a high-pressure balloon dilation in her intermediate bronchus through videobronchoscope (BF-260, Olympus Corp., Tokyo, Japan), with the balloon produced by Johnson & Johnson Corporation of USA in China, 10mm diameter ×40mm length (Figure ).The procedure for our high-handed videobronchoscopy balloon dilation was as follows: A) Insert the bronchoscope into her bronchus. B) Insert the guide wire along the working hole of the bronchoscope. C) Pull out the videobronchoscope from her bronchus and reinsert the bronchoscope into her bronchus. D) Insert the high-handed balloon along the guide wire into the narrowest part of her bronchus and inject physiologic saline through the balloon duct with a booster pump to increase the pressure of the balloon (from 0 to 14atm). E) Remove the bronchoscope from her bronchus. She was seated for 40 minutes with continual balloon dilation at high pressure (14atm) and without the videobronchoscope in her bronchus. F) Re-insert the bronchoscope into her bronchus and remove the balloon and the guide wire from her bronchus. G) Remove the bronchoscope from her bronchus after the operation is finished. One month after continuous anti-TB treatment, CT film showed that gradual expansion of her right lower lobe had occurred until it had completely opened (Figure ), and her clinical symptoms had disappeared. An examination using an electronic bronchoscope showed the diameter of the intermediate bronchus was 9mm (Figure ). Five weeks later, she was checked twice through the bronchoscope and there was no re-stenosis in her intermediate bronchus. She then took anti-TB drugs for 12 months and recovered completely and without re-stenosis. No re-stenosis was observed after continuous anti-TB treatment for 13 months. There were no side effects.


A 49-year-old woman presented to our department with massive genital bleeding. Vaginal examination revealed a protruding mass in the cervical os. A cervical biopsy was performed and the pathological examination of the biopsy showed undifferentiated carcinoma ().
She was diagnosed with stage IIB cervical cancer by rectal examination and was admitted to our hospital. The levels of squamous cell carcinoma antigen (SCC) and carcinoembryonic antigen (CEA) were elevated to 7.2 ng/mL and 7.18 ng/mL, respectively. Magnetic resonance imaging (MRI) of the pelvis revealed a large mass infiltrating to the uterine corpus and vagina ().
Hemoglobin level was 5.0 g/dL and a total of 8 units of packed red blood cells were transfused. She underwent concomitant chemoradiotherapy (CCRT) consisting of 50 Gray whole pelvis irradiation in 25 fractions at 2 Gray per fraction, high-dose-rate intracavitary brachytherapy 24 Gray/4 fractions, and six weeks of paclitaxel (60 mg/m2/week) and carboplatin (AUC = 2/week) administration. When the therapy was completed, MRI revealed almost complete remission. The levels of SCC and CEA decreased to normal ranges. Cytological test of the cervix was negative. The patient remained free of disease for 10 months. Then a routine follow-up CT suspected spleen metastasis. Although five courses of nedaplatin (70 mg/m2/4 weeks) were administered, the level of SCC was elevated to 19.45 ng/dL and the mass in the spleen enlarged ().
An exploratory laparotomy was performed and the spleen was removed (). The result of pathological examination was compatible with metastasis from cervical cancer ().
Four months after the surgery, CT revealed clavicular, axillary, mediastinal, and para-aortic lymph nodes metastases. Three courses of irinotecan (60 mg/m2/3 weeks) were administered. Then CT revealed stable disease. She underwent 7 courses of systemic chemotherapy consisting of irinotecan (60 mg/m2/3 weeks) and nedaplatin (70 mg/m2/3 weeks). CT revealed stable disease again. Five courses of paclitaxel and carboplatin were administered. She is alive with the disease 20 months after splenectomy.


A 37-year-old male patient reported to the Department of Oral Medicine and Radiology with the chief complain of swelling on the right buccal mucosa from last one year. On clinical examination a well circumscribed red to purple lesion in color approximating 2 × 2 cm on the right buccal mucosa was noted (). The swelling was firm in consistency and nontender on palpation. The mucosa overlying the swelling was normal in color with absence of any ulceration. There was no history of chronic cheek biting and irritation was reported. Association of any extra oral swelling was not evident. On the basis of clinical features provisional diagnosis of mucocele was made. Aspiration was performed by 25-gauge needle under topical anesthesia (). A collection of frank blood was obtained which was subjected to cytological examination. Cytological examination ruled out the possibility of mucocele. Ultrasound reports confirmed that the lesion was not associated with any feeder vessel which is the characteristic feature of hemangioma.
After the correlation of clinical, cytological, and ultrasound finding, treatment was planned for surgical excision under local anesthesia (). Dissection of the lesion revealed a soft and well-encapsulated swelling. The excised specimen was sent to the Department of Oral and Maxillofacial Pathology for histopathological examination ().
Histopathological examination revealed fibrous connective tissue composed of numerous collagen fibers and proliferating fibroblasts (). Numerous dilated blood vessels and sinusoidal spaces surrounded by endothelial cells were also noted ().
On the basis of histopathological features final diagnosis of angiofibroma was made. The follow-up period was uneventful without any recurrence and other complications.


We present a case of a 44-year-old male who was started on omalizumab in 2006 due to persistent and debilitating asthma symptoms (NHLBI step 5). He had a history of severe hypersensitivity to hymenoptera venom initially diagnosed at age twelve after an anaphylactic reaction to a witnessed insect sting, presumably vespid. At that time he was hospitalized for 48 hrs, treated with intramuscular epinephrine injections, prednisone, and antihistamines. At age 35, he suffered a second and third reaction while hiking. Both reactions manifested as angioedema of face, tongue, and throat, as well as persistent urticaria and wheezing. Both events resolved with intramuscular epinephrine injections, prednisone, antihistamines, and supportive care. Medications at the time of the second and third events included nasal mometasone, mometasone/formoterol MDI, levalbuterol MDI, montelukast, tiotropium, and simvastatin.


In May 2002, a 33-year-old Italian man presented to our outpatient clinic complaining of fever, weight loss, and diarrhea. He had mucocutaneous lesions highly suggestive of Kaposi's sarcoma (KS). On physical examination he had hepatosplenomegaly; laboratory tests showed pancytopenia and polyclonal hypergammaglobulinemia. He tested HIV positive. His CD4+ T-cell count was 17 cells/μL and HIV RNA viral load was 225,181 copies/mL. A bone marrow smear showed the presence of Leishmania spp. amastigotes. As a consequence, diagnosis of VL was made and treatment with meglumine antimoniate at a dose of 20 mg/kg/day was given for 30 days. Moreover, HAART with Lamivudine-Stavudine-Lopinavir/ritonavir was started. The patient clinically improved, with progressive resolution of KS lesions and good virological response to antiretroviral therapy. Six months later, the patient suffered a clinical relapse of VL and was treated with intravenous liposomal Amphotericin B (L-AMB) at a dose of 4 mg/kg/day for 5 days and then weekly at the same dose for 4 weeks. However, several relapses of VL occurred over the following years. Each relapse was treated with the aforementioned drug schedule. In February 2008, it was decided to start maintenance therapy with L-AMB at a dose of 4 mg/kg/day once a month, in an attempt to prevent further relapses. At this time, his HAART regimen consisted of Abacavir-Lamivudine-Lopinavir/ritonavir; HIV viral load was stably below 50 copies/mL and CD4+ T-cell count was 125 cells/μL. Some weeks after starting secondary prophylaxis with L-AMB, a maculopapular and nodular rash appeared on his trunk and upper limbs (). The rash gradually worsened and, four months later, treatment with L-AMB was stopped and a cutaneous biopsy was performed. Histology revealed the presence of a granulomatous infiltrate, with lymphocytes, plasma cells, and macrophages, most of which contained Leishmania amastigotes. Restriction fragment length polymorphism-polymerase chain reaction (RFLP-PCR) was positive for L. infantum. These findings were consistent with a diagnosis of PKDL. In September 2008, the patient started complaining of fever, diarrhea, and night sweats. Paradoxically, cutaneous lesions markedly improved. On the basis of his symptoms, a relapse of VL was suspected and confirmed by bone marrow aspirate, which showed the presence of Leishmania amastigotes. Treatment with meglumine antimoniate was restarted at a dose of 20 mg/kg/day for 30 days. The patient experienced a rapid clinical response and complete disappearance of cutaneous rash. Unfortunately, the patient had several relapses of VL over the following years, though the interval between them has become wider after restarting maintenance therapy with L-AMB 4 mg/kg/day once a month. At the last followup in December 2013, the patient was in good health and was still receiving secondary prophylaxis with L-AMB. He was taking HAART with Abacavir-Lamivudine-Lopinavir/ritonavir-raltegravir with his last CD4+ T-cell count being 98 cells/μL and HIV RNA < 20 copies/mL.


A 65 year old male with an extensive operative history for Crohn’s disease, including 4 laparotomies with small bowel resections to ameliorate small bowel obstructions presented to us. His first surgery was performed in England in 1991, and the next 3 were performed in Peru, one in 1998 and two in 2007. Over the past few years he has been suffering from nutritional deficiencies and osteopenia associated with his resultant short bowel syndrome. He has had, in addition, intermittent drainage from his previous midline incision. The drainage was non-bilious, serosanguenous fluid that would egress approximately every 2–3 days. Several sutures were removed through the tract in the past but the wound still failed to heal. The drainage volume and quality did not change significantly over the past year. Although he did suffer from short bowel syndrome, he has had no change in his gastrointestinal symptomatology or body weight over the past year. On physical examination, his vital signs were within normal limits. His chest and abdominal examinations were unremarkable, except for an infra-umbilical surgical scar with, what appeared to be, a chronic sinus draining a minimal amount of serous fluid. A number of subcutaneous sutures were palpable, adjacent to the orifice of the sinus but no erythema or purulence was observed. A CT fistulogram done in 2008, 1 year after his most recent bowel resection, revealed a possible enterocutaneous fistula (Figure ).
Given his history of inflammatory bowel disease, multiple prior surgical procedures and physical findings, the working diagnosis was a chronically draining abdominal wall sinus tract most likely secondary to a previously infected suture. The possibility of an enterocutaneous fistula was also entertained; however, his clinical picture, with minimal and intermittent drainage of non-succus fluid made the latter possibility less likely. The fistula noted on the CT fistulogram 5 years ago was believed to have closed given his presentation.
After explaining the risks, benefits and possible complications of surgical intervention, our patient elected to have a wound exploration with possible laparotomy under general anesthesia for the purpose resecting this sinus tract.
An elliptical incision was made around the suspected sinus tract and followed down to the fascia. A hemostat was placed in the lumen of the sinus tract which traveled below the fascia, necessitating entry into the abdomen. Sharp dissection along the previous midline incision revealed moderate to severely thickened abdominal wall adhesions, most likely a result of prior obliterative peritonitis. Extensive lysis of adhesions and mobilization of the small bowel took approximately 10 hours. Once the extensive adhesions were lysed and the bowel mobilized, the tract was traced to a 15 cm blind ended portion of small bowel, closed on one end with the fistulous tract emanating from the other. After running the bowel from the Ligament of Trietz to the peritoneal reflection multiple times revealing the rest of the bowel to be fully continuous, it was clear that the suspected sinus tract, was in fact, a fistula tract connected to a blind segment of small bowel which had its own mesenteric blood supply. A second surgeon was called into the OR to confirm the continuity of bowel and the intraoperative findings. Only after multiple confirmations of bowel continuity, was the tract and blind segment resected.
Postoperatively, our patient regained bowel function and promptly improved in overall health. At follow-up his wound was healing well without complications with resolution of his chronic draining sinus.


A 60-year-old Indian man presented to our institution with complaints of generalized epigastric abdominal pain of moderate intensity for the previous 10 days, with associated nausea, vomiting and diarrhea of recent onset. His past medical history was significant for anemia that required blood transfusions on two separate occasions, but it was otherwise unremarkable. He had arrived in our area from India two weeks prior to presentation. A presumptive diagnosis of acute gastroenteritis was made, and the patient was started on intravenous fluids and admitted to the medical ward.
Ultrasonography, computed tomography (CT) and magnetic resonance imaging scans of the patient’s abdomen revealed thrombosis of the portal vein, splenic vein and superior mesenteric vein, along with a hypodense region in the liver suggestive of infarction and edematous bowel loops with fat-stranding indicative of mesenteric ischemia (Figures and ). A preliminary blood work-up revealed macrocytic anemia with 8.7g/dL hemoglobin and 137.1fL mean corpuscular volume. A peripheral blood smear showed numerous macrocytes, macro-ovalocytes and hypersegmented neutrophils indicative of megaloblastic anemia. His serum folate and red blood cell folate levels were normal, but his serum vitamin B12 levels were severely low at <44pmol/L. An additional work-up showed a positive 1:80 titer for anti–parietal cell antibody and a markedly positive anti–intrinsic factor antibody level of 131U, thus establishing the diagnosis of pernicious anemia. An extensive coagulation work-up showed a raised prothrombin time (13.3 seconds), an elevated activated partial thromboplastin time (43.4 seconds) and a high international normalized ratio (1.3). It also revealed a normal platelet count, normal protein C and protein S levels, and normal anti–thrombin III function, as well as a negative result for lupus anti-coagulant.
Genetic testing for factor V Leiden mutation and JAK2 gene mutation was negative. Immunoglobulin G (IgG) and IgM assays for anti-cardiolipin and anti-β2-glycoprotein antibodies were below diagnostic values, as were anti-nuclear antibody tests. However, the patient’s serum homocysteine levels were markedly high at 70μmol/L (normal 4μmol/L to 12μmol/L). The results of a test for the methyltetrahydrofolate reductase (MTHFR) gene was negative for any remarkable polymorphisms, including the C677T mutation. A genetic test result for factor II mutation was also negative.
The patient was admitted to the medical ward and started on anti-coagulation therapy with warfarin and low-molecular-weight heparin, as well as parenteral vitamin B12 therapy, after his vein thromboses were deemed surgically incorrigible. During his hospital stay, he developed progressive ascites that required periodic drainage. On day 20 of admission, the ascitic fluid drainage turned turbid and a CT scan showed mesenteric perforation (Figure ). An emergent laparotomy revealed mesenteric gangrene with perforation, and a bowel resection with ileostomy and mucus fistula creation was performed. The patient’s recovery was complicated by Bacteroides ovatus bacteremia and Klebsiella pneumoniae sepsis, which resulted in septic shock and multi-organ dysfunction syndrome necessitating intensive care. He eventually developed progressively rising bilirubin and falling albumin levels as well as profound jaundice and encephalopathy, indicative of end-stage hepatic failure. Despite aggressive anti-coagulation therapy being administered continuously throughout the patient’s admission, and in spite of his vitamin B12 and homocysteine levels turning normal, his thromboses did not resolve. This resulted in splenic infarction as well as progressive liver failure with severe hepatic encephalopathy. The patient eventually died due to hepatic failure three months into his hospital stay.


A 71-year-old Malay female was referred to the Department of Ophthalmology, Tan Tock Seng Hospital (Singapore) in October 2011 complaining of blurred vision in the superior field of the right eye, which had persisted for the previous five days. The patient did not exhibit any associated symptoms, such as eye redness, pain or floaters and the patient was not myopic. The patient was admitted to the internal medical unit with suspected pneumonia and was also a carrier of the human immunodeficiency virus for which she was undergoing Highly Active Anti Retroviral Therapy (HAART). The patient had no other medical history. Written informed consent was obtained from the family of the patient.
Examination revealed best-corrected visual acuity of 6/9 bilaterally. Slit-lamp examination of the anterior segment was unremarkable, however, mild cataracts were apparent. Examination of the posterior segment showed a choroidal lesion spanning ~8 mm and involving the superior half of the macula and the superior temporal quadrant of the right eye, with an overlying area of exudative retinal detachment (). In addition, there was a small choroidal lesion inferior to the lesion. The posterior segment of the left eye was normal. The two optic discs were considered to be normal and had a cup-to-disc ratio of 0.4. The pupillary light reflex was normal, with no relative afferent defects observed and the extraocular movements were normal.
A B-scan ultrasound of the right eye showed the superior temporal lesion measuring 7.7 mm laterally, 9.5 mm radially and a maximum thickness of 2.6 mm, in addition, internal reflectivity was medium to high (). There was an overlying area of exudative retinal detachment. The second choroidal lesion at the inferior equator was small with a maximal thickness of 1.5 mm and medium to high internal reflectivity. Fluorescein angiography demonstrated early blocked hypofluorescence with progressive hyperfluorescence at the border of the lesion in the later phases and pinpointed leakages. Indocyanine green angiography showed blocked hypofluorescence with hyperfluorescence at the border of the lesion.
A full blood count indicated normocytic normochromic anemia, however, was otherwise normal. The C-reactive protein level was markedly elevated and the levels of liver transaminases were mildly raised. Serology for syphilis was identified as negative, however, immunoglobulin G serology for the cytomegalovirus and toxoplasmosis were positive. Serum electrolyte levels and the patient’s coagulation profile were normal. Notably, a chest X-ray showed increased air space shadowing in the left lower lobe, which was consistent with pneumonia; the right lung lobes were normal (). It was hypothesized, based on the clinical findings, that the patient was presenting with ocular tuberculosis or choroidal metastasis, therefore, a CT scan of the whole body was performed. The CT scan of the thorax revealed a large lobulated mass in the right upper lobe, measuring 3.3×1.6 cm (). Metastases were indicated by multiple, subcentimeter lesions in the other regions of each lung, as well as by multiple hypodense lesions in the liver and multiple lytic lesions in the thoracolumbar spine. Multiple enlarged bilateral supraclavicular, pretracheal, subcarinal and right hilar lymph nodes were also observed. Thus, the patient was advised to undergo a confirmatory lung biopsy, which revealed a non-small cell lung carcinoma that was indicative of an adenocarcinoma. Immunohistochemistry demonstrated that there were more thyroid transcription factor 1-positive cells than p63-positive cells in addition to faint diffuse staining for the two markers. Immunohistochemistry, therefore, indicated an adenocarcinoma. Although the preservation of the DNA in this case was not sufficient to allow a confident analysis of exon 18, the other three axons of clinical significance were wild-type. Mutations in exons 19 and 21 represented >90% of the total mutations. Furthermore, the KRAS mutation was identified in codon 13 of exon 2 and there was no rearrangement of the ALK gene. However, pleural fluid cytology did not reveal any malignant cells. Based on the clinical findings, it was hypothesized that the choroidal lesions in the right eye were potentially metastatic. A repeat chest X-ray was conducted two weeks following the initial presentation, which showed interval enlargement of the right-sided pleural effusion and further compressive collapse of the right lung. Bony destruction in the lateral aspects of the right, fifth to seventh ribs and erosion of T11 was noted, which indicated a neoplastic process was occurring. The patient’s family was counseled regarding the terminal nature of the illness and the prognosis of 6–12 months was reiterated to them. Palliative management was advised based on discussion with the family and the patient continued receiving HAART therapy for the retroviral disease. However, the patient succumbed to the cancer within the following two weeks.


A 73-year-old female presented to the Internal Department of Oncology, Shandong Cancer Hospital and Institute (Jinan, China) with pain in the left hip that had persisted for two months. Plain X-rays showed no abnormalities of the pelvic bones, however, magnetic resonance imaging (MRI) of the left hip was performed and showed abnormal signals involving the left innominatum, with soft tissue formation. The signal intensity was low on T1-weighted images (), high or isointense on T2-weighted images () and hyperintense on short TI inversion recovery ().
Technetium-99m (99mTc) radionuclide bone scans were performed to rule out multiple bone lesions, and increased tracer uptake was shown in the left innominatum, including the ilium, acetabulum and ischium ().
An 18F-fluorodeoxyglucose-positron emission tomography-computed tomography (FDG-PET-CT) scan was performed to identify the original site of the tumor. Abnormal 18F-FDG uptake was found in the left innominatum, with a peak standardized uptake value of 60.7, lytic lesions and soft-tissue lump formation ~7.8×4.5×8.8 cm in size ().
Next, a fine-needle aspiration biopsy of the lesion was performed and a histopathological examination showed diffuse, round tumor cells of approximately the same size. Immunohistochemistry showed that the cell membrane was strongly positive for cluster of differentiation (CD)20 and negative for CD79α, CD3, CD78, CD138, cytokeratin (CK) and CK8/18, supporting a B-cell origin (). Further examinations, including bone marrow aspiration and biopsy, and CT scans of the neck, chest and abdomen, were normal.
The patient was diagnosed with PLB of the left innominatum, classified as stage I–E according to the Ann Arbor system (). The patient was treated with 50 Gy radiation therapy in 25 fractions over five weeks, followed by six cycles of CHOP regimen (1,000 mg cyclophosphamide, 80 mg epirubicine and 2 mg vincristine on day one, and 100 mg prednisone on days one to five, every three weeks). Following the third cycle of CHOP, the patient experience abnormal pain in the left hip. The FDG-PET-CT examination was repeated, but no abnormal FDG uptake was observed (). However, the neck of the left femur was broken, with displacement of the distal section. At present, the patient is being regularly followed up and has remained disease-free since the last treatment, with the exception of the broken femoral neck.


A 35-year-old female, gravida 3, para 2, was admitted to the Gynecological Ward of The First People’s Hospital of Foshan (Guangzhou, China) with a chief complaint of vaginal bleeding following intercourse for 1 month. The father of the patient had a history of nasopharyngeal carcinoma. Clinical examination identified an ulcerated, mobile, and solid-cystic 4 cm-diameter mass on the left lateral aspect of the upper half of the vagina, with no indication of abnormality in the external genitalia, uterus or ovaries. There were no palpable lymph nodes. The patient was clinically diagnosed with a cyst of vagina, and thus a tumor resection of vaginal wall was performed. During the operation, the tumor presented as poorly circumscribed, gray-white, and easily bled at contact, resulting in difficulty to remove the entire tumor. Tissue samples were collected and sent for frozen-section examination, and a primary vaginal malignant tumor was reported, which accorded with the initial experience-based speculation of the surgeon. Following the final diagnosis shown through results from paraffin-sections, the patient and her family were fully informed about the disease and decided to transfer the patient to a specialized cancer hospital.
Further pathological analyses of the specimen confirmed that the tumor was a primary vaginal melanoma. Immunocytochemistry results revealed that the tumoral cells were positive for HMB45, Melan-A, S-100, CD56, CD99, EMA, and negative for PAS. A malignant melanoma was therefore confirmed by the pathological diagnosis. One month after the local excision, the patient presented at Cancer Center of Sun Yat-Sen University (Guangzhou, China) for further therapy. A 3×2 cm pigmented mass was identified in the middle of the anterior vaginal wall. The urethra was normal and there was no grossly palpable lymph node. The patient underwent a Piver III radical hysterectomy, bilateral pelvic lymphadenectomy, left adnexectomy, right salpingectomy, total vaginectomy, total urethrectomy, cystostomy, and suspension of right ovary. Specimen observation following the surgical procedure indicated that the length of the removed vagina was 8 cm. Histopathological results of the specimen revealed the entire vaginal wall was involved, no tumor tissue was present in the surgical margins, and no lymphatic invasion was identified. Immunohistochemistry demonstrated positive staining for HMB-45 and S-100. Three weeks later, the patient presented with a serous-type of leakage from the perineum which was proven as urine by laboratory tests. The patient was subsequently diagnosed with a vesico-vaginal fistula. A surgical procedure to repair the vesico-vagina fistula was thus performed. The general condition of the patient rapidly deteriorated and two months following the surgery, a gynecological examination revealed a pelvic metastasis. A 6×5 cm mass was palpated under the left recta with involvement in the retrovaginal septum. In addition, a 4×5 cm mass on the right recta was found, which was extended onto the pelvic wall. An enlargement of the inguinal lymph node on the right side was also identified. Six months following the surgery, the patient had difficulties in deffacating, and a CT scan indicated the mass had covered the entire pelvic cavity. The patient was then lost to follow up.


A 59-year-old man underwent endoscopic nasal polypectomy. The polyp, which originated from the right inferior turbinate (), was removed easily with minimal bleeding, and nasal packing was performed with Merocel after surgery. The packing was removed the next day, but continuous bleeding from the right nasal cavity developed. To prevent further bleeding, nasal packing was performed again in the right nasal cavity. On the second day after surgery, the packing was removed successfully without further bleeding. However, the man later visited the emergency department due to nasal bleeding. Nasal packing into both nasal cavities was performed again and the bleeding stopped, but rebleeding developed after two days even with intranasal packing.
The laboratory results before nasal polypectomy were all within normal ranges (hemoglobin 9.2 g/dL, platelet count 320 × 103/μL, prothrombin time 9.8 seconds, and activated partial thrombin time 40.8 seconds). When he initially visited the emergency department due to nasal bleeding after polypectomy, hemoglobin was decreased, but platelet count, prothrombin time, and activated partial thrombin time were still in the normal ranges (hemoglobin 6.5 g/dL, platelet 208 × 103/μL, prothrombin time 10.0 seconds, and activated partial thrombin time 39.5 seconds).
Emergent surgical cauterization was attempted in the operation room. The bleeding originated from the posterior nasal cavity, but the exact bleeding focus could not be identified and was not controlled by electrical cauterization. To prevent hypovolemic shock because of blood loss, the surgery was stopped, and anterior/posterior packing with Vaseline gauze was performed. The patient was moved to the intensive care unit (ICU) with tracheal intubation to prevent aspiration, and arterial embolization was performed the next day. During angiography, a pseudosac was found at the distal part of the left maxillary arterial branch, but a definite bleeding focus could not be found (). Embolization was performed at the pseudosac area, but it was not successful and bleeding persisted. Nasal packing was again performed in the ICU.
The patient did not take any anticoagulative medications and did not have hypertension, diabetes mellitus, or other cardiovascular diseases. However, three years before, he had an episode of hematemesis, which was diagnosed later as gastric angiodysplasia by endoscopy, but a bleeding focus was not found. The hematemesis lasted for several days, and the bleeding eventually stopped spontaneously. Additionally, he had history of bleeding for four days after a dental procedure several years before.
Under suspicion of a coagulative disorder, we consulted a hematologist and performed a coagulation factor assay and testing for platelet function. Factor VIII was 30% and collagen/ADP closure time was prolonged. These results indicated that platelet function was abnormal, and a platelet dysfunction disorder was suspected. Therefore, we conducted platelet transfusion for three days, and the bleeding eventually became sticky and formed a hematoma. On the sixth day after platelet transfusion, the bleeding stopped completely, and we were able to remove tracheal intubation. Red blood cell (RBC) transfusion and albumin supplementation were also necessary. The patient was discharged without any complications after two weeks. To confirm the platelet disorder, further blood testing was performed after platelet transfusion to stabilize the patient. Therefore, the diagnosis with additional confirmatory blood tests was completed ten days after the final platelet transfusion. The laboratory results corresponded to Von-Willebrand disease type IIa. Ristocetin response was decreased, and a low level of VWF multimer was also identified by VWF multimer study.
Six months after discharge, the patient fell and developed a subacute subdural hemorrhage (). Three neurologic surgeries to remove intracranial hematoma were performed with massive platelet transfusion. His overall function has improved but with the complication of hemiplegia that is being treated with rehabilitation.


A 29-year-old gravida 2 para 1 Caucasian female was referred for maternal-fetal-medicine consultation at 26-week gestation due to suspected fetal anomalies. Obstetrical ultrasound examination, confirmed by fetal magnetic resonance imaging (MRI), demonstrated craniofacial duplication, several abnormalities of the brain and skull, thoracolumbosacral dysraphism with neural tube defect and likely Chiari II malformation, large congenital diaphragmatic hernia (CDH) with liver and bowel noted in the left chest, hypoplastic left lung, and possible horseshoe kidney (). Fetal echocardiogram findings were consistent with Tetralogy of Fallot (TOF). The parents had a multidisciplinary consultation with maternal-fetal medicine, neonatology, genetics, cardiology, radiology, and palliative care. Given the multiple, severe congenital anomalies, the medical team and family planned for limited resuscitative efforts and anticipated comfort care after birth.
A 2440 g male infant was delivered via repeat cesarean section at 36-week gestation secondary to preterm labor. At birth, the infant was pale and cyanotic with no spontaneous cry and poor tone. He had shallow spontaneous respirations with poor aeration on auscultation. The left mouth was small and fixed. No glottis was visualized when the laryngoscope was inserted into the right mouth. The infant was then wrapped in a warm blanket and given to the family to hold. He died in his parents' arms a few minutes after birth.
The parents consented to a complete postmortem evaluation. Physical exam demonstrated complete facial duplication with tetrophthalmos (four eyes), two noses, two mouths, two chins, and one fully formed ear on each side of the head with a hypoplastic pinna in the midline. Two anterior and two posterior fontanels were palpable. There was also a ruptured myelomeningocele measuring 6.3 cm × 3.9 cm in the thoracolumbar region and a scaphoid abdomen. The first and second toes of each foot overlapped and the nails were hypoplastic.
Dissection of the cranial cavity revealed brain duplication with fusion of the parietal and occipital lobes of each brain. The right and left brains fused at the mesencephalon and brainstem. Each brain had an optic chiasm, a pituitary gland, and a single large ventricle with loss of the ependymal lining and no third ventricle identified. There was a single midbrain, rudimentary cerebellar tissue and fourth ventricle, atresia of the cerebral aqueduct with rudimentary and hypoplastic cerebral peduncles, bilateral absence of the corpus callosum, bilateral polymicrogyria, and dysplasia of the right and left cortices. Dissection of the thorax and abdomen revealed left CDH with intestine, stomach, spleen, and the left lobe of the liver herniated into the left pleural cavity with concomitant pulmonary hypoplasia. Cardiac findings included TOF (pulmonary atresia, ventricular septal defect, overriding aorta, and pulmonary arteries), atrial septal defect, patent ductus arteriosus, superior pulmonary veins draining to the left atrium (no inferior pulmonary veins), dilated right atrium, and hypoplastic left atrium. A common oropharynx connected two separate nasopharynxes and two separate oral cavities. There was a single larynx and esophagus. The intestines were malrotated with the appendix located in the left upper abdominal quadrant. Ectopic right and left kidneys were located in the lower abdomen and an accessory spleen was present. Testes were undescended bilaterally and the right testis was atrophic. The placental pathology revealed normal fetal membranes and chorionic villi consistent with third trimester gestation with a two-vessel umbilical cord ().
Whole-genome microarray-based comparative genomic hybridization (aCGH) using Oxford Gene Technology revealed a male karyotype (46 X,Y), a 983 kb deletion on chromosome 4q34.3, a 562 kb gain on chromosome Xp22.31p22.2, and a 32 kb gain on chromosome 13q12.11. Quantitative polymerase chain reaction of maternal blood indicated the duplications on chromosomes Xp22.31p22.2 and 13q12.11 were maternally inherited. The 4q34.3 deletion was not maternally inherited, however. Unfortunately, due to the family's financial burden, genetic testing was not completed on the father.