A 52-year-old man with a past medical history significant for atrial fibrillation, diabetes type 2 and hypertension for 30 years was admitted with early satiety for the past 3 months and progressive dyspnea on exertion for 3 weeks. He also complained of gradual unintentional weight loss of approximately 100 pounds over the last 10 years. He had been a non-smoker and a moderate alcohol user for the last 30 years. On initial presentation to the hospital, he denied shortness of breath at rest or chest discomfort and was in no acute distress.
His blood pressure was 204/93 mm Hg on the left arm and 194/88 mm Hg on the right arm with an irregular heart rate of 54 beats/min and oxygen saturation of 96% on room air. The lungs were clear to auscultation, jugular venous distension was noted at 13 cm above the sternal angle and 3+ bilateral leg edema with scrotal edema. Cardiac auscultation was pertinent for 3/6 systolic ejection murmur over the left sternal border. His abdomen felt firm and lacking in tympanicity, but it was non-tender in all four quadrants. Bowel sounds were audible but faint. Laboratory values were notable for a brain natriuretic peptide of 980 ng/l, creatinine of 1.2 mg/dl, BUN of 20 mg/dl, HCO3 of 31 mEq/l, INR of 1.23, Hb of 12.5 g/dl, Na of 140 mEq/l, K of 3.7 mEq/l and negative cardiac troponins. He was monitored in the Intensive Care Unit for decompensated heart failure with uncontrolled hypertension.
To promote diuresis and reduce afterload, he was administered intravenous furosemide 80 mg, oral metolazone 2.5 mg, oral hydralazine 75 mg every 6 h, oral clonidine 0.3 mg, lisinopril 40 mg, isosorbide mononitrate 120 mg and losartan 100 mg. Overnight, he had an episode of moderate self-limited epistaxis with minimal improvement in blood pressure control. He remained in slow atrial fibrillation with a lowest recorded heart rate of 38 beats/min. However, he denied dizziness at any time despite a negative fluid balance of 1.8 liters. Renal function worsened with an increase of creatinine to 1.6 mg/dl.
An echocardiogram showed a left ventricular ejection fraction of 30% with four-chamber dilatation, moderately reduced right ventricular systolic function, severe mitral regurgitation and moderate pulmonary hypertension. He underwent right heart catheterization showing a right atrial pressure of 12 mm Hg, right ventricular pressure (systolic/diastolic) of 60/12 mm Hg, pulmonary artery pressure of 60/23 mm Hg and pulmonary capillary wedge pressure of 20 mm Hg and a cardiac index of 2.3 liters/min. A left heart catheterization revealed clean coronaries, and alcohol was thought to be the likely etiology of non-ischemic cardiomyopathy in this patient. Urine catecholamine and vanillylmandelic acid were measured and a magnetic resonance angiography of the renal arteries was performed to evaluate for secondary causes of hypertension. This showed non-obstructed renal arteries bilaterally.
A minimally enhancing 22 × 16 × 23 cm mass was visualized in the left upper and mid-abdomen displacing the spleen and stomach posterosuperiorly, the pancreas anteriorly and the left kidney inferiorly (fig. ). The left adrenal gland was not visible. A contrast-enhanced CT scan of the abdomen to further characterize the mass showed no infiltration of the surrounding vasculature or organs, and the left adrenal gland was visualized although it could not be confirmed if the mass was arising from the adrenals (fig. ). The suggested radiological differential diagnoses were: liposarcoma, teratoma and myelolipoma. A needle biopsy of this mass showed adipose tissue with foci of extramedullary hematopoiesis consistent with myelolipoma without any evidence of malignancy, confirming this mass to be a benign myelolipoma (fig. ).
Urinary epinephrine, metanephrine and vanillylmandelic acid were within normal limits. A renal perfusion scan showed a small left kidney with reduced perfusion contributing 22% to the overall tubular function without obstructive uropathy. The lower extremity edema was partly ascribed to be due to inferior vena cava compression, and diuretics were decreased due to worsening renal function. Given the bradycardia and severe congestive heart failure, an electrophysiologist was consulted for evaluation and need for pacemaker implantation. Bradycardia was ascribed to be partly due to the Cushing reflex secondary to elevated blood pressure, and as the patient had no symptoms secondary to a low heart rate, pacemaker implantation was not advised. Despite maximum doses of clonidine, isosorbide, hydralazine, losartan and lisinopril, at best a moderate blood pressure control was achieved preoperatively.
The patient was taken for surgical removal of the mass. During surgery, the mass was found to be soft, highly vascular but well encapsulated and attached only to the left adrenal gland via a small portion that was resected along with the mass (fig. ). There was minimal blood loss during the surgery, and the patient had an uneventful recovery in the postoperative period. A renal perfusion scan done 8 days after surgical removal showed 36% contribution to the overall tubular function. Blood pressure control improved after surgery and the patient was gradually titrated off clonidine, hydralazine and lisinopril. Bradycardia resolved with few episodes of atrial fibrillation with rapid ventricular response that were controlled with β-blockers and digoxin. The antihypertensive medications that the patient was discharged on were carvedilol 40 mg, valsartan 320 mg and isosorbide mononitrate 240 mg.


The patient was a 4-month-old female with a history of prenatal bilateral hydroureteronephrosis. On day 1 of life, the renal ultrasound showed severe bilateral upper pole urinary tract dilation and a large right-sided ureterocele. There was no vesicoureteral reflux (VUR) on a cyclical voiding cystourethrogram (VCUG). The patient was started on antibiotic prophylaxis. At 8 weeks of age, a diuretic renal scan demonstrated the following relative function: right upper pole (RUP), 16%; right lower pole (RLP), 31%; left upper pole (LUP), 24%; and left lower pole (LLP), 29%. However, the formal radiologist interpretation reported that the RUP function was exaggerated because the renal parenchyma could not be easily separated from liver activity. The LUP drained with a postdiuretic T ½ of 16 minutes and the RUP with a T ½ of 71 minutes. The patient was initially scheduled for an upper urinary tract approach due to the absence of VUR. The initial plan was to perform a left upper to lower ureteroureterostomy (UU) because the LUP renal moiety function remained preserved. However, prior to the scheduled surgical date, the patient presented with fever. Urinalysis was consistent with infection. Leukocytosis was noted. Renal ultrasound revealed a large amount of debris throughout the urinary tract (). Incision of the ureterocele and percutaneous LUP nephrostomy tube placement were performed. An ectopic LUP ureteral orifice in the urethra was confirmed. Postoperative VCUG revealed RUP grade 5, RLP grade 3, and LLP grade 2 VUR. Staged upper and lower urinary tract procedures were planned. The surgical plan was determined on the basis of shared decision making with the parents. A robot-assisted laparoscopic left upper to left lower UU and RUP partial nephroureterectomy were scheduled, with plans for lower urinary tract reconstruction at a later date.
Cystoscopy was performed to place a double-pigtail indwelling ureteral stent into the LLP ureter and an open-ended ureteral catheter into the RLP ureter. The patient was repositioned on her flank for a left upper urinary tract procedure. Access was obtained through a midline umbilical incision. One 8.5-mm robotic camera umbilical trocar, one 8-mm trocar below the xiphoid, and one 8-mm trocar in the left lower quadrant were placed (). Our pediatric institution routinely utilizes an 8.5-mm camera because it is smaller. We have noticed that the optics of the 8.5-mm camera are not equivalent to those of the 12-mm robotic camera, but it does not compromise the surgical procedure. The robot was docked ipsilaterally to the side of surgery. The left retroperitoneum was exposed, including reflecting the colon. The lower pole ureter was left in situ, with minimal dissection to preserve the blood supply. Using robotic Potts scissors, a longitudinal ureterotomy was made in the midureter of the lower pole system, matching the diameter of the obstructed upper pole ureter. The upper pole ureter was divided transversely. The upper to lower UU anastomosis was performed by using running 6-0 PDS with a double-armed needle, which was made by tying two sutures together. The suture length was 4 and 6 cm from the needle to the knot for the two arms (). Redundant obstructed midureter was excised. The distal ureters were left undisturbed in the pelvis. The stump of the ectopic upper pole ureter was left open. The trocars were removed and sterile dressings were applied.
The patient was repositioned on her flank for a right upper urinary tract procedure. The umbilical camera and subxiphoid trocars were replaced. A new 8-mm trocar was placed in the right lower quadrant (). The robot was docked ipsilaterally to the side of surgery. The upper pole vessels were identified and divided with a 5-mm LigaSure Blunt (). A percutaneous Prolene suture was placed in the upper pole parenchyma to provide traction and exposure. The upper pole ureter was opened. The floor of the collecting system was identified. The upper pole parenchyma was excised after identifying the avascular plane between the upper and lower pole moieties. The dilated upper pole ureter was mobilized and transected below the level of the lower pole renal hilum. The upper pole ureter was then mobilized from above the hilum, until it was completely delivered, without passing instruments behind the lower pole vessels. The refluxing stump of the upper pole ureter was controlled with two 0-PDS Endoloops. Surgicel and FloSeal were placed on the bed of the resection. Trocar site fascial incisions were closed with 4-0 Vicryl. The RLP ureteral catheter was removed. There were no intraoperative complications or significant blood loss. The total operative time from initial cystoscopy to skin closure was 458 minutes. The console time for UU was 115 minutes and that for partial nephroureterectomy was 145 minutes. A 6-Fr urethral catheter was left indwelling and removed on postoperative day 1. The patient tolerated clamping of the LUP nephrostomy tube on postoperative day 1. No other drains were used. Scheduled postoperative acetaminophen was used for pain management, initially intravenously and subsequently transitioned to oral. Postoperative narcotics or ketorolac were not administered. The patient was discharged on postoperative day 1.
The nephrostomy tube was removed 2 weeks after surgery. The left ureteral stent was removed 6 weeks after surgery (). Postoperative renal ultrasound showed decompression of the left upper system without ipsilateral lower pole hydronephrosis and a well-perfused RLP renal moiety without any significant perinephric fluid collection (). The patient will remain on antibiotic prophylaxis until the lower urinary tract reconstruction is performed after infancy during the toddler years.


A 16-year-old male patient was referred from the pediatrics clinic to our clinic with a diagnosis of 11β-hydroxylase deficiency-driven CAH after the determination of bilateral testicular masses. The patient's history revealed a diagnosis of 11β-hydroxylase deficiency-driven CAH at a young age. Although the patient's laboratory data from an early age were not available, the patient had been using dexamethasone 1×0.75 mg and spironolactone 1×100 mg irregularly.
The physical examination revealed multiple bilateral solid nodules in the testes of approximately 0.5 to 1.5 cm. The results of routine hematologic and biochemical tests and the levels of prolactin, luteinizing hormone, and follicle-stimulating hormone were normal. The testosterone level was at the lower limit (231.6 ng/dL). The values of serum markers for testis tumors, such as alpha-fetoprotein (AFP), beta-human chorionic gonadotropin (βhCG), and lactate dehydrogenase (LDH), were within normal ranges. Cortisol, ACTH, and dehydroepiandrosterone sulfate levels were likewise normal, unlike the elevated levels of 17-hydroxyprogesterone and 11-deoxycortisol, which were 23 ng/mL (<2 ng/mL) and 238 ng/mL (<8 ng/mL), respectively.
Scrotal ultrasonography revealed the following testicular sizes: right, 41 mm×21 mm, and left, 43 mm×22 mm. Both testes contained multiple solid heterogenic hypoechoic nodules of different sizes ranging from approximately 21 mm×15 mm to 14 mm×10 mm with well-drawn borders and increased vascularity as indicated by the arrows (). Scrotal magnetic resonance imaging revealed multiple solid nodules with the largest diameter being 18 mm in the right testis and 11 mm in the left. In T1A the nodules were hyperintense and in T2A they were hypointense. After intravenous gadolinium injection, intense homogeneous contrast accumulation was seen in the nodular lesions with contoured lobes and well-drawn borders (). No pathological findings were visible in the computerized tomography of the abdomen.
Semen analysis showed oligoasthenoteratozoospermia. The patient's sperms were cryopreserved in case he underwent bilateral orchiectomy on the basis of malignant pathological findings. Because the testicular tumor could not be differentiated as malignant or benign on the basis of the laboratory and imaging findings, the patient underwent a right high inguinal testicular exploration. A 1.8-cm nodule was totally excised for frozen section analysis. Because the report of the frozen section analysis was benign, orchiectomy was not performed. After the tunica albuginea was sutured, the testis was placed back into the scrotum.
Histopathological analysis revealed that the cells forming the mass were similar to Leydig cells with separated fibrous tissue; significant, nucleolus, eosinophilic cytoplasm; and polygonal shapes forming in some areas nests and at other areas layers. Mitosis was rare and necrosis was not seen. The cell cytoplasmic fluid contained lipofuscin but no Reinke crystals. Immunohistochemical analysis revealed that the tumor cells totally diffused with vimentin and Melan-A but were focally immunopositive to inhibin and creatine. Placental alkaline phosphatase and Ae1/Ae3 were immunonegative and the tissue was defined as TART (). The patient was diagnosed with TART, glucocorticoid treatment was initiated, and the patient was followed up.


A 3-year and 6-month-old male child presented to us with complaints of intermittent fever for 1 month. The parents complained of refusal for feeds and lethargy. He had a history of polydipsia and polyuria. Antenatal ultrasound scans had identified an absent right kidney with hydronephrosis of the left kidney. His parents had been advised surgery for left pelviureteric junction obstruction at the age of 2 months, but they had refused.
At presentation to us, the child had chronic renal failure with severe metabolic acidosis. A large renal lump was palpable in the left flank. Blood investigations revealed Hb of 6 gm%, total leukocyte count of 15,900/cumm, serum sodium of 116 mmol/L, serum potassium of 2.1 mmol/L, serum creatinine of 2.8 mg/dL and blood urea nitrogen of 97 mg/dL. Urine examination revealed pyuria with significant growth of Escherichia coli. Ultrasound of the kidney revealed absent right kidney with gross hydronephrosis and parenchymal thinning on the left side. Debris was noted in the dilated pelvicalyceal system.
The child was stabilized in pediatric intensive care for fluid and electrolyte imbalance and was administered culture specific antibiotics.
The DTPA scan revealed non-visualization of the right kidney with severely diminished parenchymal uptake on the left side without any excretion over 24 hours.
After stabilization, he was taken for open left pyeloplasty. The kidney was grossly dilated with thinned-out parenchyma. The dilated pelvicalyceal system was full of an unusual, white creamy material with thin flakes, not resembling pus []. The system was also lined by the same material. The flakes were gently aspirated out and sent for culture and histopathology. Anderson-Hyne's dismembered pyeloplasty was performed with a nephrostomy tube. The post-operative course was uneventful. Hisopathology revealed focal areas of mature squamous epithelium with keratinization. The flakes were suggestive of an acellular keratin-forming membranous sheath [].
At a follow-up of 1 year, the child is asymptomatic with normal renal function.


A 69-year-old male presented with intermittent feeling of blurred vision with mild imbalance while walking for 2 days followed by acute loss of vision in both his eyes. He had undergone partial gastrectomy for poorly differentiated adenocarcinoma of the pylorus before 1 month. He had no other health related problems previously. His family history was unremarkable and he had no history of alcohol or tobacco consumption. The immediate post-operative period was unremarkable. Ophthalmologic evaluation revealed total loss of visual acuity with no light appreciation or finger counting with preserved bilateral pupillary reaction to light. There was mild opthalmoparesis with reduced saccadic and pursuit movements toward the sound and patient's own finger movements. There was bilateral horizontal gaze evoked nystagmus with fast component in the direction of gaze. Bilateral optic fundi were normal. He had symmetric trunk and gait ataxia, but no limb ataxia or dysarthria. His alertness, mood, memory, behavior and other cognitive functions were well-preserved. His other systemic and neurologic examination was unremarkable. An urgent plain and contrast magnetic resonance imaging (MRI) of the brain was done to rule out either of posterior circulation stroke (Bilateral Posterior Cerebral Artery infarct), intracranial metastasis and paraneoplastic neurological dysfunction in view of recently operated carcinoma of stomach.
MRI brain revealed bilateral symmetrical hyper-intensities over the mammillary bodies and periventricular regions of the third ventricle, bilateral inferior colliculi on T2-weighted and fluid attenuated inversion recovery (FLAIR) images [Figure , , and ] with homogenous enhancement postcontrast [Figure and ] with no restriction on diﬀusion-weighted and apparent diffusion coefficient (ADC) images (ruling out ischemia/cytotoxic edema). In addition to these classic findings of WE, focal hyper-intensities were also observed in bilateral post-chiasmatic optic tract on FLAIR images []. Based on the imaging findings and clinical background of patient, diagnosis of non-alcoholic acute Wernicke's syndrome was considered and treatment with intravenous thiamine hydrochloride (500 mg/d) was promptly started. His visual symptoms significantly improved in 24 h and his vision became completely normal in 72 h. His ataxia improved gradually over the next 3 weeks. He was discharged with the advice to take intravenous thiamine injections once every month to prevent the thiamine deficiency. A follow-up MRI brain carried out after 1 month revealed complete resolution of MR signal intensities [Figure and ].


A 69-year-old Japanese man was admitted with a 2-month history of dysphagia and tarry stools. The results of physical examination were unremarkable. Routine laboratory tests revealed leukocytosis (11,900 cells/mm3). The tumor marker levels were as follows: SCC antigen, 2.5 ng/ml (normal range, 0~0.5 ng/ml) and CYFRA 21-1, 17 ng/ml (normal range, 0~1.5 ng/ml). Upper gastrointestinal endoscopy revealed erosive esophagitis and a cauliflower-shaped protruding mass located in the gastric cardia. The mass arose from the lesser curvature (). Histological examination of biopsy specimens revealed a well-differentiated SCC. Several biopsy specimens were collected from the gastric mucosa surrounding the tumor. No Helicobacter pylori was detected, but the mucosa of the esophagogastric junction was mildly inflamed. An upper gastrointestinal barium series revealed an elevated mass arising from the lesser curvature of the gastric cardia (). Computed tomography (CT) of the abdomen with intravenous administration of a contrast dye revealed a large heterogeneous mass on the dorsal wall of the stomach, with no evidence of metastatic disease outside the stomach (). Total gastrectomy with splenectomy, distal pancreatectomy, and Rouxen-Y reconstruction was performed, together with a lower thoracic esophagectomy via a left thoracotomy. There was no evidence of regional or distant metastasis. On opening the stomach, an ulcerated mass in the cardia, measuring 7.5×9.0 cm, was observed along the lesser curvature (). Pathological examination of sections of the resected specimen stained with hematoxylin and eosin revealed a well-differentiated SCC with frequent typical keratin pearl formation, mosaic patterns of cell arrangement, and intracellular bridges (). The esophageal and pyloric margins were free of tumor involvement, but tumor cells infiltrated the serosa of the dorsal margin. No nodal metastasis was apparent. The tumor had not invaded the mucosa of the esophagogastric junction. In addition, no squamous cells were detected in the normal portion of the stomach. The clinical stage was stage IIB (T4aN0M0), according to the TNM classification, version 7. Therefore, primary SCC of the stomach was diagnosed. The patient refused adjuvant chemotherapy. However, 18-months after the operation, a CT scan revealed a metastatic tumor that was 4.0 cm in diameter in segment 6 of the liver, accompanied by para-aortic lymph node metastasis. Although the patient immediately received systemic chemotherapy with 5-fluorouracil plus cisplatin and docetaxel plus cisplatin, the recurrent tumors progressed. Liver metastases developed subsequently, and the patient died 36 months after the first operation.


A 35-year-old female HIV patient presented with headache and altered mental status. She was referred for whole body PET/CT for detection of involvement of CNS and other sites. PET/CT showed an intense uptake in the multiple lesions noted in both the cerebrum [], with a standardized uptake value (SUV max) of 30-35. A diagnosis of primary CNS lymphoma was made and she was started on chemotherapy.


A 23-year-old female patient presented in April 2012 with a flare of psoriasis, involving the trunk, extremities and the scalp. She had a similar flare at the age of 13, and also history of patch-type scalp AA, both resolved following topical steroid treatment. Two months later, in June 2012, she experienced localized hair loss on the scalp, concomitantly with complete resolution of the psoriatic plaque in this region. On examination, a 3 cm patch of nonscarring hair loss was observed, and dermoscopic examination revealed perifollicular pigmentation, exclamation hairs and yellow dots, corresponding to AA. Interestingly, the psoriatic plaque engulfed the AA area, but sharply stopped at its border []. The patient was treated topically for her AA with clobetasol propionate, with stabilization of hair loss when the patch reached a size of 5 cm × 6 cm in September 2012. In parallel, the patient had reappearance of psoriatic plaques inside the area of hair loss, which coincided with the presence of terminal hairs localized to these psoriatic plaques [].