No abstract

An 18-year-old male adolescent presented with sudden onset, colicky epigastric pain. On physical examination, tenderness was over right upper quadrant and Murphy's sign was positive. No loco-regional swelling or tenderness was noted in the extremities. His medical history was otherwise unremarkable. Preliminary laboratory investigations revealed leukocytosis of 14.02 10 3 /mL (normal range, 5-10 10 3 /mL), serum lactate dehydrogenase of 235 U/L (normal range, 45-90 U/L), serum bilirubin of 1.5 mg/dL (normal range, 0.4-2.0 mg/dL) and alkaline phosphatase of 88 U/L (normal range, 60-120 U/L). Serum alanine transaminase and r-glutamyl transpeptidase values were normal. Viral serology results for hepatitis B and hepatitis C were negative. Transabdominal ultrasound revealed heterogenic hepatic tumour 10.4 ¥ 5.7 cm in size and moderate ascites. Contrast-enhanced computed tomography revealed a huge lobulated solid tumour 14 ¥ 11 ¥ 10 cm in size over segments 5 and 6 of the right lobe of liver. There is fluid accumulation in the subhepatic space. Normal appearance of gall bladder without stone formation and compression of the pancreatic head by the mass were noted as well (Fig. 1). Cystadenocarcinoma of gall bladder versus hepato-cellular carcinoma was the preoperative differential diagnosis. Intra-operative findings revealed hemoperitoneum and an intra-abdominal tumour with rupture over subhepatic space, connecting to the gall bladder and liver. During dissection, the tumour was found to be partially encapsulated and easily dissected from the retro-peritoneum, adrenal gland and kidney (Fig. 2). As the frozen section reported malignancy, the patient underwent cholecystectomy, total resection of the tumour, hepato-duodenal lymph node dissection and omentectomy. His postoperative recovery was uneventful and was discharged on the 7th post-operative day. Pathology report: grossly, the tumour measured 13 ¥ 8 ¥ 7 cm in size, grey and mildly firm in consistency, focal areas revealed massive necrosis (Fig. 3). On microscopic examination, the tumour composed of hypercellular small blue round cell tumour cells. Ewing's sarcoma was confirmed by periodic acid-schiff (PAS) staining (+), neuron-specific enolase (NSE) (+) (Fig. 4) and reverse transcription polymerase chain reaction (RT-PCR) result of translocation t (11; 22) (q24; q12). There was no extraskeletal lesion found on bone scan. Subsequent adjuvant combination chemotherapy was received at the oncology department. Fig. 1. Contrast-enhanced computed tomography showed fluid accumulation in the subhepatic space and normal appearance of gall bladder without stone formation (arrow) and compression of the pancreatic head by the mass (arrowhead).Fig. 2. A partially encapsulated intra-abdominal tumour with rupture over subhepatic space (arrow), connecting to the gall bladder (G) and liver (L). Fig. 3. The tumour measured 13 ¥ 8 ¥ 7 cm in size, grey and mildly firm in consistency (arrow); focal area revealed massive necrosis (arrowhead). G, gall bladder.