Intra-abdomen Ewing's sarcoma

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.

Ewing sarcoma of the liver with multilocular cystic mass formation: a case report

“…To our knowledge, only three published case reports discuss ES arising in the liver [ 6 – 8 ] and because of the rarity of this entity, little information on the imaging features is available. Two previous case reports described very large solid tumors without cystic lesions on CT [ 6 , 7 ], and a third case report described an enlarged liver without mass lesion on CT scan [ 8 ]; CT scan in our case showed a multilocular cystic lesion. These findings indicate that CT scans in patients with ES arising in the liver can reveal a polycystic tumor, solid tumor, or an enlarged liver without mass lesion.…”

An Unusual Location of Extraosseous Ewing's Sarcoma

“…To our knowledge, this entity has only been described once [13]. Other cases with an EO ES in the mesocolon [14], a male with an acute abdominal EO ES next to the left colon [37], EO ES in the liver [38] and EO ES in the small intestine [10, 39] have been described. This case indicates that EO ES should be included in the differential diagnosis of lesions arising in the omentum.…”

Primary hepatic Ewing sarcoma: a very infrequent case report

“…To the best of our knowledge there were four hepatic ES cases in English literature. [5][6][7][8] In our case she had multiloculated cystic liver lesion and diagnosed by CT scan and biopsy. In the English literature this was the second case of primary hepatic ES, which was presented with multiloculated liver cystic lesion.…”

“…There were two cases of hepatic ES in the literature which were presented with hepatic solid mass lesion. 6,8 The case reported by Cambruzzi et al was an 18-year-old male showing solid hepatic mass with the complaints of abdominal pain. Preoperative hepatic artery embolization was performed and then the patient was operated for hepatic resection.…”

“…Pathological specimen should be evaluated with immunohistochemical markers including, CK, CD138, a WT1, synaptophysin, muscle specific actin, desmin, myogenin, LCA and leukocyte common antigen (CD45) to exclude other small round cell tumors including neuroblastoma, myeloma, desmoplastic small round cell tumor, mesenchymal chondrosarcoma, alveolar rhabdomyosarcoma, non-Hodgkin's lymphoma and small cell osteosarcoma. [8][9][10] Neuroblastoma shows immunoreactivity to NSE, S-100 and Leu-7 like ES, but negative for vimentin. Lymphoblastic lymphoma is immunoreactive to CD99 but different in that it tests positive to CD45 and Tdt.…”