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

Abstract

BackgroundEwing sarcoma is a rare tumor that occurs commonly in the long bones of children or adolescents that can also arise in soft tissues including the extremities, retroperitoneum, chest wall, and rarely in the liver as primary sites. We report a case of Ewing sarcoma arising primarily in the liver and, to our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver.Case presentationA 27-year-old Japanese woman was admitted with sudden onset right upper abdominal pain. Clinical examination revealed a multilocular cystic mass consisting of thickened, irregular septa and nodal walls in the right hepatic lobe. Ultrasound-guided aspiration biopsy of the liver mass showed clusters of small atypical round cells and the clinical preoperative diagnosis was mucinous cystadenoma of the liver. The patient underwent an extended right hepatectomy and histopathological findings revealed sheet-like proliferation of small- to medium-sized round cells. Tumor cells were positive for periodic acid-Schiff reaction and immunoreactive for glycoprotein C99 and gene NKX2.2, as well as the neuroendocrine markers, CD56 and synaptophysin. EWS-FLI-1 fusion transcript type 1 was detected by reverse transcriptase polymerase chain reaction. Pathological and molecular analysis confirmed the diagnosis of Ewing sarcoma arising primarily in the liver and the patient received adjuvant systemic chemotherapy with vincristine, doxorubicin, and cyclophosphamide, alternating with ifosfamide and etoposide. We found no evidence of recurrence 15 months after completing chemotherapy.ConclusionWe present an extremely rare case of Ewing sarcoma arising primarily in the liver. To our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver, and the first case with a multilocular cystic liver mass. Imaging examinations of the other three reported cases showed solid tumors and a diffuse enlarged liver without mass lesion. Clinicians should consider the possibility of Ewing sarcoma in young patients with a multilocular cystic mass with thick and/or irregular cyst walls in the liver.

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.…”

“…Ozaki et al reported the first hepatic multiloculated cystic lesion diagnosed with hepatic ES. 7 In that case a 27-year-old Japanese woman was admitted with abdominal pain. Multiloculated cystic lesion on liver was revealed with clinical examination.…”

Extraoseus Ewing‘s Sarcoma, Primary Affection of Uterine Cervix – Case Report

“…V literatuře se nejčastěji popisuje postižení ledvin, Zöllner et al evidují 152 případů [3], dále např. plic [4], žaludku [5], jater [6], urologických [7] či gynekologických orgánů.…”

Ewing Sarcoma of the Jejunum: A Case Report and Literature Review.

“…Extra skeletal cases are rare, and these patients generally present at an older age and demonstrate a greater overall 5-year survival than skeletal Ewing sarcoma tumours [14,15]. Reports of primary liver involvement have been noted, as well as gastrointestinal sites of origin, including the stomach, small intestine, and colorectal [16][17][18][19]. Nevertheless, ES is extremely rare in the small bowel.…”