Primary hepatic Ewing sarcoma: a very infrequent case report

Abstract

Ewing sarcoma (ES) is a member of small round cell tumors of which contains Wilms' tumor, neuroblastoma, rhabdomyosarcoma and lymphoblastic lymphoma. ES occurs most commonly in the bone but infrequently occurs in the soft tissues without involvement of the bones. Primary involvement of the liver is even rarer. We report the case of 24-year-old female patient with abdominal pain. Abdominal computed tomography scan showed hepatic large cystic lesion. Liver biopsy showed tumoral cells with small narrow stoplasmic and hyper chromatic nucleus evaluated as small round cell tumor. Immunohistochemical study of the lesion revealed CD99 and bcl-2 positive, cytokeratin, LCA, CD138, chromogranin, synaptophysin, myoD1, terminal deoxynucleotidyl transferase and myoglobin negative the diagnosis primary hepatic ES. A research for any other tumor involvement by using positron emission tomography yielded only hepatic involvement revealed. The patient treated with vincristine-cyclophosphamide-adriamycin and ifosfamide-etoposide alternately chemotherapy then hepatic tumor regressed. Patient operated on hepatic wedge resection. She has continued on chemotherapy and she has been doing well at the sixth month of diagnosis.

Primary Ewing sarcoma of the liver: Diagnosis, management, and prognosis: A case report and literature review

“…We could find only 11 cases of ES/PNET primarily involving the liver (Table 1). [4][5][6][7][8][9][10][11][12][13][14] Visceral involvement of Ewing's sarcoma family of tumors has been described in the kidney, gastrointestinal tract (small bowel and rectum), pancreas, gall bladder, vagina, vulva, uterine cervix, breast, ovaries, urinary bladder, ureter, prostate, seminal vesicles, testis, penis, maxillary sinus, trachea, lung, and parotid gland. 16 The liver is a common site of ES/PNET metastasis but it is exceptionally a primary location.…”

“…In adults, ESFT arises in more than 50% of cases in soft tissues (trunk, retroperitoneum, intra‐abdominal tissues, and viscera). The primary involvement of the liver is extremely rare, and only 11 cases have been reported 4–14 . These tumors are aggressive, with a high tendency to relapse and metastasize especially in the lungs, bone marrow, brain, and lymph nodes 15 .…”

“…In adults, ESFT arises in more than 50% of cases in soft tissues (trunk, retroperitoneum, intra-abdominal tissues, and viscera).The primary involvement of the liver is extremely rare, and only 11 cases have been reported. [4][5][6][7][8][9][10][11][12][13][14] These tumors are aggressive, with a high tendency to relapse and metastasize especially in the lungs, bone marrow, brain, and lymph nodes. 15 The present work aims to report a new case on primitive hepatic ES and review all cases of primitive hepatic ES/PNET reported in the literature and describe clinical, radiological, histological, cytogenetical, therapeutic, and prognosis features of this singular tumor site in the different cases.…”