Ewing sarcoma of the uterus: A case report

A case is described of Ewing sarcoma of the uterus, an atypical presentation of an already rare cancer. A 55-year-old woman presented with abdominal pain, abnormal uterine bleeding and a uterine mass that measured 11 × 10 × 14.5 cm and demonstrated heterogeneous enhancement with possible areas of central necrosis, concerning for sarcoma. She had a complete surgical resection with total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, bilateral pelvic lymph node dissection, and excision of mesenteric tumor implants. Her final pathology showed primary Ewing sarcoma-primitive neuroectodermal tumor of the uterus with metastatic spread to the peritoneal cavity. She finished 14 cycles of vincristine-doxyrubicin-cyclophosphamide–ifosfamide, etoposide chemotherapy with no evidence of recurrent metastatic disease at 6-month follow-up. Ewing sarcoma is a rare cancer, predominantly seen in adolescents, that typically are of the bone, although in rare instances it can arise from soft tissue; even rarer are presentations in the female genital tract. Even with typical presentations of Ewing sarcoma of the bone, metastatic disease has an overall poor prognosis. The scarcity of cases of metastatic Ewing sarcoma–peripheral neuroendocrine tumors of the uterus makes the condition especially difficult to study. This report describes a case of Ewing sarcoma of the uterus treated by complete surgical resection and aggressive multimodal chemotherapy. •Ewing sarcoma of the uterus is an atypical presentation of an already rare cancer.•The incidence of extraosseous sites of Ewing sarcoma is 0.4/1,000,000.•Due to the rarity of neuroendocrine tumors of the gynecologic tract, it is difficult to histologically differentiate between central and peripheral primary neuroendocrine tumors.•There is no set standard of care for primary neuroendocrine tumors in the uterus, but treatment protocols might differ between central- and peripheral-type tumors.•In the case reported, patient was treated with full surgical excision and vincristine/doxorubicin/cyclophosphamide–ifosfamide/etoposide (VDC-IE) every 2 weeks for 14–17 cycles.