CLO21-015: A Unique Case of Duodenal Extraosseous Ewing Sarcoma in an Elderly Filipina

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  • 1 San Antonio Military Medical Center, San Antonio, TX

Introduction: Although it typically presents as a cancer of the long bones in Caucasian boys, Ewing sarcoma may rarely arise in the soft tissue of older females. This family of small, round, blue cell tumors share distinct chromosomal translocations involving the Ewing sarcoma gene on chromosome 22. Approximately 70 percent of patients affected by localized disease survive for at least 5 years, but this is usually in young patients. Based on numerous reports, adults may have less favorable outcomes. In this presentation, we describe a unique occurrence of extraosseous Ewing sarcoma arising in the duodenum of a 66-year-old Filipina. Description: A 66-year-old female presented with abdominal pain, vomiting, hypotension and weight loss. On arrival to the ED, her hemoglobin was 5.4 g/dL and CT demonstrated a localized 10.3 x 8.8 x 12.3 cm mass arising from the superior wall of the third portion of the duodenum. Endoscopic biopsy showed a round cell tumor and FISH indicated a rearrangement of the EWSR1 locus at 22q12. She underwent gastrojejunostomy which relieved her obstructive symptoms, but was complicated by persistent bleeding from her ulcerative mass. Radiation therapy led to resolution of her bleeding and neoadjuvant chemotherapy with vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC/IE) was initiated. Interval CT obtained for evaluation of complications in the fourth week of chemotherapy indicated a significant decrease in mass volume. Curative intent therapy is ongoing. Discussion: Ewing sarcoma is predominantly an osseous tumor which affects children. This case presentation features a very uncommon occurrence of gastrointestinal extraosseous Ewing sarcoma in an elderly Filipina. Neoadjuvant radiation and chemotherapy resulted in interval mass volume decrease within a month of initiation of VDC/IE. While many reports link unfavorable outcomes to older age, the Ewing sarcoma family of tumors tend to respond to chemotherapy. Fit elderly patients who can tolerate reasonably high doses of alkylating agents may have similar survival to the pediatric population.

Corresponding Author: Julie R Heitz, MD
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