A Case of Epstein-Barr Virus (EBV)-associated Multiple Small Intestinal Leiomyosarcoma

Abstract

A 60-year-old woman who had been attending the outpatient's clinic of nephrology in our hospital because of systemic scleroderma and microscopic polyangiitis developed melena. Gastrointestinal endoscopy showed no abnormal findings, but a small intestinal capsule endoscopy revealed a submucosal tumor with ulceration in the ileum and a submucosal tumor on its anal side. The patient was referred to our department for uncontrollable bleeding with severe anemia (Hb 4.1 g/dL). Intraoperative findings revealed submucosal tumors 30 cm and 100 cm distant from the Bauhin valve. Intraoperative endoscopy revealed no findings other than the two lesions. A partial resection of the small intestine was performed including both lesions. Histopathological examination revealed spindle-shaped tumor cells forming a bundle and cord-like arrangement and positive for Epstein-Barr virus-encoded RNA in situ hybridization (EBER-ISH). EBV-associated small intestinal leiomyosarcoma is a rare disease and we report this case with a review of the literature.