Abstract
A 76-year-old woman was referred to our hospital because of a one-month history of fever, weight loss and intermittent abdominal pain. Abdominal computed tomography scan showed an intussusception of the small intestine caused by a tumor, and emergency ileal resection was performed. The tumor which was solid and 65×40×30mm in diameter and had a smooth surface was found at the site of intussusception. Histopathological findings showed spindle-shaped tumor cells with prominent unclear atypia and abnormal mitosis. Immunohistochemical study showed tumor cells to be positive for α-SMA and Desmin, but negative for KIT, CD34, S-100 and DOG1. In accordance with the GIST treatment guidelines and the WHO Classification of Tumors of the Digestive System, small bowl leiomyosarcoma was diagnosed. We analyzed for genetic mutation of c-kit and PDGFRA to rule out a GIST, and the mutation was not identified. Therefore, the tumor was definitely diagnosed as ileal leiomyosarcoma. Small bowel leiomyosarcoma is a rare entity and should be diagnosed using immunohistochemical and genetic studies. The prognosis is poor even with surgical resection.
