A Case Report of Perforative Jejunal Malignant Schwannoma Associated with von Recklinghausen's Disease

Abstract

We present a rare case of perforated jejunal malignant schwannoma associated with von Recklinghausen's disease. A 39-year-old woman admitted with severe abdominal pain was found in chest radiography to have free air under the diaphragm, and abdominal computed tomography showed a tumor, 12cm in diameter, with internal necrosis and air, necessitating emergency surgery based on a diagnosis of panperitonitis caused by perforation tumor. The perforated tumor protruded from the jejunum, necessitating partial resection of the jejunum together with the tumor. A small perforation was found between the resected jejunum and tumor. Histologically. the tumor consisted of spindle cells showing a nuclear palisading and abnormal mitotic figures. The tumor was immunohistochemically positive for S-100 protein, but negative for actin. In Japan, 12 cases of small intestinal malignant schwannoma associated with von Recklinghausen's disease were reported between 1955 and 2002. Only 2 cases, including ours, involved perforation of the schwannoma and a perforation between the jejunum and schwannoma. The remaining 10 cases did not involve any perforation and 1 case involved perforation of the jejunum. We suggest that perforation of the small intestinal malignant schwannoma was caused by perforation from the small intestine to malignant schwannoma.