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

Takaaki Eto, Makoto Takahashi, Yoshio Miura, Hideto Sakimoto, Norio Masumoto, Minoru Yamaki, Masahiro Ohira, Midori Sasaki and Toshihiro Nishida^*

Department of Surgery and Pathology^*, Chugoku Rosai General Hospital

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, 12 cm 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.

Key words
perforative malignant schwannoma, small intestine, von Recklinghausen's disease

Reprint requests
Takaaki Eto Department of Surgery, Chugoku Rosai General Hospital 1-5-1 Hirotagaya, Kure, 737-0193 JAPAN

Accepted
April 30, 2003

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