Cystic Gastrointestinal Stromal Tumor associated with von Recklinghausen's Disease Mimicking Ovarian Tumor -A Case Report-

Hajime Noguchi, Michiharu Sugitani, Kazuhiko Nishimura, Takumi Ochiai, Tuyoshi Okada, Masayuki Kitajima, Hirohiko Kamiyama and Tadao Okano*

Department of Surgery, Toubu Chiiki Hospital, Tokyo Metropolitan, Health and Medical Treatment Corporation
Division of Clinical Laboratory, Pathology, Toubu Chiiki Hospital, Tokyo Metropolitan, Health and Medical Treatment Corporation*

We report a unique case of "cystic" gastrointestinal stromal tumor (cystic GIST) associated with von Recklinghausen's disease. A 61-year-old woman with abdominal distension deteriorating for three months underwent laparotomy for removal of a cystic pelvic mass suspected of being an ovarian cyst. An 11 cm long cystic tumor was found originating from the small intestine 100 cm rostral to Bauhin's valve. The tumor and part of the intestine were resected and an end-to-end anastomosis performed. The postoperative course was uneventful. Pathological examination showed a GIST with positive staining for c-kit, CD34, Vimentin, and α-smooth muscle actin, and negative staining for S-100 and Desmin. To the best of our knowledge, this is the first case of cystic gastrointestinal stromal tumor associated with von Recklinghausen's disease to be reported in the Japanese literature.

Key words
von Recklinghausen's disease, gastrointestinal stromal tumor

Reprint requests
Hajime Noguchi Department of Surgery, Tobu Chiiki Hospital, Tokyo Metropolitan Health and Medical Treatment Corporation
5-14-1 Kameari, Katushikaku, Tokyo, 125-8512 JAPAN

Accepted
January 28, 2004

To read the PDF file you will need Abobe Reader installed on your computer.