A Case of von-Recklinghausen's Disease with Multiple Gastrointestinal Stromal Tumors (in the narrow sense) in The Small Intestine

Hiroyuki Takamura, Noboru Nagai, Ken Hasebe, Masaaki Urade, Miwa Imai^*and Masao Yagi

Department of Surgery, Public Central Hospital of Mattoh Ishikawa
Department of Health Science, Ishikawa Prefectural Nursing University^*

We report a case of von-Recklinghausen's disease with multiple gastrointestinal stromal tumors (GIST) causing massive intestinal bleeding. A 46-year-old man admitted for massive melena with severe abdominal pain underwent surgery after angiography and CT-angiography (CT-A) showed hemorrhagic multiple hypervascular tumors in the jejunum and ileum. Laparotomy showed conglomerates of dumbbel submucosal tumors (SMT) in the jejunum and ileum and confirmed melena was caused by hemorrhage from one of the SMTs. We resected these tumors, finding some small SMTs in the small intestine that we also resected. All were found to be GIST in the narrow sense, being immunohistochemically positive for the KIT receptor, vimentin, and CD34, and negative for muscle and neural markers. The comparatively larger tumors showed low-grade malignancy with mitotic 1∼5/10 HPF. This case is remarkable for being complicated by von-Recklinghausen's disease and for the multiple small intestinal GIST, with comparatively larger tumors showing low-grade malignancy.

Key words
von-Recklinghausen's disease, gastrointestinal stromal tumor

Reprint requests
Hiroyuki Takamura Department of Emergency and Clinical Medicine, School of Medicine, Kanazawa University
13-1 Takara-machi, Kanazawa, 920-8641 JAPAN

Accepted
March 27, 2002

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