GIST (neural type) of the Jejunum Causing Intraperitoneal Hemorrhage -A Case Report in a Patient with von Recklinghausen Disease-

Eiichi Kihana, Shigeru Kurisu, Takeshi Hatta, Takashi Koyama, Yasuhumi Kita, Masahiko Umeki, Masaki Shibata

Department of Surgery, Hyogo Prefectural Awaji Hospital

A previously healthy 25-year-old man, with multiple café au lait spots, presented with vomiting and a sudden onset of abdominal pain. Ultrasound examination and a CT scan showed a 6×7 cm tumor in the pelvic cavity. At laparotomy, the tumor, which protruded from the subserosa of the jejunum, 20 cm on the anal side from Treitz's ligament, was excised. The tumor was well encapsulated but partly ruptured and bleeding. Further inspection revealed three other tumors 0.5 cm in diameter, located in the jejunum, 60, 70 and 100 cm apart from Treitz's ligament. Histologically, the main tumor was composed of spindle-shaped cells with an interweaving bundle pattern. The three small tumors showed the same histological findings. Immunohistochemically, the tumor cells were partly positive for S-100 protein and for NSE, but negative for desmin and for smooth muscle actin. From the above evidence the tumor was diagnosed as a gastrointestinal stromal tumor (GIST), neural type, low grade malignant.

Key words
von Recklinghausen disease, GIST

Reprint requests
Eiichi Kihana Department of Surgery, Hyogo Prefectural Awaji Hospital
1-6-6 Shimogamo, Sumoto, 656 JAPAN

Accepted
September 9, 1997

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