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Accession number;03A0146672
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| Title;A Case of Jejunal Gastrointestinal Stromal Tumor (GIST) Associated with Von Recklinghausen's Disease. |
| Author;
WATABE TOMOO
(Tobuchiikibyoin Geka)
KIHARA AKIRA
(Tobuchiikibyoin Geka)
KOMIYAMA HIROMITSU
(Tobuchiikibyoin Geka)
SENGOKU HIRONOBU
(Tobuchiikibyoin Geka)
NOGUCHI HAJIME
(Tobuchiikibyoin Geka)
OCHIAI TAKUMI
(Tobuchiikibyoin Geka)
NISHIMURA KAZUHIKO
(Tobuchiikibyoin Geka)
SUGITANI MICHIHARU
(Tobuchiikibyoin Geka)
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Journal Title;Gastroenterol Endosc
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Journal Code:G0608B
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ISSN:0387-1207
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VOL.45;NO.2;PAGE.162-167(2003)
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| Figure&Table&Reference;FIG.7, TBL.1, REF.17 |
| Pub. Country;Japan |
| Language;Japanese |
| Abstract;We report a case of jejunal GIST with von Recklinghausen's disease. A 35 year-old woman was admitted to our hospital with symptoms of melena and exertional dyspnea. She was pointed with severe anemia on hematological examination. She had had multiple cafe au lait spots and papules in the whole body since her childhood. The skin lesion was neurofibromatosis on histological diagnosis. Fluoroscopic and endoscopic examinations of the small intestine revealed a submucosal jejunal tumor, 3 * 2cm in size, which located 7cm anal distal to Treitz's ligament. Partial resection of the jejunum was performed. Histologically, the tumor was composed of uniform spindle-shaped cells with fascicular pattern and no mitotic figures. While, on immunohistochemistry, tumor cells were positive for CD34 and c-kit, and negative for .ALPHA.-SMA and S100 protein. Therefore, the final histological diagnosis was an uncommitted type of GIST with uncertain malignant potential. Most of non-epitherial tumors associated with von Recklinghausen's disease are neurogenic tumors. On the other hand, GIST is too rare as the complication of von Recklinghausen's disease. Only 10 GIST cases related to von Recklinghausen's disease has been reported in Japan including this one. However, this clinical experiences emphasize that GIST of the small intestine should be considered as an important possible complication of von Reckliughausen's disease patients with anemia and/or melena. (author abst.) |
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