A Case of von Recklinghausen's Disease Complicated by Carcinoma of the Papilla of Vater and Multiple Gastrointestinal Stromal Tumors of the Jejunum

真一, 生田; 智明, 安井; 司, 相原; 秀範, 吉江; 俊宏, 坂東; 正夫, 光信; 綾子, 杉原; 若樹, 山中

doi:10.5833/jjgs.40.599

Cited by 3 publications

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“…GIST is thought to increase by the activation of tyrosine kinase of the Kit receptor through mutation of the protooncogene c-Kit gene [ 4 ]. To the best of our knowledge, only 28 cases of GIST associated with NF-1 have been reported, and they are summarized in table 1 [ 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. There were 14 males and 14 females (male/female ratio 1.0).…”

Section: Discussionmentioning

confidence: 99%

Duodenal Gastrointestinal Stromal Tumor Treated by Wedge Resection in a Patient with Neurofibromatosis Type 1: Report of a Case and Review of the Japanese Literature

Takeuchi

Matsumoto²,

Kusumoto³

et al. 2009

Case Rep Gastroenterol

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A case of duodenal gastrointestinal stromal tumor (GIST) treated by wedge resection in a patient with neurofibromatosis type 1 (NF-1) is reported. A 55-year-old man with a history of NF-1 was admitted for surgery for a duodenal tumor. Upper gastrointestinal endoscopy revealed a 2.5 cm duodenal submucosal tumor. Abdominal computed tomography showed a homogenously enhanced mass in the third portion of the duodenum. The patient successfully underwent wedge resection of the duodenal tumor. Histological examination revealed proliferation of spindle tumor cells arranged in a bundle pattern. This tumor was immunohistochemically positive for c-Kit and CD34, and negative for S-100 and α-SMA. A mitotic count showed 3 mitoses per 50 high-power fields. The tumor was diagnosed as a low-risk GIST. The patient’s postoperative course was uneventful. GIST in a patient with NF-1 is rare, only 27 cases being reported in the Japanese literature.

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