von Recklinghausen病に合併した小腸多発gastrointestinal stromal tumorの1例

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  • A CASE OF AN ILEUS BY MULTIPLE GASTROINTESTINAL STROMAL TUMORS OF THE SMALL INTESTINE IN PATIENT WITH VON RECKLINGHAUSEN'S DISEASE

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A 59-year-old man was admitted to our hospital because of vomiting. Abdominal CT, MRI and X-Ray studies showed multiple tumors in the abdomen causing small bowel obstruction so he went through an operation. In the abdomen, there were many tumors with diameters from 5mm to 10cm on the small bowel: from the deuodenum to the ileum. In particular, a 10cm tumor was strangling the small bowel. We performed resection of the segment of small bowel where larger tumors concentrated, avoiding a massive resection of the small bowel to preserve the quality of life of the patient. Pathologically the tumors were vimentin(++), CD34(++), C-kit(++), α-SMA(-) and S100(-)_??_(±), accordingly they were thought to be uncommitted type of GIST. Their degree of malignancy was considered to be borderline because of the presence of few cell divisions in spite of large size of the tumors. After the operation, he was prescribed imatinib mesylate. We could not find growing tumors or metastasis during the follow up of 22 manths.

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