A Case of Neurofibromatosis Type 1 Associated with Rectal Cancer with Pagetoid Spread, Gastric Cancer, and Small Intestinal GIST

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  • Ikeda Jun
    Department of Digestive Surgery, Japanese Red Cross Kyoto Daiichi Hospital
  • Shimomura Katsumi
    Department of Hepato-biliary-pancreatic Surgery, Japanese Red Cross Kyoto Daiichi Hospital
  • Urata Yoji
    Department of Pathology, Japanese Red Cross Kyoto Daiichi Hospital

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  • 神経線維腫症1型にPagetoid spreadを伴う直腸癌に，胃癌と小腸GISTを合併した1例

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Abstract

<p>A 78-year-old woman with neurofibromatosis type 1 had difficulty in defecation and perianal itching, and presented to our hospital for surgery with a diagnosis of rectal cancer with perianal skin lesions and gastric cancer. Skin biopsy revealed adenocarcinoma with pagetoid spread. Small intestinal gastrointestinal stromal tumor (GIST) was also suspected due to a movable intra-abdominal mass lesion in addition to gastric and rectal tumors. Laparoscopic abdominoperineal resection, small intestinal local resection, and perineal skin resection with femoral flap reconstruction were performed. The pathological findings showed rectal adenocarcinoma with focal neuroendocrine differentiation, pagetoid spread on perianal skin from the rectal tumor, and small intestinal GIST. In secondary surgery, laparoscopic distal gastrectomy was performed. This was an extraordinarily rare case of neurofibromatosis type 1 with small intestinal GIST associated with rectal cancer with pagetoid spread and gastric cancer.</p>

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