Report of Two Cases of Neurofibromatosis Type 1 Associated with Multiple Gastrointestinal Stromal Tumors and a Neuroendocrine Tumor of the Small Intestine

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  • 小腸GISTと神経内分泌腫瘍を併発した神経線維腫症1型の2例
  • 症例 小腸GISTと神経内分泌腫瘍を併発した神経線維腫症1型の2例
  • ショウレイ ショウチョウ GIST ト シンケイ ナイブンピ シュヨウ オ ヘイハツ シタ シンケイ センイ シュショウ 1ガタ ノ 2レイ

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Abstract

Case 1 : A 42-year-old woman with neurofibromatosis type 1 (NF1) was admitted to the hospital for close exploration of anal bleeding. Endoscopic study showed a submucosal tumor and an elevated lesion just distal to the tumor in the small intestine. Bleeding from the jejunal mass was diagnosed. Partial resection of small intestine was performed, when a nodule at the small bowel mesentery was detected and removed. The resected specimen had two tumors which were diagnosed as gastrointestinal stromal tumor (GIST) and neuroendocrine tumor (NET), respectively. The mesenteric nodule was GIST as well.<BR>Case 2 : A 73-year-old woman with NF1 visited our hospital for further examination of hypertension, and small bowel masses were detected on an abdominal CT scan. Capsule endoscopy revealed a tumor in the duodenum and three tumors in the small intestine. Laparoscopic-assisted partial resection of small intestine was performed. The duodenal mass was not resected because the patient's general condition was poor. The resected intestine had three tumors. Two of those were diagnosed as GIST and the remaining one was diagnosed as NET.<BR>Although clinical cases of NF1 combining with either GIST or NET are sometimes reported, both diseases occurred concomitantly in those patients are very rare. We thus report our two cases of NF1 with both GIST and NET of the small intestine, with some bibliographic comments.

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