A case of neuroendocrine tumor (NET) of the ampulla of Vater and gastrointestinal stromal tumor (GIST) of duodenum with neurofibromatosis type 1 (NF1)

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  • 神経線維腫症1型に合併した十二指腸乳頭部神経内分泌腫瘍および十二指腸gastrointestinal stromal tumorの1例

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Abstract

<p>We describe the case of a 71-year-old woman with neurofibromatosis type-1 (NF1) diagnosed with a neuroendocrine tumor (NET) of the ampulla of Vater and a gastrointestinal stromal tumor (GIST) of the duodenum. The patient was admitted to our hospital and presented with multiple cutaneous neurofibromas and café-au-lait spots on the face and body. Esophagogastroduodenoscopy revealed an exposed protruding tumor of the Vater and a submucosal tumor in the descending part of the duodenum. CT showed both tumors, but somatostatin receptor scintigraphy visualized no tumor in the duodenum. The patient was diagnosed with NET of the Vater and GIST of the duodenum based on histopathological examination of biopsy specimens, and subsequently underwent subtotal stomach-preserving pancreaticoduodenectomy with regional lymph node dissection. Histopathological examination after the procedure confirmed NET (G1) of the Vater with lymph node metastases and GIST of the duodenum.</p>

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  • Tando

    Tando 33 (4), 734-743, 2019-10-31

    Japan Biliary Association

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