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제1형 신경섬유종증 환자에서 신경내분비종양과 위장관기질종양 2예
최원영 ( Won Young Choi ),홍승덕 ( Seung Dok Hong ),김하나 ( Ha Na Kim ),최유덕 ( Yoo Duk Choi ),황준일 ( Jun Eul Hwang ),심현정 ( Hyun Jeong Shim ),배우균 ( Woo Kyun Bae ) 대한내과학회 2011 대한내과학회지 Vol.81 No.6
Patients with neurofibromatosis type 1 (NF1) are at increased risk of developing tumors throughout the gastrointestinal tract, including neuromas, gastrointestinal stromal tumors (GISTs), and periampullary somatostatin-rich carcinoids. The simultaneous occurrence of a GIST and a well-differentiated neuroendocrine carcinoma in a patient with NF1 is very rare. Here, we report two cases of the coexistence of a low-risk GIST in the jejunum with a well-differentiated neuroendocrine carcinoma in the duodenum in patients with NF1. These cases strengthen the known association of GIST with neuroendocrine carcinoma in patients with NF1. (Korean J Med 2011;81:786-791)