십이지장 선암을 동반한 제1형 신경섬유종증 1예
A case of adenocarcinoma, signet ring cell type, in 2nd portion of duodenum associated with neurofibromatosis type I
전승민, 이나리, 이종섭, 박재현, 박상은,
소속 상세정보
전승민 ( Jeon Soung-Min )
샘안양병원 내과
이나리 ( Lee Na-Ri )
샘안양병원 내과
이종섭 ( Lee Jong-Sup )
샘안양병원 내과
박재현 ( Park Jae-Hyun )
샘안양병원 일반외과
박상은 ( Park Sang-Eun )
샘안양병원 내과
KMID : 0882420040670060642
Abstract
저자들은 상복부 동통을 주소로 내원한 64세의 제1형 신경섬유종증 남자 환자에서 동반된 십이지장 선암 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.
The neurofibromatoses are a rare group of hereditary diseases of autosomal dominant fashion with the overall incidence of one in 3,000~4,000 and with two distinct forms, type I (Von Recklinghausen’s neurofibromatosis) characterized by skin lesions including multiple cutaneous neurofibromas, axillary and groin freckling, and cafe-au-lait spots and type II by the presence of bilateral vestibular schwannomas. The neurofibromatosis type I is associated with the mutation of NF-I gene on chromosome 17q 11.2, which has a tumor suppressive role and with resultant development of the wide variety of tumors with the malignant incidence of about 2~16 %. Although the cases of malignant tumors in the neurofibromatosis type I have been reported in various tumors with the neural origin, lymphoma, pheochromocytoma and some cases of gastrointestinal tumors, the gastrointestinal tumors were found in only hepatobiliary system and large and small bowels and the cases of the malignant tumors of upper GI origin in duodenum and stomach have not been reported yet in Korea. We report a case of a 64-year-old man admitted with epigastric pain and diagnosed to adenocarcinoma, signet ring cell type, in 2nd portion of duodenum associated with neurofibromatosis type I.
키워드
Neurofibromatosis type I;Adenocarcinoma;Duodenum
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