결장암을 동반한 Turcot씨 증후군 1예
A Case of Turcot’s Syndrome Combined with Colon Cancer in a 15-year-old Girl
이석렬, 이길연, 이기형, 임언, 김효종, 이주희,
소속 상세정보
이석렬 ( Lee Seok-Ryul )
경희대학교 의과대학 외과학교실
이길연 ( Lee Kil-Yeon )
경희대학교 의과대학 외과학교실
이기형 ( Lee Kee-Hyung )
경희대학교 의과대학 외과학교실
임언 ( Leem Won )
경희대학교 의과대학 신경외과학교실
김효종 ( Kim Hyo-Jong )
경희대학교 의과대학 내과학교실
이주희 ( Lee Joo-Hee )
경희대학교 의과대학 해부병리학교실
KMID : 0356720020180030200
Abstract
Turcot’s syndrome is a rare hereditary disease marked by the association of central nervous system neuroepithelial tumor with colonic polyposis. Authors report herein a case of a 15-year-old girl diagnosed as having Turcot’s syndrome, otherwise known as brain tumor-polyposis syndrome, combined with sigmoid colon cancer. The patient was carried out craniostomy and brain tumor removal. The tumor was confirmed histologically to be oligodendroglioma. The patient visited the department of internal medicine for bloody diarrhea during 6 months. Colonoscopy and biopsy was done. The patient was diagnosed as having Turcot’s syndrome combined with sigmoid colon cancer, and was then transferred to the department of surgery for treatment of sigmoid colon cancer. Total proctocolectomy & IPAA (ileal pouch-anal anastomosis) was carried out. Multiple polyps were found in the colon, two large masses were confirmed histologically to be adenocarcinoma. The remaining polyps were adenomas. This case report describes the characteristic features of Turcot’s syndrome presented by this patient.
키워드
Turcot씨 증후군;뇌종양-용종 증후군;결장암;회장낭-항문문합술
Turcot’s syndrome;Brain tumor-polyposis syndrome;Colon cancer Ileal pouch-anal anastomosis (IPAA)
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