A Case of Turcot's Syndrome Combined with Colon Cancer in a 15-year-old Girl.

, Lee, Seok Ryeol; , Lee, Kil Yeon; , Lee, Kee Hyung; , Leem, Won; , Kim, Hyo Jong; , Lee, Ju Hie

Case Report

Journal of the Korean Society of Coloproctology 2002;18(3):200-204.

Lee, Seok Ryeol , Lee, Kil Yeon , Lee, Kee Hyung , Leem, Won , Kim, Hyo Jong , Lee, Ju Hie

¹Department of Surgery, Kyung-hee University Hospital, Seoul, Korea. keehlee@chollian.net
²Department of Neurosurgery, Kyung-hee University Hospital, Seoul, Korea.
³Department of Internal Medicine, Kyung-hee University Hospital, Seoul, Korea.
⁴Department of Anatomical Pathology, Kyung-hee University Hospital, Seoul, Korea.

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 and 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.