뇌수모세포종 및 가족성 선종성 용종증으로 발현한Turcot 증후군 1예

저자들은 수모세포종이 있는 24세 여자 환자에서 가족성 선종성 용종증이 동반된 Tucot 증후군 1예를 경험하였으며 문헌고찰과 함께 보고하는 바이다. Turcot syndrome is characterized by the concurrence of a primary neuroepithelial brain tumor and multiple colorectal polyposis. We report a case of a 24-year-old woman diagnosed with Turcot syndrome. At first, the patient was diagnosed as having a medulloblastoma after a tumorectomy of the 4th ventricle mass. The patient underwent radiotherapy and chemotherapy. After high-dose chemotherapy, neutropenic fever and severe mucositis developed. For an evaluation of the persistent hematochezia and diarrhea, a colonoscopy was performed. It revealed pseudomembranous colitis and multiple polyps in the entire colon. According to the family history, her father had undergone a total colectomy due to colon cancer and polyposis of the entire colon. Her brother also was found to have multiple polyps in the colon by a colonoscopy. The patient was diagnosed with Turcot syndrome. (Korean J Pediatr Gastroenterol Nutr 2007; 10: 206∼210) KCI Citation Count: 1