Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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Medulloblastoma and Familial Adenomatous Polyposis in a 24-year-old Female Patient: A Case Report of Turcot Syndrome

뇌수모세포종 및 가족성 선종성 용종증으로 발현한 Turcot 증후군 1예

  • Jeong, Soo-In (Department of Pediatrics. Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Suh, Jung-Min (Department of Pediatrics. Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Ji-Hyuk (Department of Pediatrics. Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Hae-Jung (Department of Pediatrics. Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Jee-Hyun (Department of Pediatrics. Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Sung, Ki-Woong (Department of Pediatrics. Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Song, Hye-Jung (Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Choe, Yon-Ho (Department of Pediatrics. Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 정수인 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 서정민 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 이지혁 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 이해정 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 이지현 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 성기웅 (성균관대학교 의과대학 삼성서울병원 소아과학교실) ;
  • 송혜정 (성균관대학교 의과대학 삼성서울병원 진단병리과학교실) ;
  • 최연호 (성균관대학교 의과대학 삼성서울병원 소아과학교실)
  • Received : 2007.07.31
  • Accepted : 2007.08.30
  • Published : 2007.09.30
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Abstract

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.

저자들은 수모세포종이 있는 24세 여자 환자에서 가족성 선종성 용종증이 동반된 Tucot 증후군 1예를 경험하였으며 문헌고찰과 함께 보고하는 바이다.

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