Multiple ependymomas in a patient with Turcot's syndrome

✍ Scribed by Torres, Carlos F.; Korones, David N.; Pilcher, Webster

• Publisher: John Wiley and Sons
• Year: 1997
• Tongue: English
• Weight: 200 KB
• Volume: 28
• Category: Article
• ISSN: 0098-1532
• DOI: 10.1002/(sici)1096-911x(199701)28:1<59::aid-mpo11>3.0.co;2-5

✦ Synopsis

A 21-year-old woman was diagnosed with tous polyposis coli (APC) gene. Following partial Turcot's syndrome (TS) at age 16 years. She had resection of the two ependymomas, she was two ependymomas, one was located in the left treated with radiation and chemotherapy. middle cerebellar peduncle and the other in the One year after surgery, paraspinal desmoid low sacral spinal canal. Her mother and brother tumors were found and removed. She is presboth had colectomies for colonic polyposis. Her ently 42 months postsurgical resection of the maternal uncle and grandfather also had this neural tumors and has remained central nervous disease and both died from cancer of the colon system tumor-free. The occurrence of multiple in their fourth decade of life.

ependymoma in TS has not been reported, and The patient was found to have hyperpig-the control of this patient's ependymomas is conmented spots in the retina, skull osteomas and sistent with other reports of long-term survival normal neurological examinations. The bone with TS and glial tumors. scan and CSF were normal and she had a germ-ᮊ 1997 Wiley-Liss, Inc. line mutation in the segment 3 of the adenoma-