PMID: 8950338Jan 1, 1997Paper

Multiple ependymomas in a patient with Turcot's syndrome

Medical and Pediatric Oncology
C F TorresW H Pilcher

Abstract

A 21-year-old woman was diagnosed with Turcot's syndrome (TS) at age 16 years. She had two ependymomas, one was located in the left middle cerebellar peduncle and the other in the low sacral spinal canal. Her mother and brother both had colectomies for colonic polyposis. Her maternal uncle and grandfather also had this disease and both died from cancer of the colon in their fourth decade of life. The patient was found to have hyperpigmented spots in the retina, skull osteomas and normal neurological examinations. The bone scan and CSF were normal and she had a germline mutation in the segment 3 of the adenomatous polyposis coli (APC) gene. Following partial resection of the two ependymomas, she was treated with radiation and chemotherapy. One year after surgery, paraspinal desmoid tumors were found and removed. She is presently 42 months postsurgical resection of the neural tumors and has remained central nervous system tumor-free. The occurrence of multiple ependymoma in TS has not been reported, and the control of this patient's ependymomas is consistent with other reports of long-term survival with TS and glial tumors.

References

Jul 1, 1991·Journal of Surgical Oncology·A K SohrabiA L Watne
Sep 1, 1989·Diseases of the Colon and Rectum·M KropilakE McGannon
Nov 1, 1988·Diseases of the Colon and Rectum·L JarvisD Beckly
Mar 12, 1987·The New England Journal of Medicine·E I TraboulsiS B Welsh
Jun 1, 1982·Journal of Medical Genetics·S B Cohen
Mar 30, 1995·The New England Journal of Medicine·S R HamiltonB Tetu
Mar 1, 1994·Gut·A K GurbuzS R Hamilton

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Citations

Oct 1, 2011·Chinese Journal of Cancer·Yuan YaoMichael D Taylor
Jul 26, 2005·Journal of Neurobiology·Marie P FogartyRobert J Wechsler-Reya
Sep 3, 2010·AJR. American Journal of Roentgenology·Nicholas Andrew Koontz, Christopher P Hess
Oct 24, 2006·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·M L GarrèA Naselli
Jun 28, 2019·Neuro-oncology·Ivo S MuskensJoseph L Wiemels
Jul 21, 1999·Arquivos de neuro-psiquiatria·L F TorresR Ramina
Jul 2, 2020·Neuro-oncology·Chenan ZhangKyle M Walsh

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