Central Nervous System Tumors

Central nervous system (CNS) tumors appear in less than 1% of FAP patients. The association of colonic polyposis and CNS tumors is termed Turcot syndrome [142]. About two thirds of Turcot syndrome cases are associated with APC gene mutations and therefore are considered variants of FAP (Turcot type 2) [143]. They usually exhibit typical colonic polyposis and CNS tumors including medulloblas-toma, anaplastic astrocytoma, and ependymomas. One third of patients with Turcot syndrome have a variant of HNPCC (Turcot type 1) [143]. A review of documented Turcot cases determined the average age at death to be 20.3 years. This raises the difficult question as to whether prophylactic colectomy, and specifically restorative proctocolectomy, should be performed [144].

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