All of the following hereditary conditions prediscope to CNS tumors, except
Correct Answer: Xeroderma pigmentosum
Description: Heriditary syndromes associated with Brain tumors Syndrome Gene(locus) CNS neoplasm Von Hippel-Lindau VHL (3p) Hemangioblastoma of retina, cerebellum and spinal cord Li-Fraumeni p53 (17p) Malignant glioma Retinoblastoma Rb (13q) Retinoblastoma, pineoblastoma, malignant glioma Neurofibtomatosis type 1(Von Recklinghausen's disease) NF1 (17q) Neuroma, Schwannoma, meningioma, optic glioma MEN1 (wermer syndrome) MEN1 (11q) Pituitary adenoma, malignant schwannoma Turcot's syndrome APC (5q) Medulloblastoma, malignant glioma Gorlin (basal cell nevus) PTCH(9q) Medulloblastoma Tuberous sclerosis TSC1 (9q) TSC2 (16p) Astrocytoma Neurofibtomatosis 2 NF2 (22q) Schwannoma, glioma, ependymoma Ref: Harrison's 19th edition Pgno : 599
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