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Familial syndromes associated with intracranial tumours: a review
Adrianna M Ranger1, Yatri K Patel, Navjot Chaudhary
1Department of Clinical Neurological Sciences, Western University, 800 Commissioners Road East, London, ON, N6A 5W9, Canada, adrianna.ranger@gmail.com.
Background:
Most cancers of the central nervous system (CNS) occur sporadically in the absence of any known underlying familial disorder or multi-systemic syndrome. Several syndromes are associated with CNS malignancies, however, and their recognition has significant implications for patient management and prognosis. Patients with syndrome-associated CNS malignancies often have multiple tumours (either confined to one region or distributed throughout the body), with similar or different histology.
Objective:
This review examines syndromes that are strongly associated with CNS cancers: the phakomatosis syndromes, familial syndromes such as Li-Fraumeni and familial polyposis syndromes and dyschondroplasia.
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