Update on the management of familial central nervous system tumor syndromes Journal Article
| Authors: | Hottinger, A. F.; Khakoo, Y. |
| Article Title: | Update on the management of familial central nervous system tumor syndromes |
| Abstract: | Hereditary central nervous tumor syndromes are a varied group of conditions that include neurofibromatosis type 1 and 2, tuberous sclerosis, Von Hippel-Lindau disease, and Cowden, Turcot, and Gorlin syndromes. The responsible genes have been identified in most of these disorders. These genes typically act as tumor suppressor genes, maintain normal cellular function and homeostasis, and regulate cell growth and differentiation. Familial central nervous system tumors are mostly inherited as autosomal dominant traits and involve germline mutations. Neoplastic development occurs when a somatic mutation inactivates the second allele. These patients also present unique challenges for their management. This review highlights the clinical manifestations, molecular genetics, pathophysiology, and current treatment options of these disorders with a focus on neuro-oncologic manifestations of the diseases. Copyright © 2007 by Current Medicine Group LLC. |
| Keywords: | cancer chemotherapy; somatic mutation; clinical feature; clinical trial; review; sorafenib; cisplatin; sunitinib; pathophysiology; cancer radiotherapy; molecular genetics; allele; cell function; neurofibromatosis; diagnostic procedure; neurofibromatosis 1; carboplatin; cell growth; diagnosis, differential; vincristine; clinical protocol; cell differentiation; lomustine; central nervous system tumor; central nervous system neoplasms; gene identification; tipifarnib; familial cancer; li-fraumeni syndrome; von hippel lindau disease; homeostasis; tumor suppressor protein; rapamycin; basal cell nevus syndrome; cowden syndrome; tuberous sclerosis; turcot syndrome; optic nerve glioma; autosomal dominant inheritance; neoplastic syndromes, hereditary; hamartoma syndrome, multiple; vigabatrin; hippel-lindau disease; neurofibromatosis 2 |
| Journal Title: | Current Neurology and Neuroscience Reports |
| Volume: | 7 |
| Issue: | 3 |
| ISSN: | 1528-4042 |
| Publisher: | Springer |
| Date Published: | 2007-05-01 |
| Start Page: | 200 |
| End Page: | 207 |
| Language: | English |
| DOI: | 10.1007/s11910-007-0031-5 |
| PUBMED: | 17488585 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 3" - "Export Date: 17 November 2011" - "CODEN: CNNRB" - "Source: Scopus" |
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