Abstract | BACKGROUND: Published studies have shown that familial risks in the primary central nervous system (CNS) tumors are usually histology-specific. If genetic factors indeed determine tumor histology it would be expected that histological types would agree between affected first-degree relatives (FDRs). MATERIAL AND METHODS: This study was conducted using the nationwide Swedish Family- Cancer Database. FDR pairs were defined where both of them had the same histological subtype of CNS tumor. The histological concordance was determined using kappa agreement test. RESULTS: We identified 858 familial patients (333 parent-offspring pairs, 97 sibling pairs) with primary CNS tumors. Proportion of spinal hemangioblastoma out of all familial hemangioblastomas (21%) was significantly higher than that in sporadic patients (7%; P=0.001). The highest kappa value was found for hemangioblastoma among parent-child pairs (kappa=86%, 95% CI: 74-98%). There was a moderate agreement for concordant neurinoma among father-daughter pairs (kappa=48% 95% CI: 15-81%). Low grade glioma showed significant agreement among mother-daughter (kappa=33%, 95% CI: 9-57%) and father-daughter pairs (kappa=39%, 95% CI: 11-67%), but not in mother-son (kappa=10%, 95% CI: -13% to 32%) and father-son pairs (kappa=9%, 95% CI: -1% to 40%). There was histological agreements for meningioma in mother-offspring (kappa range=20-27%) but not in father-daughter (kappa=14%, 95% CI: -8% to 35%) and father-son pairs (kappa=9%, 95% CI: -12% to 30%). CONCLUSIONS:
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Authors | M Babaei, M Fallah, K Sundquist, K Hemminki |
Journal | Cancer epidemiology
(Cancer Epidemiol)
Vol. 39
Issue 3
Pg. 334-9
(Jun 2015)
ISSN: 1877-783X [Electronic] Netherlands |
PMID | 25799913
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Copyright | Copyright © 2015 Elsevier Ltd. All rights reserved. |
Topics |
- Adult
- Aged
- Central Nervous System Neoplasms
(genetics)
- Child
- Databases, Factual
- Family
- Female
- Genetic Predisposition to Disease
- Humans
- Male
- Middle Aged
- Sweden
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