title: Cancer incidence in families with multiple glioma patients.
abstract: Twenty-four Finnish families with 2 or more glioma patients were identified through questionnaires sent to 369 consecutive glioma patients receiving surgery at Tampere University Hospital during 1983-94. To explore whether unusual cancer susceptibility is involved, the cancer risk of 2,664 family members was estimated using population-based data from the Finnish Cancer Registry. Among the total cohort of relatives, 88 cancers were observed during 1953-97. The overall cancer risk among 12 families with juvenile onset gliomas was significantly decreased (standardized incidence ratio [SIR] 0.6, 95% confidence interval [CI]: 0.4-0.9). Among 12 families with adult onset gliomas, the overall cancer risk was equal to that of the reference population (SIR 1.1, 95% CI: 0.8-1.4) whereas the risk of skin melanoma (SIR 4.0, 95% CI: 1.5-8.8) and meningioma (SIR 5.5, 95% CI: 1.1-16) were significantly increased. Several other tumors, including those associated with neurofibromatosis 1 and 2, tuberous sclerosis and Li-Fraumeni and Turcot syndromes were surveyed, but no elevated risks were observed. In conclusion, the presence of meningiomas and skin melanomas in glioma families may indicate a novel association as a cancer susceptibility trait.
authors.affiliation: Department of Pathology, Tampere University Hospital, Tampere, Finland.
authors.forename: Niina
authors.initials: N
authors.lastname: Paunu
authors.forename: Eero
authors.initials: E
authors.lastname: Pukkala
authors.forename: Pekka
authors.initials: P
authors.lastname: Laippala
authors.forename: Risto
authors.initials: R
authors.lastname: Sankila
authors.forename: Jorma
authors.initials: J
authors.lastname: Isola
authors.forename: Helena
authors.initials: H
authors.lastname: Miettinen
authors.forename: Kalle O J
authors.initials: KO
authors.lastname: Simola
authors.forename: Pauli
authors.initials: P
authors.lastname: Helén
authors.forename: Heikki
authors.initials: H
authors.lastname: Helin
authors.forename: Hannu
authors.initials: H
authors.lastname: Haapasalo
mesh_terms: D000328:Adult; D017668:Age of Onset; D001932:Brain Neoplasms / Q000453:epidemiology* / Q000235:genetics; D016001:Confidence Intervals; D005190:Family; D005260:Female; D005387:Finland / Q000453:epidemiology; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D005910:Glioma / Q000453:epidemiology* / Q000235:genetics; D006801:Humans; D015994:Incidence; D008297:Male; D016609:Neoplasms, Second Primary / Q000453:epidemiology* / Q000235:genetics; D012042:Registries / Q000706:statistics & numerical data; D012307:Risk Factors; D012907:Smoking / Q000453:epidemiology