Show simple item record

dc.contributor.authorGadalla, S M
dc.contributor.authorPfeiffer, R M
dc.contributor.authorKristinsson, S Y
dc.contributor.authorBjörkholm, M
dc.contributor.authorLandgren, O
dc.contributor.authorGreene, M H
dc.date.accessioned2016-05-02T19:21:52Zen
dc.date.available2016-05-02T19:21:52Zen
dc.date.issued2016-03en
dc.date.submitted2016en
dc.identifier.citationEur. J. Neurol. 2016, 23 (3):542-7en
dc.identifier.issn1468-1331en
dc.identifier.pmid26508558en
dc.identifier.doi10.1111/ene.12886en
dc.identifier.urihttp://hdl.handle.net/2336/607698en
dc.descriptionTo access publisher's full text version of this article click on the hyperlink at the bottom of the pageen
dc.description.abstractPatients with myotonic dystrophy (DM) are at high risk of brain cancer. This study describes the spectrum of brain neoplasms in DM patients.
dc.description.abstractData from 1119 DM patients identified from the National Swedish Patient Register between 1987 and 2007 were linked to the National Cancer and the Cause of Death Registers. Standardized incidence ratios (SIRs) and cumulative incidence to quantify the relative and absolute risks of brain neoplasms were calculated and the Kaplan-Meier estimator was used for survival analysis. Patient follow-up started at birth or the age at the start of Swedish cancer registration (1 January 1958) and ended at the age of brain neoplasm diagnosis, death or on 31 December 2007.
dc.description.abstractTwenty patients developed brain neoplasm during follow-up {median age 53, range 2-76 years, accounting for a five-fold excess risk of brain tumors during the patient lifetime [SIR = 5.4, 95% confidence interval (CI) 3.4-8.1, P = 1 × 10(-5) ]}. Astrocytoma was the most common histological subtype (n = 16, 80%), and almost all cases (n = 19) developed after age 20. No statistically significant differences in gender-specific risks (SIR in men 6.3 and in women 3.8, P-heterogeneity 0.46) were observed. After accounting for competing mortality related to DM, the cumulative incidence of brain neoplasms reached 2.9% (95% CI 1.8%-4.7%) by age 70. Five-year survival after brain tumor diagnosis was 52% (95%CI 29%-75%) overall (number at risk 8) and 34% (95% CI 26%-47%) for malignant neoplasms (number at risk 5).
dc.description.abstractDespite the high relative risk of DM-related brain tumors, the absolute risk is modest. Nonetheless, careful evaluation of DM patients with new central nervous system symptoms is warranted.
dc.description.sponsorshipSwedish Cancer Society Stockholm County Council Karolinska Institutet Foundations National Cancer Institute, USA N02CP31003-3en
dc.language.isoenen
dc.publisherWiley-Blackwellen
dc.relation.urlhttp://dx.doi.org/ 10.1111/ene.12886en
dc.relation.urlhttp://onlinelibrary.wiley.com/doi/10.1111/ene.12886/epdfen
dc.rightsArchived with thanks to European journal of neurologyen
dc.subjectHeilinnen
dc.subjectKrabbameinen
dc.subjectVöðvasjúkdómaren
dc.subjectHEM12
dc.subject.meshBrainen
dc.subject.meshNeoplasmsen
dc.subject.meshIncidenceen
dc.subject.meshMyotonic Dystrophyen
dc.subject.meshMortalityen
dc.titleBrain tumors in patients with myotonic dystrophy: a population-based study.en
dc.typeArticleen
dc.contributor.department[ 1 ] NCI, Clin Genet Branch, Div Canc Epidemiol & Genet, NIH, 9609 Med Ctr Dr, Bethesda, MD 20892 USA [ 2 ] NCI, Biostat Branch, Div Canc Epidemiol & Genet, NIH, 9609 Med Ctr Dr, Bethesda, MD 20892 USA [ 3 ] Karolinska Univ Hosp Solna, Dept Med, Div Hematol, Stockholm, Sweden [ 4 ] Karolinska Inst, Stockholm, Sweden [ 5 ] Univ Iceland, Fac Med, Reykjavik, Iceland [ 6 ] Landspitali Natl Univ Hosp, Dept Hematol, Reykjavik, Iceland   Organization-Enhanced Name(s)      Landspitali National University Hospital [ 7 ] Mem Sloan Kettering Canc Ctr, 1275 York Ave, New York, NY 10021 USAen
dc.identifier.journalEuropean journal of neurologyen
dc.rights.accessNational Consortium - Landsaðganguren
html.description.abstractPatients with myotonic dystrophy (DM) are at high risk of brain cancer. This study describes the spectrum of brain neoplasms in DM patients.
html.description.abstractData from 1119 DM patients identified from the National Swedish Patient Register between 1987 and 2007 were linked to the National Cancer and the Cause of Death Registers. Standardized incidence ratios (SIRs) and cumulative incidence to quantify the relative and absolute risks of brain neoplasms were calculated and the Kaplan-Meier estimator was used for survival analysis. Patient follow-up started at birth or the age at the start of Swedish cancer registration (1 January 1958) and ended at the age of brain neoplasm diagnosis, death or on 31 December 2007.
html.description.abstractTwenty patients developed brain neoplasm during follow-up {median age 53, range 2-76 years, accounting for a five-fold excess risk of brain tumors during the patient lifetime [SIR = 5.4, 95% confidence interval (CI) 3.4-8.1, P = 1 × 10(-5) ]}. Astrocytoma was the most common histological subtype (n = 16, 80%), and almost all cases (n = 19) developed after age 20. No statistically significant differences in gender-specific risks (SIR in men 6.3 and in women 3.8, P-heterogeneity 0.46) were observed. After accounting for competing mortality related to DM, the cumulative incidence of brain neoplasms reached 2.9% (95% CI 1.8%-4.7%) by age 70. Five-year survival after brain tumor diagnosis was 52% (95%CI 29%-75%) overall (number at risk 8) and 34% (95% CI 26%-47%) for malignant neoplasms (number at risk 5).
html.description.abstractDespite the high relative risk of DM-related brain tumors, the absolute risk is modest. Nonetheless, careful evaluation of DM patients with new central nervous system symptoms is warranted.


This item appears in the following Collection(s)

Show simple item record