Document Detail

Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood-onset - a population-based study in Sweden.
MedLine Citation:
PMID:  25375987     Owner:  NLM     Status:  Publisher    
Context: Craniopharyngiomas in adults have been associated with excess mortality. Objective: The aim of the study was to investigate mortality and morbidity in childhood-onset and adult-onset craniopharyngioma patients. Methods: Craniopharyngioma patients were identified and followed in Swedish national health registries, 1987-2011. The inclusion criteria for the craniopharyngioma diagnosis were internally validated in 28% of the study population against patient records. Settings: Nationwide population-based study. Patients: 307 patients (151 men; 156 women) were identified and included (mean follow-up: 9 years, range 0-25). The inclusion criteria had a positive predictive value of 97% and a sensitivity of 92%. Intervention: None. Main Outcome Measures: Standardized mortality ratios (SMRs) and standardized incidence ratios (SIRs) with 95% confidence intervals were calculated using the Swedish population as reference. Results: During the study 54 patients died compared with the expected number of 14.1, resulting in an SMR of 3.2 (2.2-4.7) for men and 4.9 (3.2-7.2) for women. Childhood-onset (n=106) and adult-onset craniopharyngioma (n=201) had SMRs of 17 (6.3-37) and 3.5 (2.6-4.6), respectively. Patients with hypopituitarism (n=250), diabetes insipidus (n=110), and neither of these (n=54) had SMRs of 4.3 (3.1-5.8), 6.1 (3.5-9.7), and 2.7 (1.4-4.6), respectively. SMR due to cerebrovascular diseases was 5.1 (1.7-12). SIRs were 5.6 (3.8-8.0) for type 2 diabetes mellitus (T2DM), 7.1 (5.0-9.9) for cerebral infarction, 0.7 (0.2-1.7) for myocardial infarction, 2.1 (1.4-3.0) for fracture, and 5.9 (3.4-9.4) for severe infection. The SIR for all malignant tumors was 1.3 (0.8-2.1). Conclusions: This first nationwide population-based study of craniopharyngioma patients demonstrated an excess mortality that was especially marked in patients with childhood-onset disease and among women. Death due to cerebrovascular diseases was increased five-fold. Hypopituitarism and diabetes insipidus were negative prognostic factors for mortality and morbidity. Craniopharyngioma patients suffered from an increased disease burden related to T2DM, cerebral infarction, fracture, and severe infection.
Daniel S Olsson; Eva Andersson; Ing-Liss Bryngelsson; Anna G Nilsson; Gudmundur Johannsson
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-11-6
Journal Detail:
Title:  The Journal of clinical endocrinology and metabolism     Volume:  -     ISSN:  1945-7197     ISO Abbreviation:  J. Clin. Endocrinol. Metab.     Publication Date:  2014 Nov 
Date Detail:
Created Date:  2014-11-6     Completed Date:  -     Revised Date:  2014-11-7    
Medline Journal Info:
Nlm Unique ID:  0375362     Medline TA:  J Clin Endocrinol Metab     Country:  -    
Other Details:
Languages:  ENG     Pagination:  jc20143525     Citation Subset:  -    
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