Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood onset: a population-based study in Sweden.
J Clin Endocrinol Metab
; 100(2): 467-74, 2015 Feb.
Article
en En
| MEDLINE
| ID: mdl-25375987
ABSTRACT
CONTEXT Craniopharyngiomas (CPs) in adults have been associated with excess mortality. OBJECTIVE:
The aim of the study was to investigate mortality and morbidity in patients with childhood-onset and adult-onset CP.METHODS:
PATIENTS with CP were identified and followed in Swedish national health registries, 1987 through 2011. The inclusion criteria for the CP diagnosis were internally validated against patient records in 28% of the study population. SETTINGS This was a nationwide population-based study. PATIENTS A total of 307 patients (151 men and 156 women) were identified and included (mean follow-up, 9 years; range, 0-25 years). The inclusion criteria had a positive predictive value of 97% and a sensitivity of 92%. INTERVENTION There were no interventions. MAIN OUTCOMEMEASURES:
Standardized mortality ratios (SMRs) and standardized incidence ratios (SIRs) with 95% confidence intervals were calculated using the Swedish population as the 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. PATIENTS with childhood-onset (n = 106) and adult-onset (n = 201) CP 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. The SMR due to cerebrovascular diseases was 5.1 (1.7-12). SIRs were 5.6 (3.8-8.0) for type 2 diabetes mellitus, 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 patients with CP demonstrated excess mortality that was especially marked in patients with childhood-onset disease and among women. Death due to cerebrovascular diseases was increased 5-fold. Hypopituitarism and diabetes insipidus were negative prognostic factors for mortality and morbidity. PATIENTS with CP had increased disease burden related to type 2 diabetes mellitus, cerebral infarction, fracture, and severe infection.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Neoplasias Hipofisarias
/
Craneofaringioma
Tipo de estudio:
Incidence_studies
/
Prognostic_studies
Límite:
Adolescent
/
Adult
/
Aged
/
Aged80
/
Child
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Child, preschool
/
Female
/
Humans
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Infant
/
Male
País/Región como asunto:
Europa
Idioma:
En
Revista:
J Clin Endocrinol Metab
Año:
2015
Tipo del documento:
Article
País de afiliación:
Suecia