Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood onset: a population-based study in Sweden.
BACKGROUND
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.


METHODS
This was a nationwide population-based study.


METHODS
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%.


METHODS
There were no interventions.


METHODS
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.
Department of Endocrinology (D.S.O., A.G.N., G.J.), Sahlgrenska University Hospital, SE-413 45 Gothenburg, Sweden; Department of Occupational and Environmental Medicine (E.A.), Sahlgrenska University Hospital, SE-405 30 Gothenburg, Sweden; and Department of Occupational and Environmental Medicine (I.-L.B.), Örebro University Hospital, SE-701 85 Örebro, Sweden.
Daniel S
DS
Olsson
Eva
E
Andersson
Ing-Liss
IL
Bryngelsson
Anna G
AG
Nilsson
Gudmundur
G
Johannsson
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D003397:Craniopharyngioma / Q000401:mortality*; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D010911:Pituitary Neoplasms / Q000401:mortality*; D012042:Registries; D013548:Sweden / Q000453:epidemiology; D055815:Young Adult