Excess Mortality and Morbidity in Patients with Craniopharyngioma, Especially in Patients with Childhood Onset: A Population-Based Study in Sweden

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 Outcome Measures: 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.