Introduction: The treatment of craniopharyngiomas is associated with long-term morbidity.
Aim of the study: To assess the long-term functional outcome and mortality rates after treatment for craniopharyngiomas, we audited our data with special focus on cardiovascular, neurological and psychosocial morbidity.
Patients and methods: Between 1965 and 2002, 54 consecutive patients underwent surgery for craniopharyngiomas at the Leiden University Medical Centre (LUMC). Fifteen patients (25%) received additional postoperative radiation therapy. The median follow-up period was 10 years (range 1-37 years).
Results: Long-term cure rate was 82% and long-term recurrence rate 18%. Visual fields/visual acuity stabilized or improved in 74% of cases. The long-term prevalence rate of hypopituitarism was 89%. In addition, long-term cardiovascular, neurological and psychosocial morbidity rates were high: 22% (risk factors 57%), 49% and 47%, respectively. Female sex was an independent predictor of increased cardiovascular, neurological and psychosocial morbidity (odds ratio 3.78, P = 0.031). Ten patients (18%) died during an 828 person-year follow-up. The actuarial patient survival rates 5, 10 and 20 years after the initial operation were 95, 85 and 85%, respectively. The standardized mortality ratio (SMR) was 2.88 [95% confidence interval (CI) 1.35-4.99].
Conclusion: Craniopharyngioma is associated with excessive long-term multisystem morbidity and mortality, especially in female patients, despite a high cure rate. These observations indicate that dedicated long-term follow-up of these patients is required. The purpose of the follow-up should be: first, to look for recurrences and to ensure appropriate endocrine replacement, especially oestrogen replacement in premenopausal females; and second, to achieve intensive control of glucose, lipids, blood pressure and weight, as in any other patient with increased risk for cardiovascular disease.