Late mortality in pediatric patients with craniopharyngioma.

Ten year survival rates for patients with craniopharyngioma vary from 24 to 100%. A review of the database of all children diagnosed with craniopharyngioma in British Columbia (BC) revealed that several patients died >10 years after diagnosis. This retrospective study investigates the causes and timing of deaths and reports the overall survival in this population based group of patients. A chart review was conducted on all patients aged <17 years, diagnosed in BC with craniopharyngioma between 1967 and 2003. Imaging studies were reviewed by a neuroradiologist. All deaths in the province are reported to a central agency, which allowed identification of patients who died after being lost to clinical follow up. Forty-one patients were identified with nine deaths (aged 11.9-36.9 years). The four patients who died more than 10 years after diagnosis represent 23% of the 17 patients followed for more than 10 years. Three died more than 20 years after diagnosis. The known causes of death were progressive disease (1), uncontrolled diabetes insipidus (1), panhypopituitarism with multi-organ failure (1), pontine infarction (1) and middle cerebral artery infarction in a patient with Moyamoya disease secondary to radiotherapy (1). Two deaths appeared to be seizure related and 1 occurred after orthopedic surgery and remains unexplained. One patient died due to liver failure of unknown etiology. The 10 year overall survival (OS) was 84.1% (95% CI 71.2, 97.1) and the 20 year OS 76.5% (95% CI 58.1, 94.9). Patients remain at risk of premature death more than 10 years after diagnosis. The cause specific late mortality was multifactorial but was rarely due to disease progression. New approaches to craniopharyngioma treatment and life long follow up of cases are required.