December 19, 2014
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Childhood-onset craniopharyngioma linked to high mortality

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Craniopharyngioma appears to be linked to excess mortality, particularly in women and in patients with childhood-onset disease, according to recent findings.

In the nationwide, population-based study, researchers identified 307 patients with craniopharyngioma (151 men) from the Swedish National Patient Registry and the Swedish Cancer Registry to determine rates of morbidity and mortality for both childhood- and adult-onset disease.

The researchers specifically studied the following conditions: type 2 diabetes, myocardial infarction, fracture, severe infection, cerebral infarction and severe vision impairment. The mean follow-up was 9 years. Person-years at risk were determined from study enrollment to follow-up diagnosis, death or conclusion of study, and were stratified by gender, 5-year age groups and 1-year calendar periods.

The researchers found that there were 54 deaths during the study, which exceeded the expected number of 14.1. This resulted in a standardized mortality ratio (SMR) of 3.2 (95% CI, 2.2-4.7) for men and 4.9 (95% CI, 3.2-7.2) for women. Patients with childhood-onset disease (n=106) had an SMR of 17 (95% CI, 6.3-37), whereas those with adult-onset craniopharyngioma (n=201) had SMRs of 3.5 (95% CI, 2.6-4.6). The SMRs for patients with hypopituitarism (n=250) was 4.3 (95% CI, 3.1-5.8) and the SMR for patients with diabetes insipidus was 6.1 (95% CI, 3.5-9.7). SMR specific to cerebrovascular diseases was 5.1 (95% CI, 1.7-12).

The following standardized incident ratios (SIRs) were determined: 5.6 for type 2 diabetes (95% CI, 3.8-8), 7.1 for cerebral infarction (95% CI, 5-9.9), 0.7 for MI (95% CI, 0.2-1.7), 2.1 for fracture (95% CI, 1.4-3) and 5.9 for severe infection (95% CI, 3.4-9.4). All malignant tumors had an SIR of 1.3 (95% CI, 0.8-2.1). 

According to the researchers, these findings reflect the complexity of craniopharyngioma and its treatment.

“[Craniopharyngioma] is a rare tumor, and the tumor itself and its management can lead to a complex clinical picture dominated by endocrine, metabolic and neuropsychological consequences,” the researchers wrote. “This first nationwide study reveals a marked excess mortality and a prominently shortened lifespan in [craniopharyngioma] patients.”

Disclosure: See the full study for a complete list of the researchers’ financial disclosures.