2.3.1.1 Prevalence and Incidence
Table 23 summarizes available data on the prevalence of craniopharyngiomas and nonadenomatous sellar lesions.
| POPULATION | DATA SOURCE | PREVALENCE | REFERENCE |
| US subjects undergoing at least one pituitary MRI scan from 1999 to 2009 (n=2,598) | Medical records Pituitary Center, Cedars Sinai Medical Center, Los Angeles, California | Nonadenomatous sellar masses accounted for 18% of visible lesions, of which the most common were Rathke’s cleft cyst (19%), craniopharyngioma (15%), and meningioma (15%). Metastases accounted for 5% of non-pituitary lesions and breast cancer was the most common primary source. | Famini et al. 2011107 |
| Canada, patients presenting with a sellar mass (n=1,005) | Data from all pituitary-related referrals within the province of Nova Scotia were prospectively collected in interlinked computerized registries starting in November 2005. | There was a population prevalence rate of sellar masses of 0.1%. Of these patients, 837 (83%) had pituitary adenomas and 168 (17%) had non-pituitary lesions. The relative prevalence and standardized incidence ratio, respectively, of various sellar masses were: nonfunctioning adenomas (38.4%; 2.34), prolactinomas (34.3%; 2.22), Rathke’s cyst (6.5%; 0.5), GH-secreting adenomas (6.5%; 0.3), craniopharyngiomas (4.5%; 0.2), adrenocorticotropic hormone-secreting adenomas (3.8%; 0.2), meningiomas (1.9%), and others (3.9%; 0.21). | Al-Dahmani et al. 2015108 |
Abbreviations: US, United States; MRI, magnetic resonance imaging.
2.3.1.2 Life Expectancy and Mortality
One retrospective study conducted in the United Kingdom reported that patients with after surgery for craniopharyngiomas had an SMR 8.75 (95% CI of 5.4-13.3). For women, the SMR was 10.51 (95% CI 5.04-19.3) and for men it was 7.55 (95% CI 3.77-13.52). The study concluded that patients with craniopharyngiomas had high rates of mortality and morbidity, yet the underlying causes for these mortality rates remain poorly understood.109
2.3.1.3 Key Trends and Health Outcomes
Pediatric patients with sellar masses such as craniopharyngioma or cyst of Rathke’s pouch frequently suffer disease- and treatment-related sequelae.110
A study by Daubenbuchel et al. of 177 pediatric patients (163 craniopharyngioma and 14 Rathke’s pouch) concluded that initial hydrocephalus has no impact on outcome in patients with sellar masses. Overall survival and functional capacity are impaired in survivors presenting with initial hypothalamic involvement. Progression-free survival is not affected by hydrocephalus, hypothalamic involvement, or degree of resection. Accordingly, gross-total resection is not recommended in sellar masses with initial hypothalamic involvement to prevent further hypothalamic damage.110