A case series of craniopharyngioma patients in the Puerto Rico Medical Center, 2004-2014: epidemiology and management analysis
Ortiz, M.; Ortiz, S.; Falcon, A.; Inserni, J.
Boletin de la Asociacion Medica de Puerto Rico 109(1): 5-12
2017
ISSN/ISBN: 0004-4849 Document Number: 703062
Craniopharyngioma (CP) is a benign tumor with "malignant" characteristics due to its unpredictable growth and damage to vital brain structures. The epidemiology of CP in the Puerto Rican population has never been studied, and there is no literature describing CP in Puerto Rico. Management of CP is a subject of debate, with no clear consensus on the preferred treatment modality. A case series was performed using patients diagnosed and managed in the Puerto Rico Medical Center during the years 2004-2014, with the aim of describing the epidemiology and management of CP in the Puerto Rican population. 33 records were included for both pediatric and adult populations, 13 children and 20 adults, which were evaluated for the analysis. The average incidence of CP for the period was 0.802 cases per 1,000,000 person-years. There was no statistically significant difference in incidence by gender or geographic distribution. The 5-year survival rate was 79.5% (95%CI: 48.3-93.0), and the recurrence rate was 45.6%. Due to the complexity of the cases, frequency of recurrences, and lack of consistent benefits among the alternative treatment interventions, no consensus has been reached regarding treatment. Each decision is made on a case-by-case approach, considering tumor location and involvement of hypothalamic and optic structures. Recent studies favor the use of RT after subtotal resection, especially in the pediatric population. Nonetheless, there does not seem to be a difference in survival among the alternative treatment modalities.
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