Clinical endocrinology · 2026
Pediatric and Adult Craniopharyngioma: A 37-Year Experience at a National Referral Center.
СтатьяНазвание и аннотация: Перевод в очереди
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Hernández María Isabel, Ibeas Consuelo, Fernández José Pablo, Ivanovic-Zuvic Danisa, Gómez Marisol, Gutiérrez Dina, Valenzuela Samuel, Okuma Cecilia
Аннотация
INTRODUCTION: Craniopharyngioma (CP) is a rare, recurrent central nervous system tumor with significant hormonal, visual, and metabolic sequelae.
METHODOLOGY: This retrospective cohort study analyzes 37 years of experience managing pediatric and adult CP patients at a specialized referral center in Chile, stratified by age at onset. Clinical, biochemical, imaging, and neurosurgical data from 1986 to 2023 were reviewed.
RESULTS: A total of 87 patients (1-68 years) were included, with 65 (75%) presenting in childhood (≤ 18 years) and 22 (25%) in adulthood. Mean follow-up was 9.3 years (IQR 3.7-14.1). Histology was adamantinomatous in 84%, papillary in 3%, and unspecified in 13%. The most common symptoms at presentation were headache (77%), visual disturbances (60%), and nausea (45%). At diagnosis, 54% had at least one pituitary dysfunction, most frequently thyrotropic (28.2%) and somatotropic (27.9%). Following initial treatment, pituitary dysfunction increased to 71%, with thyrotropic (59%) and corticotropic (48%) being the most affected axes. Anterior panhypopituitarism occurred in 25%, and permanent AVP deficiency in 32%. Tumor recurrence or progression was observed in 63%.
CONCLUSIONS: CP carries a high risk of recurrence and neuroendocrine complications. This study, based on 37 years of experience in a reference center, is the first national report on CP patients, with findings consistent with previous literature. The management of these patients is complex and demands a highly experienced multidisciplinary team to achieve optimal long-term outcomes.
METHODOLOGY: This retrospective cohort study analyzes 37 years of experience managing pediatric and adult CP patients at a specialized referral center in Chile, stratified by age at onset. Clinical, biochemical, imaging, and neurosurgical data from 1986 to 2023 were reviewed.
RESULTS: A total of 87 patients (1-68 years) were included, with 65 (75%) presenting in childhood (≤ 18 years) and 22 (25%) in adulthood. Mean follow-up was 9.3 years (IQR 3.7-14.1). Histology was adamantinomatous in 84%, papillary in 3%, and unspecified in 13%. The most common symptoms at presentation were headache (77%), visual disturbances (60%), and nausea (45%). At diagnosis, 54% had at least one pituitary dysfunction, most frequently thyrotropic (28.2%) and somatotropic (27.9%). Following initial treatment, pituitary dysfunction increased to 71%, with thyrotropic (59%) and corticotropic (48%) being the most affected axes. Anterior panhypopituitarism occurred in 25%, and permanent AVP deficiency in 32%. Tumor recurrence or progression was observed in 63%.
CONCLUSIONS: CP carries a high risk of recurrence and neuroendocrine complications. This study, based on 37 years of experience in a reference center, is the first national report on CP patients, with findings consistent with previous literature. The management of these patients is complex and demands a highly experienced multidisciplinary team to achieve optimal long-term outcomes.