Pediatric and Adult Craniopharyngioma: A 37-Year Experience at a National Referral Center

Hernandez, Maria Isabel; Ibeas, Consuelo; Fernandez, Jose Pablo; Ivanovic-zuvic, Danisa; Gomez, Marisol; Gutierrez, Dina; Valenzuela, Samuel; Okuma, Cecilia

Abstract

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.

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Título según WOS: ID WOS:001674438600001 Not found in local WOS DB
Título de la Revista: CLINICAL ENDOCRINOLOGY
Volumen: 104
Número: 6
Editorial: Wiley
Fecha de publicación: 2026
Página de inicio: 628
Página final: 636
DOI:

10.1111/cen.70103

Notas: ISI