Prevalence of Endocrine and Metabolic Comorbidities in a National Cohort of Patients with Craniopharyngioma

Abstract

Objective: The major part of craniopharyngioma (CP) morbidity is the tumor and/or treatment-related damage, which results in impaired function of the hypothalamic-pituitary axes and metabolic derangements. The aim of the study was to analyze the prevalence of long-term endocrine and metabolic comorbidities in a national cohort of CP patients based on the age at diagnosis and histology criteria. Design: A retrospective-prospective longitudinal cohort analysis. Methods: Forty-six patients with CP treated from 1979 onwards (19 with childhood-onset disease) in a single university institution were included in our study. Median follow-up from presentation was 12.8 years (interquartile range: 8.3-22.2 years) and comparable between age-at-diagnosis and histological subtype groups. Data on tumor histology were extracted from patients' records and re-evaluated if tissue samples were available (n = 32). Results: Childhood-onset patients presented more frequently with headache, and adult-onset with visual impairment. Prevalence of at least one pituitary axis affected increased from 54% at presentation to 100% at follow-up in childhood-onset and from 41 to 93% in adult-onset CP. Growth hormone deficiency, central diabetes insipidus, and panhypopituitarism were more prevalent in childhood-onset adamantinomatous CP (aCP) and least prevalent in adult-onset papillary CP (pCP). At followup, metabolic syndrome (MetS) was diagnosed in 80% of childhood-onset and 68% of adult-onset patients (p = 0.411). In the latter group, it tended to be more frequent in the aCP than pCP subtype (80 vs. 50%, p = 0.110). Conclusions: Long-Long-Term Outcomes in Craniopharyngioma 47 Horm Res Paediatr 2020;93:46-57 term endocrine and metabolic complications are very frequent in childhood-and adult-onset CP patients of both histological subtypes. The prevalence of MetS was higher compared to the largest cohort previously reported.

Craniopharyngioma and Metabolic Syndrome: A 5-Year Follow-Up Single-Center Experience

“…Some authors focused metabolic attention on the adult population (30)(31)(32)(33), and our results are in agreement with these, which confirm a higher prevalence of MS in patients with craniopharyngioma in adulthood. In particular, the major alterations regarded the lipid profile (30,31).…”

“…This is one of the few Italian studies to consider only patients with craniopharyngioma compared to the general population in a single center for a such long follow-up period. Points of the strength of this study are the presence of a control group, the absence of them in some of the studies analyzed ( 30 , 32 , 33 ), and the single-center recruitment reducing the possibility of any selection bias.…”

Concurrent central diabetes insipidus and cerebral salt wasting disease in a post-operative case of craniopharyngioma: a case report

“…Children were found to be particularly vulnerable to pituitary hormonal deficiency post-surgery. Incidence of a single or multiple pituitary hormone deficiency can range from 36 to 98% after surgery [ 2 – 6 ]. In addition to this, the incidence of water and sodium related disorders in children undergoing surgeries involving sellar and supra-sellar region varies from 40 to 72% [ 7 , 8 ].…”

Trans-Endoscopic Treatment of Craniopharyngioma and Recovery from Blindness in Adult Patient – a Case Report

“…The suprasellar region, in which craniopharyngiomas usually grow, is a particularly challenging operative field where surgical radicality is often associated with significant morbidity and mortality, especially when the lesion is large 1 . In recent studies, one group of authors compared series of suprasellar craniopharyngiomas removed endoscopically endonasally and set indications for endonasal removal as the first method of choice [7][8][9] . Small to moderate symmetric craniopharyngiomas, not extending laterally to optic nerves and carotid bifurcation can be removed endoscopically endonasally.…”