Adamantinomatous Craniopharyngioma: Genomics, Radiologic Findings, Clinical, and Prognosis

Müller, Hermann L.; Martı́nez-Barberá, Juan Pedro

doi:10.1007/978-3-030-11339-1_3

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“…In aCP, mutations in CTNNB1 are found in two thirds of patients, activating the WNT/ β-catenin pathway, leading to activation/inhibition of several other pathways. Further studies of these pathways and tumor pathogenesis are needed to identify a causative target for possible future therapeutic use [15,21]. With the promise of novel therapeutic options, which are CP histology subtype dependent, it is therefore important to increase the knowledge regarding outcomes of CP not only based on the age at diagnosis but also on the histology of the tumor.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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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.

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Section: Introductionmentioning

confidence: 99%