| Author/Editor | Jazbinšek, Sončka; Kolenc, Danijela; Bošnjak, Roman; Faganel, Barbara; Zadravec-Zaletel, Lorna; Jenko Bizjan, Barbara; Vipotnik-Vesnaver, Tina; Battelino, Tadej; Janež, Andrej; Jensterle Sever, Mojca; Kotnik, Primož | |
| Title | Prevalence of endocrine and metabolic comorbidities in a national cohort of patients with craniopharyngioma | |
| Type | članek | |
| Publication year | 2020 | |
| Volume | str. str. | |
| ISSN | 1663-2818 - Hormone research in paediatrics | |
| Language | eng | |
| 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 follow-up, 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-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. | |
| Keywords | endokrinologija presnovne bolezni pediatrija kraniofaringiom endocrinology metabolic disease paediatrics craniopharyngioma |