Sončka Jazbinšek1,2, Danijela Kolenc3, Roman Bošnjak2,4, Barbara Faganel Kotnik2,5, Lorna Zadravec Zaletel2,6, Barbara Jenko Bizjan2,7, Tina Vipotnik Vesnaver2,8, Tadej Battelino1,2, Andrej Janež2,9, Mojca Jensterle2,9, Primož Kotnik10,11. 1. Department of Endocrinology, Diabetes and Metabolism, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia. 2. Medical Faculty, University of Ljubljana, Ljubljana, Slovenia. 3. Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia. 4. Department of Neurosurgery, University Medical Center Ljubljana, Ljubljana, Slovenia. 5. Department of Hematology and Oncology, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia. 6. Department of Radiotherapy, Institute of Oncology Ljubljana, Ljubljana, Slovenia. 7. Unit of Special Laboratory Diagnostics, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia. 8. Clinical Institute of Radiology, University Medical Center Ljubljana, Ljubljana, Slovenia. 9. Department of Endocrinology, Diabetes and Metabolism, University Medical Center Ljubljana, Ljubljana, Slovenia. 10. Department of Endocrinology, Diabetes and Metabolism, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia, primoz.kotnik@mf.uni-lj.si. 11. Medical Faculty, University of Ljubljana, Ljubljana, Slovenia, primoz.kotnik@mf.uni-lj.si.
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.
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 CPpatients 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 CPpatients of both histological subtypes. The prevalence of MetS was higher compared to the largest cohort previously reported.
Authors: Sergey Gorelyshev; Alexander N Savateev; Nadezhda Mazerkina; Olga Medvedeva; Alexander N Konovalov Journal: Adv Tech Stand Neurosurg Date: 2022