Department of Endocrinology, Diabetes and Metabolism, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia.
Medical Faculty, University of Ljubljana, Ljubljana, Slovenia.
Horm Res Paediatr. 2020;93(1):46-57. doi: 10.1159/000507702. Epub 2020 May 27.
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.
A retrospective-prospective longitudinal cohort analysis.
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).
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).
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.
颅咽管瘤(CP)的大部分发病率是由肿瘤和/或治疗相关的损伤引起的,这会导致下丘脑-垂体轴功能受损和代谢紊乱。本研究的目的是根据诊断时的年龄和组织学标准,分析基于全国 CP 患者队列的长期内分泌和代谢合并症的患病率。
回顾性前瞻性纵向队列分析。
纳入了一家大学附属医院 1979 年以来治疗的 46 例 CP 患者(19 例为儿童发病)。从发病到随访的中位时间为 12.8 年(四分位距:8.3-22.2 年),在年龄组和组织学亚型组之间无差异。从患者记录中提取肿瘤组织学数据,如果有组织样本可用(n=32),则对其进行重新评估。
儿童发病患者更常表现为头痛,而成人发病患者更常表现为视力障碍。至少一个垂体轴受累的患病率从发病时的 54%增加到儿童发病患者的随访时的 100%,以及成人发病 CP 患者的 41%至 93%。在儿童发病的牙釉质型 CP(aCP)中,生长激素缺乏症、中枢性尿崩症和全垂体功能减退症更为常见,而在成人发病的乳头型 CP(pCP)中则最为少见。在随访时,80%的儿童发病和 68%的成人发病患者被诊断为代谢综合征(MetS)(p=0.411)。在后一组中,aCP 比 pCP 亚型更为常见(80%比 50%,p=0.110)。
儿童和成人发病的 CP 患者无论组织学亚型如何,长期的内分泌和代谢并发症都非常常见。与之前报道的最大队列相比,MetS 的患病率更高。