Department of Pediatric Endocrinology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, Netherlands.
Medical Sciences, University Medical Center Utrecht, University of Utrecht, Utrecht, Netherlands.
Endocr Rev. 2019 Feb 1;40(1):193-235. doi: 10.1210/er.2018-00017.
The development of hypothalamic obesity (HO) following craniopharyngioma (CP) and other suprasellar tumors leads to reduced patient quality of life. No treatment algorithms are currently available for management of HO. Depending on which hypothalamic nuclei are destroyed, the pathophysiologic mechanisms and clinical symptoms that contribute to HO differ among patients. Herein, we review the contribution of the hypothalamus to the pathophysiologic mechanisms and symptoms underlying CP-associated HO. Additionally, we performed a systematic search of MEDLINE and Embase to identify all intervention studies for weight management in patients with CP or other suprasellar tumors published until September 2017. The search yielded 1866 publications, of which 40 were included. Of these 40 studies, we identified four modalities for intervention (i.e., lifestyle, dietary, pharmacotherapeutic, or surgical) within six clinical domains (i.e., psychosocial disorders, hyperphagia, sleep disturbances, decreased energy expenditure, hyperinsulinemia, and hypopituitarism). We used the findings from our systematic review, in addition to current knowledge on the pathophysiology of HO, to develop an evidence-based treatment algorithm for patients with HO caused by CP or other suprasellar tumors. Although the individual effects of the HO interventions were modest, beneficial individual effects may be achieved when the pathophysiologic background and correct clinical domain are considered. These two aspects can be combined in an individualized treatment algorithm with a stepwise approach for each clinical domain. Recently elucidated targets for HO intervention were also explored to improve future management of HO for patients with CP and other suprasellar tumors.
颅咽管瘤(CP)和其他鞍上肿瘤引起的下丘脑性肥胖(HO)的发展导致患者生活质量下降。目前尚无针对 HO 管理的治疗算法。根据破坏的下丘脑核,导致 HO 的病理生理机制和临床症状在患者之间有所不同。在此,我们回顾了下丘脑对 CP 相关 HO 的病理生理机制和症状的贡献。此外,我们对 MEDLINE 和 Embase 进行了系统检索,以确定截至 2017 年 9 月发表的用于治疗 CP 或其他鞍上肿瘤患者体重管理的所有干预研究。该检索共产生了 1866 篇文献,其中 40 篇被纳入。在这 40 项研究中,我们确定了 6 个临床领域(即社会心理障碍、食欲过盛、睡眠障碍、能量消耗减少、高胰岛素血症和垂体功能减退)内的 4 种干预方式(即生活方式、饮食、药物治疗或手术)。我们利用系统评价的结果,以及对 HO 病理生理学的现有认识,为 CP 或其他鞍上肿瘤引起的 HO 患者制定了基于证据的治疗算法。尽管 HO 干预的个体效果不大,但如果考虑到 HO 的病理生理背景和正确的临床领域,可能会实现有益的个体效果。这两个方面可以结合起来,在每个临床领域采用逐步方法的个体化治疗算法中进行组合。我们还探讨了最近阐明的 HO 干预靶点,以改善 CP 和其他鞍上肿瘤患者的 HO 管理。