Müller Hermann L
Department of Pediatrics and Pediatric Hematology/Oncology, University Children's Hospital, Carl von Ossietzky Universität Oldenburg, Klinikum Oldenburg AöR, 26133 Oldenburg, Germany.
Biomedicines. 2025 Apr 22;13(5):1016. doi: 10.3390/biomedicines13051016.
: Craniopharyngiomas are rare sellar embryonic malformational tumors of low-grade histological malignancy. Despite high overall survival rates (92%), quality of life is frequently reduced due to adverse late effects caused by hypothalamic obesity. It is well known that morbid hypothalamic obesity is associated with the grade of hypothalamic damage. Accordingly, craniopharyngioma should be considered a paradigmatic disease, reflecting challenges in the diagnosis and treatment of acquired hypothalamic obesity. : A narrative review was performed after searching the MEDLINE/PubMed, Embase, and Web of Science databases for initial identifying articles. The search terms childhood-onset craniopharyngioma and hypothalamic obesity were used. : Despite the availability of promising therapeutic approaches, such as medication with central stimulating agents, antidiabetic drugs, glucagon-like peptide 1 (GLP1) receptor agonists, and Setmelanotide, it must be emphasized that there is currently no pharmaceutical treatment for hypothalamic obesity in craniopharyngioma proven to be effective in randomized controlled trials. For Setmelanotide, a prospective blinded randomized trial over a 12-month treatment period is ongoing. Bariatric interventions are effective, but non-reversible procedures such as bypass operations are controversial in the pediatric age group due to legal and ethical concerns. Recently, a treatment algorithm was introduced to improve the management of hypothalamic syndrome/obesity by offering more personalized treatment. Decisions on treatment strategies focusing on the preservation of visual, neuroendocrine, and hypothalamic integrity should be made by experienced multidisciplinary teams. : Treatment approaches for hypothalamic obesity are limited. Further research on novel treatment approaches for hypothalamic obesity is warranted to improve the quality of life after childhood-onset craniopharyngioma.
颅咽管瘤是一种罕见的鞍区胚胎发育异常肿瘤，组织学恶性程度低。尽管总体生存率较高（92%），但由于下丘脑肥胖引起的不良晚期效应，生活质量常常下降。众所周知，病态下丘脑肥胖与下丘脑损伤程度相关。因此，颅咽管瘤应被视为一种典型疾病，反映了后天性下丘脑肥胖诊断和治疗中的挑战。：在检索MEDLINE/PubMed、Embase和Web of Science数据库以初步确定文章后，进行了一项叙述性综述。使用了儿童期起病的颅咽管瘤和下丘脑肥胖等检索词。：尽管有前景的治疗方法，如使用中枢兴奋剂、抗糖尿病药物、胰高血糖素样肽1（GLP1）受体激动剂和Setmelanotide进行药物治疗，但必须强调的是，目前尚无在随机对照试验中被证明对颅咽管瘤下丘脑肥胖有效的药物治疗方法。对于Setmelanotide，一项为期12个月治疗期的前瞻性双盲随机试验正在进行中。减肥干预措施是有效的，但由于法律和伦理问题，像旁路手术这样的不可逆手术在儿科年龄组中存在争议。最近，引入了一种治疗算法，通过提供更个性化的治疗来改善下丘脑综合征/肥胖的管理。关于治疗策略的决策应侧重于保留视觉、神经内分泌和下丘脑完整性，应由经验丰富的多学科团队做出。：下丘脑肥胖的治疗方法有限。有必要对下丘脑肥胖的新型治疗方法进行进一步研究，以提高儿童期起病的颅咽管瘤后的生活质量。
