颅内生殖细胞肿瘤（iGCT）是一种罕见的恶性肿瘤，主要影响儿童和青少年。 iGCT 的发生率、临床表现和预后表现出高度异质性。以往的研究主要集中于消除肿瘤、减少肿瘤复发、提高生存率，而忽视了肿瘤及其治疗对神经内分泌功能的影响。在整个疾病过程中，可能会出现神经内分泌功能障碍，但经常被肿瘤科医生、神经外科医生和放射科医生忽视。然而，内分泌学家对这个问题更感兴趣，并且在疾病的不同阶段有不同的优先事项。从发病到诊断阶段，大多数 iGCT 患者可能会出现与神经内分泌功能受损相关的症状，甚至将这些症状作为该病的第一个指征。特别是，少数患有鞍区/鞍上病变的患者可能在初始症状出现后很长时间内表现出典型的影像学特征和肿瘤标志物升高。这会使诊断过程进一步复杂化。瘤周治疗阶段，神经内分泌功能呈现动态变化，需要动态评估。一旦出现尿崩症、下丘脑-垂体-肾上腺、下丘脑-垂体-甲状腺轴功能障碍，应及时给予激素替代治疗，以确保患者肿瘤治疗的成功。随后，在肿瘤治疗完成后的长期管理阶段，生长发育的评估以及相应的激素替代治疗是最令人关注和复杂的问题。因此，本文回顾了不同阶段内分泌学家对 iGCT 的兴趣。© 2023 Walter de Gruyter GmbH，柏林/波士顿。

Intracranial germ cell tumors (iGCTs) are rare malignant neoplasms that mainly affect children and adolescents. The incidence, clinical presentation, and prognosis of iGCTs exhibit high heterogeneity. Previous studies have primarily focused on eliminating tumors, reducing tumor recurrence, and improving survival rates, while neglecting the impact of the tumors and their treatment on neuroendocrine function. Throughout the entire course of the disease, neuroendocrine dysfunction may occur and is frequently overlooked by oncologists, neurosurgeons, and radiologists. Endocrinologists, however, are more interested in this issue and have varying priorities at different stages of the disease. From onset to the diagnostic phase, most patients with iGCTs may present with symptoms related to impaired neuroendocrine function, or even experience these symptoms as their first indication of the condition. Particularly, a minority of patients with sellar/suprasellar lesions may exhibit typical imaging features and elevated tumor markers long after the onset of initial symptoms. This can further complicate the diagnosis process. During the peritumor treatment phase, the neuroendocrine function shows dynamic changes and needs to be evaluated dynamically. Once diabetes insipidus and dysfunction of the hypothalamic-pituitary-adrenal and hypothalamic-pituitary-thyroid axes occur, hormone replacement therapy should be administered promptly to ensure successful tumor treatment for the patient. Subsequently, during the long-term management phase after the completion of tumor treatment, the evaluation of growth and development as well as corresponding hormone replacement therapy are the most concerning and complex issues. Thus, this paper reviews the interest of endocrinologists in iGCTs at different stages.© 2023 Walter de Gruyter GmbH, Berlin/Boston.