Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, Beijing, P.R. China.
J Pediatr Endocrinol Metab. 2023 Oct 30;36(12):1115-1127. doi: 10.1515/jpem-2023-0368. Print 2023 Dec 15.
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.
颅内生殖细胞肿瘤(iGCTs)是一种罕见的恶性肿瘤,主要影响儿童和青少年。iGCTs 的发病率、临床表现和预后存在高度异质性。既往研究主要集中在消除肿瘤、降低肿瘤复发率和提高生存率上,而忽视了肿瘤及其治疗对神经内分泌功能的影响。在疾病的整个过程中,可能会发生神经内分泌功能障碍,而肿瘤学家、神经外科医生和放射科医生往往会忽略这一点。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侧重点。从发病到诊断阶段,大多数 iGCT 患者可能出现与神经内分泌功能受损相关的症状,甚至以这些症状作为首发症状。特别是,少数鞍区/鞍上病变患者在初始症状出现很久后,可能出现典型的影像学特征和肿瘤标志物升高。这会进一步使诊断复杂化。在肿瘤周围治疗阶段,神经内分泌功能会发生动态变化,需要进行动态评估。一旦出现尿崩症和下丘脑-垂体-肾上腺轴及下丘脑-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,评估生长发育以及相应的激素替代治疗是最关注和最复杂的问题。因此,本文综述了内分泌学家在 iGCTs 不同阶段的关注点。