Liang Dan, Chen Han, Zhong Li-Yong
Department of Endocrinology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Endocr Connect. 2023 Jul 26;12(8):e230168. doi: 10.1530/EC-23-0168.
Intracranial germ cell tumors frequently arise from the midline of the brain, occasionally presenting as bifocal diseases. The predominant lesion might affect clinical characteristics and neuroendocrine outcomes.
A retrospective cohort study involving 38 patients with intracranial bifocal germ cell tumors was performed.
Twenty-one patients were assigned to the sellar-predominant group, while the other 17 patients were assigned to the non-sellar-predominant group. Differences in gender ratio, age, manifestation, the incidence of metastasis, the incidence of elevated tumor markers, human chorionic gonadotropin levels in serum and in cerebrospinal fluid, diagnostic method, and tumor type were not significant between the sellar-predominant group and the non-sellar-predominant group. Before treatment, the sellar-predominant group had a higher incidence of adenohypophysis hormone deficiencies and central diabetes insipidus than those of the non-sellar-predominant group, without significant differences. After multidisciplinary therapy, the sellar-predominant group also had a higher incidence of adenohypophysis hormone deficiencies and central diabetes insipidus than those of the non-sellar-predominant group. The differences in the hypothalamic-pituitary-adrenal (HPA) axis impairment (P = 0.008), hypothalamic-pituitary-thyroid (HPT) axis impairment (P = 0.048), and hypothalamic-pituitary-gonad (HPG) axis impairment (P = 0.029) were significant between sellar-predominant group and non-sellar-predominant group, while the others were not. At median 6 (3, 43) months of follow-up visit, sellar-predominant group had a higher incidence of adenohypophysis hormone deficiencies than those of non-sellar-predominant group. The differences in the HPA impairment (P = 0.002), HPT impairment (P = 0.024), and HPG impairment (P < 0.000) were significant, while the others were not. Further comparison of the neuroendocrine function between different subtypes of sellar-predominant patients indicated that the differences in adenohypophysis hormone deficiencies and central diabetes insipidus were not significant between the two subtype groups.
Bifocal patients with different predominant lesions present similar manifestations and neuroendocrine disorders before treatment. Non-sellar-predominant patients would have better neuroendocrine outcomes after tumor treatment. The distinction of the predominant lesion in patients with bifocal intracranial germ cell tumor plays a valuable role in predicting neuroendocrine outcomes, as well as in optimizing long-term neuroendocrine management during survival time.
颅内生殖细胞肿瘤常起源于脑中线，偶尔表现为双灶性疾病。主要病灶可能影响临床特征和神经内分泌结局。
对38例颅内双灶性生殖细胞肿瘤患者进行回顾性队列研究。
21例患者被归入鞍区为主型组，另外17例患者被归入非鞍区为主型组。鞍区为主型组和非鞍区为主型组在性别比例、年龄、临床表现、转移发生率、肿瘤标志物升高发生率、血清和脑脊液中人绒毛膜促性腺激素水平、诊断方法及肿瘤类型方面差异均无统计学意义。治疗前，鞍区为主型组腺垂体激素缺乏症和中枢性尿崩症的发生率高于非鞍区为主型组，但差异无统计学意义。多学科治疗后，鞍区为主型组腺垂体激素缺乏症和中枢性尿崩症的发生率仍高于非鞍区为主型组。鞍区为主型组与非鞍区为主型组在下丘脑 - 垂体 - 肾上腺（HPA）轴损害（P = 0.008）、下丘脑 - 垂体 - 甲状腺（HPT）轴损害（P = 0.048）及下丘脑 - 垂体 - 性腺（HPG）轴损害（P = 0.029）方面差异有统计学意义，其他方面差异无统计学意义。在中位随访6（3，43）个月时，鞍区为主型组腺垂体激素缺乏症的发生率高于非鞍区为主型组。HPA轴损害（P = 0.002）、HPT轴损害（P = 0.024）及HPG轴损害（P < 0.000）方面差异有统计学意义，其他方面差异无统计学意义。对鞍区为主型患者不同亚型之间的神经内分泌功能进行进一步比较表明，两个亚型组之间腺垂体激素缺乏症和中枢性尿崩症的差异无统计学意义。
不同主要病灶的双灶性患者在治疗前表现出相似的临床表现和神经内分泌紊乱。非鞍区为主型患者在肿瘤治疗后神经内分泌结局较好。颅内双灶性生殖细胞肿瘤患者主要病灶的区分在预测神经内分泌结局以及优化生存期间的长期神经内分泌管理方面具有重要价值。
