Occult neurohypophyseal germinomas in patients presenting with central diabetes insipidus.
Occult neurohypophyseal germinomas in patients presenting with central diabetes insipidus.
复制标题
中枢性尿崩症患者的隐匿性神经垂体生殖细胞瘤。
作者：
Tsutomu Kato;Yutaka Sawamura;Mitsuhiro Tada;Junichi Murata;Hiroshi Abe;Hiroki Shirato;Kenji Fujieda
虽然神经垂体生殖细胞瘤是尿崩症（DI）的常见初始症状，但即使通过高分辨率磁共振（MR）成像，其放射学检测也可能需要数月或数年。术语“隐匿性神经垂体生殖细胞瘤”指的是这样的病例，但其临床表现仍不清楚。在过去的5年中，7例神经垂体生殖细胞瘤患者出现DI，3例患者在DI发作时没有肿瘤证据，并作为“特发性”DI治疗。神经垂体生殖细胞瘤最终被诊断为这三名患者的肿瘤变得明显的连续磁共振成像研究和患者成功地治疗化疗和放疗。为了描述隐匿性神经垂体生殖细胞瘤的临床特征，作者分析了这些患者的内分泌学方面和MR图像，并将其与2例真正的特发性DI和4例明显的神经垂体生殖细胞瘤和DI患者进行了比较。回顾了文献中9例既往报告的病例。在生殖细胞瘤的MR图像上没有明显变化的阶段，患者通常显示垂体前叶功能障碍，特别是生长激素（GH）缺乏，或血清或脑脊液人绒毛膜促性腺激素-β升高。在出现明显的肿瘤肿块之前，垂体柄轻微肿胀伴垂体后叶正常高信号消失是MR成像的常见发现。中央DI与增大的茎，GH分泌减少，或血清人绒毛膜促性腺激素-β升高应提示隐匿性生殖细胞瘤的诊断。
Although neurohypophyseal germinoma is known be a common initial symptom in cases of diabetes insipidus (DI), its radiological detection may take months or years even by a high-resolution magnetic resonance (MR) imaging. The term "occult neurohypophyseal germinoma" denotes such cases, but its clinical picture remains obscure. Of seven patients with neurohypophyseal germinoma presenting with DI during the last 5 years, three patients showed no evidence of tumor at the onset of DI and were treated as "idiopathic" DI. Neurohypophyseal germinoma was eventually diagnosed in these three patients as the tumor became evident on sequential MR imaging studies and the patients were successfully treated with chemotherapy and radiation therapy. To delineate the clinical features of the occult neurohypophyseal germinoma, the authors analyzed endocrinological aspects and MR images in these patients and compared them with those in two patients with true idiopathic DI and four patients with overt neurohypophyseal germinoma and DI. Nine previously reported cases in the literature were reviewed. During the stage at which the germinoma gave no notable change on MR images, patients often displayed anterior pituitary dysfunction, particularly growth hormone (GH) deficiency, or an elevation of serum or cerebrospinal fluid human chorionic gonadotropin-beta. Preceding the appearance of an obvious tumor mass, a slight swelling of the pituitary stalk with loss of normal hyperintensity of the posterior pituitary lobe was a common finding on MR imaging. Central DI associated either with an enlarged stalk, decreased GH secretion, or an elevated serum human chorionic gonadotropin-beta should prompt the diagnosis of an occult germinoma.
儿童和青少年中枢神经系统生殖细胞瘤相关的激素和代谢异常及其治疗效果：10 例患者的报告。
DOI：
10.1210/jcem-52-1-9
发表时间：
1981
期刊：
The Journal of clinical endocrinology and metabolism
影响因子：
--
作者：
Sklar,CA;Grumbach,MM;Kaplan,SL;Conte,FA
通讯作者：
Conte,FA