Martínez R, Honegger J, Fahlbusch R, Buchfelder M
Department of Neurosurgery, University of Erlangen-Nürnberg, Erlangen, Germany.
Exp Clin Endocrinol Diabetes. 2003 May;111(3):162-7. doi: 10.1055/s-2003-39788.
Optico-hypothalamic gliomas (OHG) are an important differential diagnosis in suprasellar tumors. Visual impairment and hydrocephalus are the most frequent findings at presentation. However, only limited data are available about endocrine disturbances in these lesions.
38 consecutive patients (19 children) were studied retrospectively. Clinical assessment and endocrinological evaluation of pituitary and hypothalamic functions (combined pituitary stimulation test, ITT, CRH-GRH test) were performed before and after (1 week and 3 months) the neurosurgical procedure.
Only three patients showed clinical features of endocrine deficiency initially. 16 children and 15 adults out of the 38 patients (31/38, 81.6 %) showed no abnormality in their hormone values when assessed with the combined pituitary stimulation test alone or using more sophisticated investigations such as ITT and CRH-GRH test (72.2 %). Patients with hypothalamic disturbances (8 out of 38, 21 %) had endocrine abnormalities more frequently than those without (57.1 % vs. 9 %, p < 0.05), as assessed by ITT and CRH-GRH-test.
In contrast to other suprasellar tumors, endocrine deficiencies are surprisingly rare in optico-hypothalamic gliomas despite their large size, especially in the absence of hypothalamic disorders. This may be used as a criterion in the differential diagnosis of these lesions, in addition to the radiological findings.
视交叉 - 下丘脑胶质瘤（OHG）是鞍上区肿瘤的重要鉴别诊断对象。视力障碍和脑积水是其最常见的临床表现。然而，关于这些病变中内分泌紊乱的资料有限。
对38例连续患者（19例儿童）进行回顾性研究。在神经外科手术前后（1周和3个月）进行垂体和下丘脑功能的临床评估及内分泌学评价（联合垂体刺激试验、胰岛素耐量试验、促肾上腺皮质激素释放激素 - 生长激素释放激素试验）。
最初仅有3例患者表现出内分泌缺乏的临床特征。38例患者中，16例儿童和15例成人（31/38，81.6%）单独使用联合垂体刺激试验或采用更复杂的检查如胰岛素耐量试验和促肾上腺皮质激素释放激素 - 生长激素释放激素试验进行评估时，激素值无异常（72.2%）。通过胰岛素耐量试验和促肾上腺皮质激素释放激素 - 生长激素释放激素试验评估，下丘脑功能障碍患者（38例中的8例，21%）内分泌异常的频率高于无下丘脑功能障碍患者（57.1%对9%，p<0.05）。
与其他鞍上区肿瘤不同，视交叉 - 下丘脑胶质瘤尽管体积较大，但内分泌缺乏情况出人意料地罕见，尤其是在无下丘脑功能障碍时。除影像学表现外，这可作为这些病变鉴别诊断的一个标准。
