98例颅内生殖细胞瘤临床分析

目的分析颅内生殖细胞瘤的临床表现、实验室检查及影像学特征。方法回顾性分析我院1993年1月-2009年8月所收治的颅内生殖细胞瘤患者的临床表现、影像学特点和实验室检查等。结果98例颅内生殖细胞瘤分布在内分泌科26例、神经内科17例、神经外科46例、其他科室9例,男女比例为1.88：1,年龄2-56（17.4±8.31）岁。肿瘤位于鞍区者主要表现为尿崩症、垂体前叶功能减退、视力下降等;位于基底节区表现为偏侧肢体活动受限,言语不利等;位于松果体区表现为梗阻性脑积水所致颅高压表现及复视等。头颅MRI表现为单一或多发病灶,呈长T1、T2或等T1、T2异常结节影,均匀或不均匀强化。其中31例（31.63%）测定脑脊液HCG,HCG水平0.17-5316.98IU/L（中位数3.44IU/L）。此组患者90%以上脑脊液HCG〉0.5IU/L,90%以上脑脊液与血清HCG比值〉1.78。松果体区生殖细胞瘤脑脊液HCG水平最高,其次为基底节区,鞍区最低,但不同病变区域的脑脊液与血清HCG比值差别不大,分别为5.46、4.15和3.67,接近总体比值4.97。结论颅内生殖细胞瘤因病变的部位不同临床表现差异很大,充分认识其临床表现结合MRI以及脑脊液HCG水平有助于准确诊断。

Clinical analysis of 98 cases of intracranial germinoma

Objective To study the clinical features of intracranial germinoma.Methods Clinical manifestations,imaging characteristics and laboratory test results of 98 intracranial germinoma patients admitted to our hospital in January 1993-August 2009 were retrospectively analyzed.Results Of the 98 intracranial germinoma patients at the age of 2-56 years（mean 17.4±8.31 years）,with a male/female ratio of 1.88：1,26,17,and 46 were from the departments of endocrinology,neurology,neurosurgery,respectively,and 9 from other departments.The patients with their tumor located in the sellar region suffered from diabetes insipidus,hypopituitarism and vision disturbance.The patients with their tumor located in the basal ganglia region presented with hemiplegia and aphasia,while those with their tumor located in the pineal region had intracranial hypertension induced by obstructive hydrocephalus.MRI revealed single or multiple lesions in different parts of the brain with a long or equal T1/T2 signal and a homogeneous or heterogeneous enhancement.The HCG level in spinal fluid（csf）,measured in 31 cases（31.63%）,was 0.17IU/L-5316.98IU/L with a median value of 3.44IU/L.The HCG level was higher than 0.5IU/L in about 90% of the 31 patients with a HCG ratio in csf and serum over 1.78.The HCG level in geminoma located in the pineal region was higher than that located in the basal ganglia region.The HCG level in geminoma located in the sellar region was the lowest.The HCG ratio in csf and serum was 5.46,4.15,and 3.67,respectively,in different parts of the brain,similar to the overall ratio of 4.97.Conclusion The clinical features of intracranial germinoma located in different parts of the brain are quite different.An accurate diagnosis of intracranial germinoma can be established according to its clinical manifestations and MRI characteristics and HCG level.