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目的分析颅内生殖细胞瘤临床表现、检查、诊断和治疗的特点。方法对1993—2005年解放军总医院收治的45例颅内生殖细胞瘤进行回顾性分析。结果男性30例,女性15例;发病部位鞍区19例(42.2%),松果体区13例(28.9%),其他部位13例(28.9%)。临床表现颅内高压症状(53%)、视力下降及视野缺损(40%)、尿崩症(38%)、垂体前叶功能减退(27%)。影像学检查CT显示病变呈类圆形略高或高密度影,增强后明显均匀或不均匀强化。MRI显示长T1长T2或等T1等T2异常信号影,多数病变增强后明显强化。6例检查血清和脑脊液,血β-人绒毛膜促性腺激素(β-hCG)增高4例,其中2例脑脊液β-hCG也增高。诊断经手术或脑组织活检病理诊断确诊12例,经诊断性放疗确诊11例,其余诊断均根据影像结果及临床表现。治疗11例行肿瘤部分或全部切除术;9例合并梗阻性脑积水,行脑室分流术;多数患者行放疗,其中3例联合化疗,症状均有不同程度缓解。结论颅内生殖细胞瘤多发于青少年,男性多于女性,病变多在鞍区和松果体区,主要表现为颅内压升高和视力障碍,影像学检查有显著意义,血浆和脑脊液β-hCG测定有助于诊断,放射治疗是有效的治疗方法,压迫症状明显患者可行手术治疗。
Objective To analyze the clinical manifestations, examination, diagnosis and treatment of intracranial germinomas. Methods A retrospective analysis of 45 cases of intracranial germinomas admitted to PLA General Hospital from 1993 to 2005 was conducted. Results There were 30 males and 15 females. There were 19 cases (42.2%) in the sellar region, 13 cases (28.9%) in the pineal region and 13 cases (28.9%) in other parts. Clinical manifestations of intracranial hypertension symptoms (53%), vision loss and visual field defects (40%), diabetes insipidus (38%), anterior pituitary dysfunction (27%). Imaging examination CT showed lesions were slightly higher or higher density round, enhanced significantly enhanced or uneven enhancement. MRI showed long T1 long T2 or T1 and other T2 abnormal signal shadow, most lesions significantly enhanced after enhancement. Serum and cerebrospinal fluid (CSF) were detected in 6 cases. There were 4 cases of β-hCG in blood and 2 cases of β-hCG in cerebrospinal fluid. Diagnosed by surgery or brain biopsy diagnosis of pathological diagnosis of 12 cases diagnosed by diagnostic radiotherapy in 11 cases, the remaining diagnosis based on imaging findings and clinical manifestations. Treatment of 11 cases of partial or total tumor resection; 9 cases of obstructive hydrocephalus, intraventricular shunt; Most patients underwent radiotherapy, including 3 cases of combined chemotherapy, the symptoms were relieved to varying degrees. Conclusion The intracranial germinomas are more common in adolescents than in females. The lesions are mostly located in the saddle and pineal areas. The main manifestations are intracranial pressure and visual impairment. The imaging examination has significant significance. The plasma and cerebrospinal fluid β- hCG determination helps to diagnose, radiotherapy is an effective treatment, oppression symptoms are obvious in patients with operable surgery.