颅内原发性非松果体区绒癌的诊治

Diagnosis and treatment of primary intracranial non-pineal region choriocarcinoma

目的探讨颅内原发性绒癌的临床特点、诊断、治疗方法及预后。方法回顾分析了南方医院神经外科近年来收治的3例原发性非松果体区绒癌(2例鞍区、1例基底节区)的诊治。结果病例1位于鞍内并侵袭右侧海绵窦包绕颈内动脉,术前诊断为无功能性垂体腺瘤,术前1 d突发肿瘤卒中,急诊开颅手术,术后全垂体功能低下,未接受其他治疗,1个月后死亡。病例2位于鞍上三脑室,血清人绒毛膜促性腺激素(β-HCG)2300 m U/m L,行前纵裂入路手术,术后行放化疗,出院10个月后死亡。病例3位于右侧基底节区,血β-HCG 25 m U/m L,行立体定向活检明确绒癌诊断后行化疗随访中。结论颅内原发性绒癌非常罕见,以松果体区居多,鞍区、基底节区极为罕见,好发于青少年,松果体及基底节区多为男性,鞍区多为女性,其症状进展迅速,影像表现多有陈旧或新鲜出血,β-HCG是其特征性的肿瘤标记物,绒癌恶性程度高,预后极差,目前手术结合化疗已取得可喜的治疗效果。

Objective To investigate the clinical features, diagnosis, therapeutic strategy and prognosis of primary intracranial choriocarcinoma. Methods A retrospective review of three rare cases with primary intracranial non-pineal region choriocarcinoma diagnosed histopathologically was performed. Results One case was intrasellar region choriocacinoma with right cavernous sinus invaded and internal carotid artery wrapped. It was misdiagnosed with nonfunctional pituitary adenoma preoperatively. Tumor apoplexy was occurred one day before surgery. Panhypopituitarism developed after emergency opertation. The patient refused to receive any other tumor treatment and died one month later. Tumor of case 2 was located in suprasellar region and third ventricle. Serumβ-HCG was 2300 m IU/m L. Radiotherapy and chemotherapy was performed after tumor resection. The patient died ten months after being discharged. Case 3 was right basal ganglion lesion with serum β-HCG 25 m IU/m L on admission. Choriocarcinoma was diagnosed after stereotactic biopsy. The patient was receiving chemotherapy and still in follow-up. Conclusion Most of the primary intracranial choriocarcinomas are located in pineal region, only extremely rare cases occur in sellar and basal ganglion region. Male predominate in pineal and basal ganglion region tumors but female predominate in sellar region tumors. Choriocarcinoma is a highly malignant tumor which often progresses rapidly and has poor prognosis. β-HCG is a reliable marker of choriocarcinoma. Recently, chemotherapy combined with operation is the primary treatment for it, and some clinical practices have proved their effect.