Diagnosis and treatment of primary intracranial non-pineal region choriocarcinoma
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摘要:
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 mU/mL，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 mU/mL，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。 -
关键词:
- 绒毛膜癌 /
- 肿瘤，生殖细胞和胚胎性 /
- 诊断 /
- 化学疗法 /
Abstract:Objective To investigate the clinical features, diagnosis, therapeutic strategy and prognosis of primary intracranialchoriocarcinoma. Methods A retrospective review of three rare cases with primary intracranial non-pineal regionchoriocarcinoma diagnosed histopathologically was performed. Results One case was intrasellar region choriocacinoma withright cavernous sinus invaded and internal carotid artery wrapped. It was misdiagnosed with nonfunctional pituitaryadenoma preoperatively. Tumor apoplexy was occurred one day before surgery. Panhypopituitarism developed afteremergency opertation. The patient refused to receive any other tumor treatment and died one month later. Tumor of case 2 waslocated in suprasellar region and third ventricle. Serumβ-HCG was 2300 mIU/mL. Radiotherapy and chemotherapy wasperformed after tumor resection. The patient died ten months after being discharged. Case 3 was right basal ganglion lesionwith serum β-HCG 25 mIU/mL on admission. Choriocarcinoma was diagnosed after stereotactic biopsy. The patient wasreceiving chemotherapy and still in follow-up. Conclusion Most of the primary intracranial choriocarcinomas are located inpineal region, only extremely rare cases occur in sellar and basal ganglion region. Male predominate in pineal and basalganglion region tumors but female predominate in sellar region tumors. Choriocarcinoma is a highly malignant tumor whichoften progresses rapidly and has poor prognosis. β-HCG is a reliable marker of choriocarcinoma. Recently, chemotherapycombined with operation is the primary treatment for it, and some clinical practices have proved their effect. -
Key words:
- choriocarcinomas /
- neoplasms /
- germ cell and embryonal /
- diagnosis /
- chemotherapy
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图 2 病例2检查结果
A, B: MRI矢状增强轴位T2WI平扫及病理HE染色显示鞍上及三脑室内混杂信号影, 脑室扩大, 增强扫描呈环形强化, 强化边界不均一, T2WI平扫呈中间高周边低信号. C: 病理HE染色可见坏死组织及出血, 有大而异型的合体滋养层细胞, 瘤细胞大小不一, 核膜厚, 核仁明 显, 异型性明显, 核分裂多见（×400）.
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