成人髓母细胞瘤患者脑(脊)膜播散种植的诊断与治疗

Diagnosis and treatment of adult medulloblastoma seeding in the intracranial-spinal subarachnoid space

目的探讨成人髓母细胞瘤患者发生脑(脊)膜播散种植后的临床诊断与治疗策略。方法回顾分析11例成人髓母细胞瘤患者发生脑(脊)膜播散种植后的临床表现、脑脊液细胞学特点、影像学表现和治疗措施。结果 11例患者中10例采用枕下后正中入路、1例采用乙状窦后入路全切除肿瘤,恢复脑脊液循环通路。7例患者术后放射治疗前复查MRI即发现脑(脊)膜播散种植,予以放射治疗和药物化疗等综合治疗后好转;4例术后复查MRI未见脑(脊)膜播散种植,行常规放射治疗,随访3个月至3年(平均20个月),复查MRI显示脑(脊)膜播散种植。仅2例患者术前脑脊液细胞学检查发现肿瘤细胞。所有患者均行放射治疗和药物化疗,生存2例、死亡9例。结论成人髓母细胞瘤发生脑(脊)膜播散种植者预后较差,MRI较脑脊液细胞学更为敏感,予放射治疗联合药物化疗的综合治疗措施可以延长生存期、提高生活质量。

Objective To investigate the clinical diagnosis and treatment of adult medulloblastoma seeding in the intracranial-spinal subarachnoid space. Methods Eleven cases of adult medulloblastoma seeding in the intracranial-spinal subarachnoid space were retrospectively analyzed on the clinical features, cerebrospinal fluid （CSF） cytology, radiological characteristics and treatments. Results All patients underment neurosurgical procedures to remove medulloblastomas. In 10 patients, tumor was removed through suboccipital posterior midline approach and in one patient through post-sigmoid sinus approach. In 7 patients tumor cell seeding was found in the intracranial-spinal subarachnoid space before postoperative radiotherapy and disappeared after radiological and chemical treatment, while in other 4 patients tumor cell seeding was found in the intracranial-spinal subarachnoid space at 3 months to 3 years follow-up period （average 20 months） after radiotherapy. In 2 of all the patients tumor ceils were found by CSF cytology before operation. All the patients were treated with radiotherapy and adjuvant chemotherapy. Two patients were still alive, while 9 patients were dead. Conclusions Patients with adult medulloblastoma seeding in intracranial-spinal subarachnoid space have a poor prognosis. In the diagnosis of adult medulloblastomas seeding in the intracranial-spinal subarachnoid space, MRI is more sensitive than CSF cytology. Once the seeding in intracranial- spinal subarachnoid space was found, the patients should be treated with radiotherapy and adjuvant chemotherapy, which can prolong the survival time and improve the quality of life.