脊髓内黑色素性室管膜瘤1例报告及文献复习

目的探讨脊髓内黑色素性室管膜瘤的疾病特点、诊断及治疗。方法分析北京天坛医院神经外科收治的1例椎管内黑色素性室管膜瘤病例,并复习相关文献。结果核磁MRI影像表现为T1高信号,T2低信号,增强稍见强化。对比放疗前后4个月的磁共振影像,该肿瘤大小无明显变化。组织学检查见肿瘤细胞内含黑色素颗粒,经氧化、脱色后行HE染色见不典型的血管周围假菊形团和室管膜菊形团结构。肿瘤大部切除后患者神经功能障碍加重。随访5个月,神经功能障碍渐恢复,复查MRI肿瘤无增大。结论黑色素性室管膜瘤因瘤内含有丰富的黑色素而在影像显示T1高信号,T2低信号。组织学检查结合肿瘤与室管膜的密切关系可确定诊断。手术是最佳治疗方案,但切除程度应予个体化,不建议术前放疗。

Intramedullary melanotic ependymoma:a case report and literature review

To explore the features,diagnosis and therapy of intramedullary melanotic ependymoma. Methods A intramedullary melanotic ependymoma patient was treated in the Neurosurgery Department of Beijing Tiantan Hospital. The data were analyzed and literature was reviewed. Results The MR imaging had shown hyperintensity at T1 weighted imaging,hypointensity at T2 weighted imaging and light enhancement after enhancement scanning. The size of the tumor didn't change significantly at MR imaging compared to 4 months before radiotherapy. Histological examination showed melanin granule inside the tumor cell,but with atypical perivascular pseudorosettes and ependymal rosettes of the ependymoma in HE stain after the pathological section were treated by oxidation and decolorization. The nervous system functional impairment deteriorated after the tumor was partially resected,but grandually recovered without increasing at MR imaging during the 5 months follow -up.Conclusions With aboudent melanin granule inside the tumor cells,MRI of melanotic ependymoma displays hyperintensity in Tl-weighted imaging and hypointensity in T2-weighted imaging. His-tological examination combined with close relationship between the tumor and ependyma can yield the final diagno-sis.Surgery is the best therapeutic regimen for the tumor,but the extent of excision should be individualized. Considering insensitive to radiotherapy in short term,preoperative radiotherapy is not recommended.