Moynihan Timothy J
Department of Medical Oncology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Curr Treat Options Oncol. 2003 Dec;4(6):517-23. doi: 10.1007/s11864-003-0052-5.
Ependymal tumors are rare malignancies that arise from the cells that line the ventricles and central canal of the spinal cord. Although they are more common in children, adults may also be effected by ependymal tumors. Prognosis is dependent on tumor location, histology, especially for myxopapillary tumors that tend to occur in the lumbar spine, extent of surgical resection, and stage of disease. Standard therapy consists of complete resection when feasible. The exact role of adjuvant radiotherapy in patients with radiographically confirmed complete resection is poorly defined. Patients with known residual disease may benefit from local radiation therapy, but the extent of radiation field and total dose are controversial. Even in patients treated with involved field radiotherapy, most relapses occur within the original tumor bed, thus local control remains the biggest obstacle to effective therapy. Chemotherapy has little impact against this tumor and has no role in the adjuvant setting, outside of a well designed clinical trial, with the possible exception of children younger than 5 years in an effort to delay radiation. A minority of patients may respond to one of several chemotherapy regimens at the time of recurrence, but the impact of this therapy is limited. Newer treatment strategies are needed.
室管膜瘤是一种罕见的恶性肿瘤,起源于脊髓脑室和中央管内衬的细胞。虽然它们在儿童中更为常见,但成人也可能受到室管膜瘤的影响。预后取决于肿瘤位置、组织学,特别是对于倾向于发生在腰椎的黏液乳头型室管膜瘤、手术切除范围和疾病分期。标准治疗方法是在可行的情况下进行完全切除。辅助放疗在影像学证实为完全切除的患者中的确切作用尚不明确。已知有残留病灶的患者可能从局部放射治疗中获益,但放射野范围和总剂量仍存在争议。即使在接受受累野放疗的患者中,大多数复发也发生在原肿瘤床内,因此局部控制仍然是有效治疗的最大障碍。化疗对这种肿瘤影响很小,在辅助治疗中没有作用,除非在精心设计的临床试验中,5岁以下儿童可能是个例外,目的是延迟放疗。少数患者在复发时可能对几种化疗方案中的一种有反应,但这种治疗的影响有限。需要新的治疗策略。