Brandes Alba A, Franceschi Enrico, Tosoni Alicia, Blatt Valeria, Ermani Mario
Department of Medical Oncology, Azienda Unità Sanitaria Locale (USL) Bellaria-Maggiore Hospital, Bologna, Italy.
Cancer. 2007 Nov 1;110(9):2035-41. doi: 10.1002/cncr.23003.
Because medulloblastoma (MB) is rare in adults, the few studies on this condition have been retrospective, and the follow-up has tended to be short. Furthermore, the different therapeutic strategies used in these patients has made it difficult to assess survival rates and prognostic factors.
In 1989, a prospective Phase II trial was initiated to evaluate the efficacy of treatment for adults with MB. Patients were staged completely with a neuroradiologic examination of the brain and neuroaxis and by cerebrospinal fluid cytology, according to Chang's staging system. Low-risk patients received radiotherapy alone, whereas high-risk patients received 2 cycles of upfront chemotherapy followed by radiotherapy and adjuvant chemotherapy. The current article reports on the long-term results from that trial.
After a median follow up of 7.6 years, among a total of 36 adults with MB, the overall progression-free survival (PFS) and overall survival (OS) rates at 5 years were 72% and 75%, respectively. In low-risk patients, the 5-year PFS rate was 80%, and the 5-year OS rate was 80%; in high-risk patients, the 5-year PFS rate was 69%, and the 5-year OS rate was 73%.
In adult patients with MB, long-term follow-up was essential for evaluating the real impact of treatments. Low-risk and high-risk patients did not differ significantly in terms of PFS or OS.
由于成神经管细胞瘤（MB）在成人中较为罕见，针对这种疾病的少数研究都是回顾性的，且随访时间往往较短。此外，这些患者所采用的不同治疗策略使得评估生存率和预后因素变得困难。
1989年启动了一项前瞻性II期试验，以评估成人MB治疗的疗效。根据Chang分期系统，通过脑部和神经轴的神经放射学检查以及脑脊液细胞学检查对患者进行全面分期。低风险患者仅接受放疗，而高风险患者接受2个周期的前期化疗，随后进行放疗和辅助化疗。本文报道了该试验的长期结果。
在中位随访7.6年后，在总共36例成人MB患者中，5年时的无进展生存率（PFS）和总生存率（OS）分别为72%和75%。在低风险患者中，5年PFS率为80%，5年OS率为80%；在高风险患者中，5年PFS率为69%，5年OS率为73%。
在成年MB患者中，长期随访对于评估治疗的实际影响至关重要。低风险和高风险患者在PFS或OS方面没有显著差异。
