Carrie C, Lasset C, Alapetite C, Haie-Meder C, Hoffstetter S, Demaille M C, Kerr C, Wagner J P, Lagrange J L, Maire J P
Centre Léon Bérard, Lyon, France.
Cancer. 1994 Oct 15;74(8):2352-60. doi: 10.1002/1097-0142(19941015)74:8<2352::aid-cncr2820740821>3.0.co;2-h.
Medulloblastoma is a rare disease in adult patients, with an annual incidence rate of 0.05 per 100,000 per year. Results are, therefore, sparse and comprise small series over long periods. The real survival rate, the prognostic factors, the optimal postoperative radiation dose, and the role of adjuvant chemotherapy are still unknown for this disease in adults.
The authors collected 156 cases of histologically proven medulloblastoma in patients older than 18 years of age who were treated between January 1975 and December 1991 in 13 French institutions. They analyzed the prognostic factors for survival and the impact of postoperative treatment on survival.
The 5- and 10-year event free survival rates, 61 and 48%, respectively, are similar to those observed in children. The median time to recurrence is 30 months, but late relapses after 5 years remain frequent. Multivariate analysis identified postoperative performance status, spinal axis radiation dose, fourth ventricular floor involvement, and desmoplastic histologic subtype as factors significantly correlated with event free survival. No benefit of concomitant chemotherapy was demonstrated, and complete resection resulted only in severely reduced postoperative performance status.
The prognostic factors in adult medulloblastoma are comparable to those of medulloblastoma in children, but a new parameter, postoperative performance status, was identified in this adult series. Postoperative craniospinal irradiation remains the standard treatment for adults with medulloblastoma. A reduced dose to the supratentorial compartment should be tested in a prospective protocol.
髓母细胞瘤在成年患者中是一种罕见疾病,年发病率为每10万人中0.05例。因此,相关结果稀少,且长期以来仅包含少量病例系列。对于成年患者的这种疾病,其实际生存率、预后因素、最佳术后放疗剂量以及辅助化疗的作用仍不明确。
作者收集了1975年1月至1991年12月期间在法国13家机构接受治疗的156例组织学确诊的18岁以上髓母细胞瘤患者病例。他们分析了生存的预后因素以及术后治疗对生存的影响。
5年和10年无事件生存率分别为61%和48%,与儿童患者中观察到的相似。复发的中位时间为30个月,但5年后的晚期复发仍然常见。多因素分析确定术后功能状态、脊髓轴放疗剂量、第四脑室底部受累情况以及促结缔组织增生性组织学亚型是与无事件生存显著相关的因素。未显示同步化疗有获益,且完全切除仅导致术后功能状态严重下降。
成年髓母细胞瘤的预后因素与儿童髓母细胞瘤相当,但在这个成年患者系列中确定了一个新参数,即术后功能状态。术后全脑全脊髓照射仍然是成年髓母细胞瘤患者的标准治疗方法。应在前瞻性方案中测试降低幕上区域的剂量。