Bouffet E, Doz F, Demaille M C, Tron P, Roche H, Plantaz D, Thyss A, Stephan J L, Lejars O, Sariban E, Buclon M, Zücker J M, Brunat-Mentigny M, Bernard J L, Gentet J C
Department of Paediatric Oncology, Centre Leon Bérard, Lyon, France.
Br J Cancer. 1998 Apr;77(8):1321-6. doi: 10.1038/bjc.1998.220.
Early detection of relapse has been advocated to improve survival in children with recurrent medulloblastoma. However, the prognostic factors and the longer term outcome of these patients remains unclear. Pattern of recurrences were analysed in three consecutive protocols of the Société Française d'Oncologie Pédiatrique (1985-91). A uniform surveillance programme including repeated lumbar puncture combined with computerized tomography (CT) or magnetic resonance imaging (MRI) scan was applied for all registered patients. Forty-six out of 116 patients had progressive or recurrent disease. The median time from diagnosis to recurrence was 10.5 months and 76% relapses occurred during the first 2 years. Seventeen patients had asymptomatic relapses that were detected by the surveillance protocol. Forty-one patients were treated at time of progression. Twenty-three responded to salvage therapy and 11 achieved a second complete remission. The median survival time after progression was 5 months (<1-41 months), and only two patients remained alive at time of follow-up. Length of survival is primarily related to some specific patterns of relapse (time from diagnosis to recurrence, circumstances of relapse, extent of relapse) and to the response to salvage therapy. No evidence of long-term benefit appeared from any form of treatment.
早期发现复发性髓母细胞瘤患儿的复发情况,一直被提倡用于改善其生存率。然而,这些患者的预后因素及长期结局仍不明确。在法国儿科肿瘤学会的三个连续方案(1985 - 91年)中,对复发模式进行了分析。对所有登记患者实施了统一的监测方案,包括重复腰椎穿刺并结合计算机断层扫描(CT)或磁共振成像(MRI)扫描。116例患者中有46例出现疾病进展或复发。从诊断到复发的中位时间为10.5个月,76%的复发发生在头两年内。17例患者出现无症状复发,通过监测方案得以发现。41例患者在疾病进展时接受了治疗。23例患者对挽救性治疗有反应,11例实现了第二次完全缓解。进展后的中位生存时间为5个月(1 - 41个月),随访时仅有2例患者存活。生存时间主要与某些特定的复发模式(从诊断到复发的时间、复发情况、复发范围)以及对挽救性治疗的反应有关。未发现任何形式的治疗有长期益处。