The aim of the study was to analyze the long-term outcome (>20 years) after treatment of posterior fossa medulloblastoma (MB) in childhood. We analyzed data from patients treated for posterior fossa MB between 1974 (introduction of the first international treatment protocol in Norway) and 1987 (when use of radiotherapy was abandoned in children under 4 years of age). Out of 47 children, 24 survived >20 years. At the time of analysis, 16 patients (median age 41 years, range 32-52) were alive (median follow-up 34 years, range 30-42), while 8 patients died 22-41 years (median 31 years) after primary treatment: one late death (after 22 years) was due to tumor recurrence whilst other 7 deaths (after 23 to 41 years) were related to the detrimental effects of the treatment (secondary tumors, stroke, severe epilepsy and depression). Observed 20- and 30-year survival rates were 51% and 44%, respectively. Despite successful treatment of MB in childhood and satisfactory tumor control during the first 20 years following primary treatment, our data indicates that even long-term survivors may die from tumor recurrence. However, the main factors causing late mortality and morbidity in long-term survivors seem to be the complications related to radiotherapy given in childhood.

译文

该研究的目的是分析儿童期后颅窝髓母细胞瘤 (MB) 治疗后的长期结果 (>20年)。我们分析了接受后颅窝MB 1974年 (在挪威引入了第一个国际治疗方案) 和1987 (在4岁以下儿童中放弃放疗时) 治疗的患者的数据。在47名儿童中,有24名存活了20年以上。在分析时,16例患者 (中位年龄41岁,范围32-52) 存活 (中位随访34年,范围30-42),而8例患者在初次治疗后死亡22-41年 (中位31年): 1例晚期死亡 (22年后) 是由于肿瘤复发,而其他7例死亡 (23至41年后) 与治疗的有害影响 (继发性肿瘤,中风,严重癫痫和抑郁症) 有关。观察到的20年和30年生存率分别为51% 和44%。尽管在儿童时期成功治疗了MB,并且在初次治疗后的前20年中肿瘤控制令人满意,但我们的数据表明,即使是长期幸存者也可能死于肿瘤复发。然而,导致长期幸存者晚期死亡率和发病率的主要因素似乎是与儿童时期放疗有关的并发症。

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