Meazza Cristina, Ferrari Andrea, Casanova Michela, Gandola Lorenza, Collini Paola, Massimino Maura, Luksch Roberto, Spreafico Filippo, Cefalo Graziella, Polastri Daniela, Terenziani Monica, Podda Marta, Cantù Giulio, Scaramuzza Davide, Fossati-Bellani Franca
Pediatric Oncology Unit, Istituto Nazionale Tumori, Via G. Venezian, 1-20133 Milano MI, Italy.
Head Neck. 2005 Jan;27(1):49-57. doi: 10.1002/hed.20117.
Ninety pediatric patients with parameningeal rhabdomyosarcoma (PM-RMS) were treated between 1970 and 2002 at the Istituto Nazionale Tumori, Milan.
Combined treatment was implemented in high-risk patients with whole-brain irradiation and intrathecal chemotherapy between 1978 and 1981 and intrathecal methotrexate alone between 1982 and 1987. Radiotherapy was given with conventional fractionation until 1988 and hyperfractionated accelerated radiotherapy (HART) thereafter.
The 5-year disease-free survival was 41.3%, increasing from 15.4% (1970-1977) to 55.9% (1989-2002) for localized cases. Local failure rates were 41% and 24% with conventional fractionation and HART, respectively. In patients treated with HART, acute toxicities were more pronounced, but the treatment compliance was better. Severe radiation-related late sequelae were observed.
The outcome for patients with PM-RMS has improved over the years but is still not as satisfactory as that for patients with RMS of other sites. Thanks to improvements in radiotherapy techniques and chemotherapy efficacy, meningeal prophylaxis is no longer necessary. Although more evidence is needed to confirm our data, the results obtained with twice-daily HART seem promising.
1970年至2002年期间，米兰国立肿瘤研究所对90例脑膜旁横纹肌肉瘤（PM-RMS）患儿进行了治疗。
1978年至1981年，对高危患者采用全脑照射和鞘内化疗联合治疗，1982年至1987年单独采用鞘内甲氨蝶呤治疗。1988年前采用常规分割放疗，此后采用超分割加速放疗（HART）。
5年无病生存率为41.3%，局限性病例从1970 - 1977年的15.4%增至1989 - 2002年的55.9%。常规分割放疗和HART的局部失败率分别为41%和24%。接受HART治疗的患者急性毒性反应更明显，但治疗依从性更好。观察到严重的放疗相关晚期后遗症。
多年来PM-RMS患者的预后有所改善，但仍不如其他部位横纹肌肉瘤患者的预后令人满意。由于放疗技术和化疗疗效的提高，脑膜预防不再必要。尽管需要更多证据来证实我们的数据，但每日两次HART取得的结果似乎很有前景。
