Chiaravalli Stefano, Bergamaschi Luca, Livellara Virginia, Sironi Giovanna, Puma Nadia, Nigro Olga, Gattuso Giovanna, Luksch Roberto, Terenziani Monica, Spreafico Filippo, Meazza Cristina, Podda Marta, Biassoni Veronica, Schiavello Elisabetta, Morosi Carlo, Massimino Maura, Casanova Michela, Ferrari Andrea
Pediatric Oncology Unit, Medical Oncology and Hematology Department, Fondazione IRCCS Istituto Nazionale Dei Tumori, Milan, Italy.
Radiology Department, Fondazione IRCCS Istituto Nazionale Dei Tumori, Milan, Italy.
Eur J Cancer. 2022 Jul;169:179-187. doi: 10.1016/j.ejca.2022.03.037. Epub 2022 May 14.
Though the prognosis for patients with pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) is generally good, the chances of being cured after relapse are limited. This report describes a series of relapsing NRSTS patients treated at a referral center for pediatric sarcoma, investigating the pattern of relapse, salvage rates, and factors correlating with the final outcome.
The analysis concerned 103 patients <21 years old with relapsing adult-type NRSTS treated from 1985 to 2020. For risk-adapted stratification purposes, the patient outcome was examined using univariable and multivariable analyses based on patients' clinical features at first diagnosis, first-line treatments, clinical findings at first relapse, and second-line treatments.
The first relapse occurred within 2-102 months (median 14 months) after patients' first diagnosis and was local in 47%, metastatic in 34%, and both in 19%. Treatment at relapse included chemotherapy in 72 patients, radiotherapy in 38, and surgery in 55. The median overall survival (OS) was 20 months. Post-relapse OS was 56.1%, 25.8%, and 19.1% at 1, 5, and 10 years, respectively. Cox's multivariable regression analysis showed that OS was significantly better for patients with local and late relapses (occurring more than 12 months after their first diagnosis) and for those achieving secondary remission.
The outcome of patients with recurrent NRSTS is poor. The above-mentioned variables (type and time of relapse and achievement of secondary remission) were combined in a risk-adapted model to develop a tool for estimating the chance of salvage and deciding the best second-line treatment approach.
尽管小儿非横纹肌肉瘤软组织肉瘤（NRSTS）患者的预后总体良好，但复发后治愈的机会有限。本报告描述了在一家小儿肉瘤转诊中心接受治疗的一系列复发性NRSTS患者，调查复发模式、挽救率以及与最终结局相关的因素。
该分析涉及1985年至2020年期间治疗的103例年龄小于21岁的复发性成人型NRSTS患者。为了进行风险适应性分层，根据患者首次诊断时的临床特征、一线治疗、首次复发时的临床发现以及二线治疗，采用单变量和多变量分析来检查患者的结局。
首次复发发生在患者首次诊断后的2至102个月（中位时间为14个月），其中47%为局部复发，34%为转移复发，19%为两者皆有。复发时的治疗包括72例患者接受化疗，38例接受放疗，55例接受手术。中位总生存期（OS）为20个月。复发后的1年、5年和10年总生存率分别为56.1%、25.8%和19.1%。Cox多变量回归分析显示，局部复发和晚期复发（首次诊断后超过12个月发生）的患者以及实现二次缓解的患者的总生存期明显更好。
复发性NRSTS患者的结局较差。上述变量（复发类型和时间以及二次缓解的实现情况）被纳入一个风险适应性模型，以开发一种工具来估计挽救机会并决定最佳的二线治疗方法。
