Sultan Iyad, Qaddoumi Ibrahim, Rodríguez-Galindo Carlos, Nassan Anwar Al, Ghandour Khalil, Al-Hussaini Maysa
Department of Pediatric Oncology, King Hussein Cancer Centre (KHCC), Amman, Jordan.
Pediatr Blood Cancer. 2010 Jan;54(1):35-40. doi: 10.1002/pbc.22285.
Malignant rhabdoid tumors (MRTs) are aggressive and often fatal; the Surveillance, Epidemiology, and End Results (SEER) database offers an opportunity to study this rare malignancy.
From the SEER database, we extracted records of patients with a reported diagnosis of MRT and analyzed them for clinical features and survival rates by univariate and multivariate analyses.
For the 229 patients included in our data, who were diagnosed from 1986 to 2005, primary tumors were located in the central nervous system (CNS) (35%), kidneys (20%), and extra-renal non-cranial sites (ERNC-MRTs) (45%). Most patients with renal and CNS tumors were less than 18 years old (87% and 96%, respectively) while more than half of the patients with ERNC-MRTs (61%) were adults. Among staged tumors, 23% were localized, 34% regional, and 43% distant. Renal tumors had significantly more metastatic disease (47%; P = 0.006) than ERNC-MRTs. The estimated 5-year survival for the entire group was 33 +/- 3.4% (SE). Univariate and multivariate analyses showed that age at diagnosis (2-18 years), localized stage of tumors, and use of radiotherapy were significantly associated with improved survival. Adults had a better outcome than young children (<2 years old) but a poorer outcome than older children (2-18 years old); tumor stage, but not radiotherapy use, affected outcome in adults. The survival and prognostic factors of children diagnosed before and after 2000 did not differ significantly.
Our population-based study indicates that age at diagnosis, tumor stage, and use of radiotherapy favorably impact survival rates of patients with MRTs.
恶性横纹肌样瘤（MRTs）具有侵袭性，且往往是致命的；监测、流行病学和最终结果（SEER）数据库为研究这种罕见的恶性肿瘤提供了一个机会。
我们从SEER数据库中提取了报告诊断为MRT的患者记录，并通过单因素和多因素分析对其临床特征和生存率进行了分析。
在我们纳入数据的229例患者中，他们于1986年至2005年被诊断，原发肿瘤位于中枢神经系统（CNS）（35%）、肾脏（20%）和肾外非颅部位（ERNC-MRTs）（45%）。大多数肾肿瘤和CNS肿瘤患者年龄小于18岁（分别为87%和96%），而超过一半的ERNC-MRTs患者（61%）为成年人。在分期肿瘤中，23%为局限性，34%为区域性，43%为远处性。肾肿瘤的转移疾病明显多于ERNC-MRTs（47%；P = 0.006）。整个组的估计5年生存率为33±3.4%（SE）。单因素和多因素分析表明，诊断时的年龄（2至18岁）、肿瘤的局限性分期以及放疗的使用与生存率的提高显著相关。成年人的预后优于幼儿（<2岁），但比大龄儿童（2至18岁）差；肿瘤分期而非放疗的使用影响成年人的预后。2000年之前和之后诊断的儿童的生存率和预后因素没有显著差异。
我们基于人群的研究表明，诊断时的年龄、肿瘤分期和放疗的使用对MRT患者的生存率有有利影响。
