Rhabdomyosarcoma in Children: A SEER Population Based Study
Rhabdomyosarcoma in Children: A SEER Population Based Study
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DOI：
10.1016/j.jss.2011.03.001
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发表时间：
2011-10-01
影响因子：
2.2
通讯作者：
Sola, Juan E.
中科院分区：
文献类型：
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作者：
Perez, Eduardo A.;Kassira, Noor;Sola, Juan E.
Objective.检查儿童横纹肌肉瘤（RMS）的发病率和结局。方法。SEER注册表检查了20岁以下RMS患者。总体而言，确定了1544例患者，发生率为0.4414/100，000/年。男女比例为3：2。肿瘤分为胚胎型（67%）、腺泡型（32%）和多形性（1%）。肺泡型和多形性RMS在青少年中更常见，而胚胎型在年幼儿童中更常见（P = 0.0001）。多形性型（47%）和腺泡型（39%）RMS通常表现为远处病变，而胚胎型（25%）RMS则相反。大多数患者接受手术切除（81%）和放射治疗（63%）。总体而言，5年和10年生存率分别为60%和57%。单因素分析表明，年龄< 10岁、局部分期、良好部位、胚胎型、肿瘤大小< 5 cm和手术切除的生存率较高。多因素分析显示非胚胎型（HR 1.451）、非有利部位（HR 1.570）、未手术（HR 1.726）、年龄≥ 10岁（HR 1.734）、1973-1978年诊断年（HR 1.730）和远处疾病（HR 3.456）是死亡率的独立预测因素。胚胎组织学是儿童RMS最常见的类型，出现在幼儿中，预后比肺泡型或多形性型好。胚胎性肿瘤、肿瘤位置良好、年龄< 10岁、局限性病变和手术切除的患者生存率提高。(C)2011 Elsevier Inc. All rights reserved.
Objective. To examine incidence and outcomes for pediatric rhabdomyosarcoma (RMS).Methods. The SEER registry was examined for patients with RMS < 20 y old.Results. Overall, 1544 patients were identified for an incidence of 0.4414/100,000 per year. Males outnumbered females 3:2. Tumors were classified as embryonal (67%), alveolar (32%), and pleomorphic (1%). Alveolar and pleomorphic RMS were more common in adolescents, whereas embryonal type was more common in younger children (P = 0.0001). Pleomorphic (47%) and alveolar (39%) RMS commonly presented with distant disease, in contrast to embryonal (25%). Most patients had surgical resection (81%) and radiotherapy (63%). Overall, 5- and 10-y survival was 60% and 57%, respectively. Univariate analysis identified higher survival for age < 10 y, local stage, favorable site, embryonal type, < 5 cm tumor size, and surgical resection. Multivariate analysis identified non-embryonal type (HR 1.451), non-favorable site (HR 1.570), no surgery (HR 1.726), age >= 10 y (HR 1.734), 1973-1978 diagnosis year (HR 1.730), and distant disease (HR 3.456) as independent predictors of mortality.Conclusions. Embryonal histology, the most common type of pediatric RMS, presents in young children and has better prognosis than alveolar or pleomorphic types. Patients with embryonal tumors, favorable tumor location, age < 10 y, localized disease, and surgical resection have improved survival. (C) 2011 Elsevier Inc. All rights reserved.