20例横纹肌肉瘤患儿的临床特点及预后分析

Clinical features and prognosis in 20 children with rhabdomyosarcoma

摘要:

目的 分析儿童横纹肌肉瘤（rhabdomyosarcoma，RMS）的临床特点及影响预后的因素。 方法 纳入2014年2月至2020年9月就诊于中南大学湘雅医院儿科血液肿瘤专科的20例RMS患儿为研究对象，回顾性分析患儿的临床资料和随访资料。 结果 20例RMS患儿初次就诊时临床症状依次为无痛性肿块（13/20，65%）、眼球突出（4/20，20%）、腹痛（3/20，15%）。依据国际横纹肌肉瘤研究组（Intergroup Rhabdomyosarcoma Study Group，IRSG）的分期标准，Ⅰ期1例（5%）、Ⅱ期4例（20%）、Ⅲ期9例（45%）、Ⅳ期6例（30%）。20例患儿中位随访时间为19（3~93）个月，2年总体生存（overall survival，OS）率为79.5%（95%CI：20.1~24.3），2年无事件生存（event-free survival，EFS）率为72.0%（95%CI：19.5~23.9）。病理类型为多形性可降低RMS的2年OS率（P<0.05），有远处转移、IRSG分期为Ⅳ期、危险度为高危组可降低RMS的2年EFS率（P<0.05）。 结论 RMS初次就诊时临床症状无明显特异性，以无痛性肿块最常见，远处转移、IRSG分期、危险度分组可能与RMS患儿的预后有关。 ［中国当代儿科杂志，2022，24（9）：1036-1041］

Abstract:

Objective To study the clinical features of children with rhabdomyosarcoma (RMS) and the influencing factors for prognosis. Methods A retrospective analysis was performed on the clinical and follow-up data of 20 children with RMS who were admitted to the Department of Pediatric Hematology, Xiangya Hospital of Central South University, from June 2014 to September 2020. Results The most common clinical symptoms of the 20 children with RMS at the first visit were painless mass (13/20, 65%), exophthalmos (4/20, 20%), and abdominal pain (3/20, 15%). According to the staging criteria of Intergroup Rhabdomyosarcoma Study Group (IRSG), there was 1 child (5%) with stage I RMS, 4 (20%) with stage II RMS, 9 (45%) with stage III RMS, and 6 (30%) with stage IV RMS. The median follow-up time was 19 months for the 20 children (range: 3-93 months), with a 2-year overall survival (OS) rate of 79.5% (95%CI: 20.1-24.3) and a 2-year event-free survival (EFS) rate of 72.0% (95%CI: 19.5-23.9). Pleomorphic RMS was associated with the reduced 2-year OS rate (P<0.05), and distant metastasis, IRSG stage IV RMS, and high-risk RMS were associated with the reduced 2-year EFS rate (P<0.05). Conclusions RMS has no specific clinical symptoms at the first visit, with painless mass as the most common symptom. Distant metastasis, IRSG stage, and risk degree may be associated with the prognosis of children with RMS. Citation:Chinese Journal of Contemporary Pediatrics, 2022, 24(9): 1036-1041