腺泡状横纹肌肉瘤41例临床病理特征及预后分析  

Alveolar rhabdomyosarcoma:clinicopathologic characteristics and prognostic analysis of 41 cases

作　　者：秦玲[1] 李道明[1] QIN Ling;LI Dao-ming(Department of Pathology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院病理科,郑州450052

出　　处：《临床与实验病理学杂志》2023年第7期822-828,共7页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨腺泡状横纹肌肉瘤(alveolar rhabdomyosarcoma,ARMS)的临床病理学特征,分析其预后影响因素。方法收集41例ARMS和97例胚胎性横纹肌肉瘤(embryonal rhabdomyosarcoma,ERMS)的临床病理资料。对比分析两者的临床病理学特征。另对41例ARMS患者进行随访,分析其预后影响因素。结果41例ARMS中男性29例,女性12例,男女比为2.4∶1。发病年龄范围1~74岁,平均年龄22.95岁,中位年龄18.0岁。<18岁者19例,≥18岁者22例。发病部位:头颈部32例,非头颈部9例。与ERMS相比,ARMS的发病患者年龄更大,更易发生区域淋巴结转移,危险度更高(P均<0.05)。41例ARMS患者中33例获得随访,随访时间2~62.3个月。随访过程中,23例死亡,10例存活。平均生存时间23.9个月,中位生存时间17.9个月。22例成人ARMS患者中17例获得随访。随访过程中,15例死亡,2例存活。平均生存时间14.7个月,中位生存时间12.7个月。年龄和N分期均与ARMS患者预后相关(P均<0.05),但仅年龄是影响ARMS患者预后的独立因素(P<0.05)。MyoD1表达、N分期、M分期、TNM分期、IRS分期以及危险度分层与成人ARMS患者预后相关(P均<0.05)。结论ARMS发病率低,临床病理学特征与ERMS不同,生物学行为更具侵袭性。Purpose To explore the clinicopathological features of alveolar rhabdomyosarcoma(ARMS)and to analyze the prognostic factors of ARMS.Methods The clinicopatholog-ical data of 41 cases of ARMS and 97 cases of embryonal rhab-domyosarcoma(ERMS)were collected.The clinical pathological characteristics were compared and analyzed between the two tumors.In addition,41 patients with ARMS were followed up to analyze the prognostic factors.Results Among the 41 cases of ARMS,there were 29 males and 12 females,with a ratio of 2.4∶1.The age of onset ranged from 1 year to 74 years,with an average age of 22.95 years and a median age of 18.0 years.19 cases were under 18 years old,and 22 cases were over 18 years old.32 cases were located in the head and neck and 9 cases were non-head and neck.Compared with ERMS,ARMS had an older age of onset and were more likely to develop regional lymph node metastasis,with a higher risk(all P<0.05).Among the 41 patients with ARMS,33 patients were followed up.The follow-up time ranged from 2 months to 62.3 months.During follow-up,23 patients died and 10 patients survived.The average survival time was 23.9 months,and the median survival time was 17.9 months.Among the 22 patients with adult ARMS,17 patients were followed up.During follow-up,15 patients died and 2 patients survived.The average survival time was 14.7 months,and the median survival time was 12.7 months.Both age and lymph node stage were associated with the prognosis of ARMS patients(all P<0.05),but only age was an independent factor affecting the prognosis of ARMS patients(P<0.05).MyoD1 expression,N stage,M stage,TNM stage,IRS stage and risk stratification were associated with the prognosis of adult ARMS patients(all P<0.05).Conclusion The incidence rate of ARMS is low,its clinicopathological characteristics are different from that of ERMS,and its biological behavior is more invasive.

关 键 词：横纹肌肉瘤 腺泡状 胚胎性 临床病理特征 预后 免疫组织化学 

分 类 号：R738.6[医药卫生—肿瘤]