Rhabdomyosarcoma in middle to old-aged patients: analysis of clinicopathological features and prognosis in 76 cases

　　76例中老年横纹肌肉瘤的临床病理学特征和预后分析

　　Abstract：Objective Albeit rare,rhabdomyosarcoma (RMS) may occur in middle to old-aged adults.The purpose of this study was to investigate the clinicopathological characteristics and prognosis of RMS in patients ≥40 years of age.Methods The clinical,pathological and immunohistochemical profiles of the 76 cases were reviewed,and the outcomes were analyzed.Results There were 46 males and 30females with a median age of 55 years.Sixty cases occurred in somatic soft tissues,including extremities (n =25),head and neck (n =19) and trunk (n =16).Sixteen developed in the internal organs with predilection for the urogenital tract.Histologically,38 cases (50.0％) were of embryonal RMS (ERMS)subtype,29 cases (38.2％) of pleomorphic RMS (PRMS),7 cases (9.2％) of poorly-differentiated RMS (PdRMS),and 2 cases (2.6％) of alveolar RMS (ARMS).Immunohistiochemically,the tumor cells showed diffuse staining of desmin (68/68,100％),with a variable expression of myogenin (30/43,69.8％),MyoD1 (33/51,64.7％) and MSA (29/40,72.5％).The therapy data available in 54 cases showed that three inoperable cases were treated with chemotherapy and radiotherapy,51 cases underwent local excision,including 10 cases with chemotherapy,9 cases with radiotherapy,8 cases with chemotherapy and radiotherapy after surgery,and the remaining 24 cases were not administrered with postoperative adjuvant therapy.The follow-up data available in 54 cases showed that 22 patients were alive with unresectable or recurrent/disease and 32 patients were alive with no evidence of disease.The median disease-free and overall survivals were 6.0 months and 7.0 months,respectively.Twenty-seven patients exhibited progression of disease with recurrence in 8 cases and metastasis in 19 cases.The median interval to progression was 6.0 months.Conclusions The most common subtypes of RMS in middle to old aged patients are ERMS and PRMS.In general,Rhabdomyosarcomas of middle to old aged patients shows a highly aggressive behavior.Patients may benefit from multi-disciplinary therapy.

　　摘要：目的 去调查76例 40岁以上的横纹肌肉瘤临床病理学特征及预后。方法：回顾临床的，病理学的，免疫组织学的个人档案，然后分析结果。结果 46名男性和30岁女性 平均年龄55岁。60例发生在软组织，包括四肢(n = 25),头部和颈部(n = 19)和躯干(n = 16)。16例发生在内脏，好发于生殖系统。组织结构上，38例(50.0%)胚的RMS(ERMS)亚型，29例(38.2%)的多形性RMS(PRMS)，7 例 (9.2％) 无差别RMS (PdRMS），和2例(2.6%)的肺泡RMS(ARMS)。免疫组织学，染色肿瘤细胞，及myogenin (30/43,69.8％),MyoD1 (33/51,64.7％) and MSA (29/40,72.5％)的表达。

　　治疗54例数据显示：3例接受化疗和放疗，9例放射治疗，8术后例化疗和放疗，8例没有术后辅助治疗。随访54例发现22例带瘤存活 没有切除或复发和32例无瘤。完全健康和整体存活的平均值分别是6个月和7个月。27例病情更严重，8例复发19例转移。病情进展平均存活时间6个月。结论 RMS 中老年最常见类型是 ERMS and PRMS。通常中老年人横纹肌肉瘤显示很高侵润性。患者更适合多种规律治疗。