The George Washington University School of Medicine, Washington, District of Columbia.
The George Washington University School of Medicine, Washington, District of Columbia.
Urol Oncol. 2021 Nov;39(11):774-780. doi: 10.1016/j.urolonc.2021.07.008. Epub 2021 Jul 29.
While survival for pediatric bladder rhabdomyosarcoma (RMS) has recently improved with risk-based multimodality treatment protocols, survival for adult bladder RMS remains to be poor. Survival is poor likely because adult bladder RMS is rare, understudied, and consequently lacking in high-level evidence to inform standardization of treatment. In addition, adult bladder RMS exhibits high rates of recurrence. The purpose of this systematic review is to determine associations between patient clinicopathologic factors and recurrence for adult primary bladder RMS, as well as to provide an updated survey of the various treatments employed for this disease in adults.
Studies involving adult primary bladder RMS were acquired from MEDLINE (OVID), Scopus, and Cochrane Central Register of Controlled Trials from 1947 to 2018. Cases with no metastatic disease at diagnosis and at least 6 months follow-up were included. Multivariable Cox-regression hazard analysis was utilized to determine associations of age, sex, histology, and TNM stage with recurrence. Kaplan-Meier analysis and log-rank testing was used to calculate overall survival (OS) for patients who underwent surgical treatment only, and to evaluate differences in survival between radical cystectomy and partial cystectomy.
20 articles were selected for the review, and 22 cases were obtained. The mean age of the patients was 55.7 ± 18.4 (range = 28-83). With a mean follow-up time of 21.4 ± 18.6 months, 36.4% of the patients experienced disease recurrence. Recurrence was not associated with age, sex, histology, or stage (p = 0.366, p = 0.754, p = 0.889, and p = 0.590, respectively). Most patients underwent surgery only as their initial therapy (n = 12), while the remaining had chemotherapy, radiation, or some combination of these therapies (including surgery). The median OS of patients who underwent surgery only was 21.0 months (95% CI: 0.0-44.6 months). Among these patients, no difference in OS between radical cystectomy and partial cystectomy was found (p = 0.841).
Adult bladder RMS is a rare, lethal tumor with a high proclivity for recurrence. No association between age, sex, histology, or stage and recurrence was found. Radical cystectomy is not superior to partial cystectomy in terms of survival, suggesting a role for bladder preservation in select patients. Our study is the first to provide a comprehensive summary of the various treatments employed with clinical outcomes for adult primary bladder RMS.
尽管儿科膀胱癌横纹肌肉瘤（RMS）的生存率最近因基于风险的多模式治疗方案而有所提高，但成人膀胱癌 RMS 的生存率仍然较差。生存率较差的原因可能是成人膀胱癌 RMS 较为罕见、研究不足，因此缺乏高级别的证据来指导治疗的标准化。此外，成人膀胱癌 RMS 的复发率较高。本系统回顾的目的是确定成人原发性膀胱 RMS 患者的临床病理因素与复发之间的关联，并提供成人膀胱癌 RMS 各种治疗方法的最新调查结果。
从 1947 年至 2018 年，从 MEDLINE（OVID）、Scopus 和 Cochrane 对照试验中心获取了涉及成人原发性膀胱 RMS 的研究。纳入了诊断时和至少 6 个月随访时无转移疾病的病例。采用多变量 Cox 回归风险分析确定年龄、性别、组织学和 TNM 分期与复发之间的关联。Kaplan-Meier 分析和对数秩检验用于计算仅接受手术治疗的患者的总生存率（OS），并评估根治性膀胱切除术与部分膀胱切除术之间的生存差异。
共选择了 20 篇文章进行综述，获得了 22 例病例。患者的平均年龄为 55.7±18.4 岁（范围 28-83 岁）。平均随访时间为 21.4±18.6 个月，36.4%的患者出现疾病复发。复发与年龄、性别、组织学或分期无关（p=0.366，p=0.754，p=0.889，p=0.590）。大多数患者仅接受手术作为初始治疗（n=12），而其余患者接受了化疗、放疗或这些治疗的联合治疗（包括手术）。仅接受手术治疗的患者的中位 OS 为 21.0 个月（95%CI：0.0-44.6 个月）。在这些患者中，根治性膀胱切除术与部分膀胱切除术之间的 OS 无差异（p=0.841）。
成人膀胱癌 RMS 是一种罕见的致命肿瘤，易复发。年龄、性别、组织学或分期与复发无关。根治性膀胱切除术在生存方面并不优于部分膀胱切除术，这表明在某些患者中保留膀胱可能具有作用。我们的研究首次提供了成人原发性膀胱 RMS 各种治疗方法及其临床结局的综合总结。
