Department of Radiation Oncology, University of Utah School of Medicine, Salt Lake City, Utah.
Department of Urology, University of Utah School of Medicine, Salt Lake City, Utah.
Cancer Med. 2018 Dec;7(12):6030-6039. doi: 10.1002/cam4.1872. Epub 2018 Nov 19.
Primary prostate sarcomas (PPS) are rare. Outcomes for this cancer have not been well characterized.
Subjects with a PPS diagnosed between 1973 and 2014 were identified in the SEER database. Subjects were stratified by disease stage and types of therapies received. Disease-specific survival (DSS) and Overall survival (OS) was estimated by Kaplan-Meier analysis and cohorts were compared with a univariate and multivariable Cox regression.
The incidence of PPS among all prostate cancer diagnoses was 0.02%. Subjects younger than age 26 years at diagnosis represented 29% of cases, and 32% of primary prostate sarcomas were rhabdomyosarcoma histology.
The median age at diagnosis was 9 years. Between age 0-25 years rhabdomyosarcoma accounted for 96.4% of primary prostate sarcoma diagnoses, after age 25 rhabdomyosarcoma represented 15% of new diagnoses. The 10-year DSS and OS for rhabdomyosarcoma was 47% and 44%.
NON-RHABDOMYOSARCOMA HISTOLOGIES: The median age at diagnosis was 71 years. The most common diagnoses were leiomyosarcoma (33%) and carcinosarcoma (28%). Localized, regional, or distant disease occurred in 40%, 34%, and 26% of cases. The 10-year DSS and OS were 26% and 14%. In locally advanced cases, RT added to surgery trended toward improved DSS (P = 0.10).
Disease-specific survival and OS for non-rhabdomyosarcoma histologies appear inferior to those of rhabdomyosarcoma. The addition of RT to surgical resection may improve DSS in locally advanced non-rhabdomyosarcoma. This is the largest report of the incidence, stage distribution, and survival for this extremely rare urologic malignancy providing valuable prognostic information.
原发性前列腺肉瘤（PPS）较为罕见。目前对于这种癌症的预后尚未明确。
本研究通过 SEER 数据库，确定了 1973 年至 2014 年间被诊断为 PPS 的患者。根据疾病分期和治疗类型对患者进行分层。通过 Kaplan-Meier 分析估计疾病特异性生存率（DSS）和总生存率（OS），并通过单变量和多变量 Cox 回归对队列进行比较。
在所有前列腺癌诊断中，PPS 的发病率为 0.02%。诊断时年龄小于 26 岁的患者占 29%，32%的原发性前列腺肉瘤为横纹肌肉瘤组织学类型。
中位诊断年龄为 9 岁。0-25 岁的横纹肌肉瘤占原发性前列腺肉瘤诊断的 96.4%，25 岁后，横纹肌肉瘤占新诊断病例的 15%。横纹肌肉瘤的 10 年 DSS 和 OS 分别为 47%和 44%。
中位诊断年龄为 71 岁。最常见的诊断为平滑肌肉瘤（33%）和癌肉瘤（28%）。40%、34%和 26%的病例发生局限性、区域性或远处疾病。10 年 DSS 和 OS 分别为 26%和 14%。在局部进展病例中，RT 联合手术治疗趋势上可提高 DSS（P=0.10）。
非横纹肌肉瘤组织学类型的疾病特异性生存率和 OS 似乎低于横纹肌肉瘤。RT 联合手术切除可能会提高局部晚期非横纹肌肉瘤的 DSS。这是对这种罕见泌尿系统恶性肿瘤发病率、分期分布和生存情况的最大报告，提供了有价值的预后信息。
