Castle Jennifer T, Levy Brittany E, Allison Derek B, Rodeberg David A, Rellinger Eric J
Department of Surgery, Markey Cancer Center, University of Kentucky, Lexington, KY 40536, USA.
Department of Surgery, University of Kentucky, Lexington, KY 40536, USA.
Cancers (Basel). 2023 May 22;15(10):2864. doi: 10.3390/cancers15102864.
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric and adolescent population, with 350 new cases diagnosed each year. While they can develop anywhere in the body, the genitourinary tract is the second most common primary location for an RMS to develop. Overall survival has improved through the increased use of protocols and multidisciplinary approaches. However, the guidelines for management continue to change as systemic and radiation therapeutics advance. Given the relative rarity of this disease compared to other non-solid childhood malignancies, healthcare providers not directly managing RMS may not be familiar with their presentation and updated management. This review aims to provide foundational knowledge of the management of RMSs with an emphasis on specific management paradigms for those arising from the genitourinary tract. The genitourinary tract is the second most common location for an RMS to develop but varies greatly in symptomology and survival depending on the organ of origin. As the clinical understanding of these tumors advances, treatment paradigms have evolved. Herein, we describe the breadth of presentations for genitourinary RMSs with diagnostic and treatment management considerations, incorporating the most recently available guidelines and societal consensus recommendations.
横纹肌肉瘤（RMS）是儿童和青少年中最常见的软组织肉瘤，每年有350例新确诊病例。虽然它可在身体任何部位发生，但泌尿生殖道是RMS发生的第二大常见原发部位。通过增加方案的使用和多学科方法，总体生存率有所提高。然而，随着全身治疗和放射治疗的进展，管理指南仍在不断变化。鉴于与其他非实体儿童恶性肿瘤相比，这种疾病相对罕见，未直接管理RMS的医疗服务提供者可能不熟悉其表现和最新管理方法。本综述旨在提供RMS管理的基础知识，重点关注泌尿生殖道来源的RMS的特定管理模式。泌尿生殖道是RMS发生的第二大常见部位，但症状和生存率因起源器官而异。随着对这些肿瘤临床认识的提高，治疗模式也在不断演变。在此，我们描述了泌尿生殖道RMS的广泛表现，并考虑了诊断和治疗管理，纳入了最新的指南和社会共识建议。
