Weiss Aaron R, Ferrari Andrea, Mascarenhas Leo, Bisogno Gianni
Department of Pediatrics, MaineHealth Maine Medical Center, Portland, ME, USA.
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Via G. Venezian, 1 20133 Milano, MI, Italy; Department of Oncology and Hematology-oncology, University of Milan, Milan, Italy.
Hematol Oncol Clin North Am. 2025 Aug;39(4):727-748. doi: 10.1016/j.hoc.2025.04.004. Epub 2025 May 14.
Soft tissue sarcomas (STS) are rare mesenchymal tumors representing up to 7% of all cancers in children. In the pediatric population, rhabdomyosarcoma (RMS) is the most common histology while the remainder is composed of several distinct histotypes collectively known as nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). RMS and NRSTS have individualized staging and treatment paradigms. Through cooperative group clinical trials, our understanding of STS and outcomes have improved. However, patients with distant metastases and who relapse continue to fare poorly. Global collaborations are necessary to make greater progress for this highest risk population.
软组织肉瘤（STS）是罕见的间充质肿瘤，占儿童所有癌症的7%。在儿科人群中，横纹肌肉瘤（RMS）是最常见的组织学类型，其余则由几种不同的组织学类型组成，统称为非横纹肌肉瘤软组织肉瘤（NRSTS）。RMS和NRSTS有各自的分期和治疗模式。通过合作组临床试验，我们对STS的认识和治疗结果有所改善。然而，有远处转移和复发的患者预后仍然很差。为了使这一高危人群取得更大进展，全球合作是必要的。
