Voltan Karen, Baptista André Mathias, Etchebehere Maurício
Departamento de Ortopedia e Traumatologia, Faculdade de Ciências Médicas da Universidade Estadual de Campinas, Campinas, SP, Brasil.
Instituto de Ortopedia e Traumatologia, Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.
Rev Bras Ortop (Sao Paulo). 2021 Aug;56(4):419-424. doi: 10.1055/s-0040-1712136. Epub 2020 Sep 25.
Musculoskeletal sarcomas are rare diseases that require attention. They often present high degree of malignancy at diagnosis and, if underestimated, they can evolve aggressively locally and systemically. They present as soft tissue sarcoma and bone sarcomas, with soft tissue being four to five times more common. Most soft tissue sarcomas occur in the extremities. The most common subtypes in children and adolescents are rhabdomyosarcoma and synovial sarcoma; in adults, undifferentiated pleomorphic sarcoma, liposarcoma, leiomyosarcoma, myxofibrosarcoma and synovial sarcoma; all with a high degree of histological malignancy. Many soft tissue sarcomas are confused with benign soft tissue tumors, 100 times more common, so they are resected without the necessary planning, resulting in amputation of a limb that could have been preserved. As in all cancers, the most important prognostic factor is metastatic disease. When it is present, the overall survival rate falls around 20 to 30%. Survival rates are generally similar between bone and soft tissue sarcomas. So soft tissue sarcomas, in addition to being more prevalent, are as aggressive as bone sarcomas, deserving a lot of attention from orthopedic surgeons, who are often the first line of care of carriers of these tumors.
肌肉骨骼肉瘤是需要关注的罕见疾病。它们在诊断时往往呈现出高度恶性，如果被低估，可能会在局部和全身迅速发展。它们表现为软组织肉瘤和骨肉瘤，其中软组织肉瘤更为常见，是骨肉瘤的四到五倍。大多数软组织肉瘤发生在四肢。儿童和青少年中最常见的亚型是横纹肌肉瘤和滑膜肉瘤；在成人中，是未分化多形性肉瘤、脂肪肉瘤、平滑肌肉瘤、黏液纤维肉瘤和滑膜肉瘤；所有这些都具有高度的组织学恶性。许多软组织肉瘤与良性软组织肿瘤混淆，良性软组织肿瘤的发生率要高100倍，因此它们在没有必要规划的情况下就被切除，导致本可保留的肢体被截肢。与所有癌症一样，最重要的预后因素是转移性疾病。当出现转移性疾病时，总体生存率会降至20%至30%左右。骨肉瘤和软组织肉瘤的生存率通常相似。所以软组织肉瘤除了更为普遍外，与骨肉瘤一样具有侵袭性，值得骨科医生给予高度关注，骨科医生往往是这些肿瘤患者的首诊医生。
