Radiotherapy Department, Theagenio Anticancer Hospital of Thessaloniki, Thessaloniki, Greece.
Department of General Surgery, University Hospital of Larisa, Biopolis, 415 00, Larisa, Thessaly, Greece.
Arch Dermatol Res. 2022 Sep;314(7):625-631. doi: 10.1007/s00403-021-02268-1. Epub 2021 Jul 17.
Cutaneous sarcomas are a heterogeneous group of rare mesenchymal neoplasms representing less than 1% of malignant tumors. Histology report remains the cornerstone for the diagnosis of these tumors. The most important clinicopathologic parameters related to prognosis include larger tumor size, high mitotic index, head and neck location, p53 mutations, depth of infiltration and histological grade, vascular and perineural invasion as well as the surgical margins status. Applying advanced biopsy techniques might offer more precise assessment of surgical margins, which constitutes a significant precondition for the management of these tumors. The management of cutaneous soft tissue sarcomas requires a multidisciplinary approach. Surgery remains the standard treatment, nonetheless adjuvant therapy may be required, consisting of radiotherapy, chemotherapy, and molecular targeted therapies to improve treatment outcomes. The role of molecular profiling in the treatment of uncontrolled disease is promising, but it may be offered to a relatively small proportion of patients and its use is still considered experimental in this setting. Due to the rarity of the disease, there is a need for knowledge and experience to be shared, pooled, organized and rationalized so that recent developments in medical science can have a major impact on the disease course. Multicenter clinical trials are needed to improve the care of patients with cutaneous sarcomas.
皮肤肉瘤是一组罕见的间叶性肿瘤,占恶性肿瘤的不到 1%。组织学报告仍然是这些肿瘤诊断的基石。与预后相关的最重要的临床病理参数包括更大的肿瘤大小、高有丝分裂指数、头颈部位置、p53 突变、浸润深度和组织学分级、血管和神经周围侵犯以及手术切缘状态。应用先进的活检技术可能会提供更精确的手术切缘评估,这是这些肿瘤治疗的重要前提。皮肤软组织肉瘤的治疗需要多学科方法。手术仍然是标准治疗,但可能需要辅助治疗,包括放疗、化疗和分子靶向治疗,以改善治疗效果。分子谱分析在治疗不可控疾病中的作用很有前途,但它可能只提供给相对较少的患者,并且在这种情况下,其使用仍被认为是实验性的。由于该疾病的罕见性,需要分享、汇集、组织和合理化知识和经验,以便医学科学的最新发展能对疾病进程产生重大影响。需要开展多中心临床试验,以改善皮肤肉瘤患者的治疗。