Hayes A J, Thomas J M
Sarcoma Unit, Royal Marsden Hospital, London, UK.
Postgrad Med J. 1997 Nov;73(865):705-9. doi: 10.1136/pgmj.73.865.705.
Any soft tissue swelling beneath the deep fascia should be considered a sarcoma until proven otherwise. As the most important factor in the primary treatment of these cancers is the adequacy of the primary surgical resection, it is vital to diagnose these malignant tumours pre-operatively. The modern treatment of soft tissue sarcomas may involve all modalities, but the most important aspect of treatment of a primary localised sarcoma is wide excisional surgery preserving limb function. Radiotherapy is a vital adjunct in high-grade tumours, or in tumours whose resectability is limited either by size or anatomical proximity to vital structures. Apart from a few chemosensitive sarcomas, the role of chemotherapy is limited to treatment of metastatic disease where documented response rates are no greater than 30%. As 50% of patients with high-grade sarcomas will die from metastatic disease, improvements in survival rates will only come from improvements in response to systemic therapy. No controlled trials have shown any survival benefit for adjuvant chemotherapy, although a recent meta-analysis of published data has shown a trend to increased survival at two years. Multicentre randomised trials are ongoing. The prognosis of these lesions is highly variable, but is intimately related to the anatomical site (i.e., resectability), and also the grade and size of the tumour.
深筋膜下的任何软组织肿胀在未被证伪前均应被视为肉瘤。由于这些癌症主要治疗中最重要的因素是初次手术切除的充分性，因此术前诊断这些恶性肿瘤至关重要。软组织肉瘤的现代治疗可能涉及所有治疗方式，但原发性局限性肉瘤治疗的最重要方面是在保留肢体功能的情况下进行广泛切除手术。放射治疗是高级别肿瘤或因大小或与重要结构的解剖位置关系而导致可切除性受限的肿瘤的重要辅助治疗手段。除了少数对化疗敏感的肉瘤外，化疗的作用仅限于治疗转移性疾病，其记录的缓解率不超过30%。由于50%的高级别肉瘤患者将死于转移性疾病，生存率的提高仅来自全身治疗反应的改善。尽管最近对已发表数据的荟萃分析显示两年生存率有上升趋势，但尚无对照试验表明辅助化疗有任何生存获益。多中心随机试验正在进行中。这些病变的预后差异很大，但与解剖部位（即可切除性）以及肿瘤的分级和大小密切相关。
