肉瘤是一组罕见的结缔组织实体瘤。 目前已知的就有 50 多种不同的组织学亚型。
最常见的情况是表现为软组织肿胀,可能伴随或不伴随疼痛。应考虑的鉴别诊断有淋巴瘤、转移癌和良性病变(例如,脂肪瘤和神经瘤)。
有些肉瘤与特定的基因突变相关,尤其是染色体易位。
通过活检进行诊断,活检应采集足够数量的组织以进行准确的病理诊断。
病理结果应由在肉瘤组织学方面有一定经验的病理学专家进行复核。 实验室诊断方法可能包括免疫组织化学、细胞遗传学和分子基因检测。
治疗基于肿瘤分期和组织学亚型,只有部分亚型对化疗敏感。
肉瘤是一组起源于间质(结缔组织)细胞的罕见实体瘤,占所有成人恶性肿瘤的 1%。 它们可分为 2 大类:软组织肉瘤(包括脂肪、肌肉、神经及神经鞘、血管以及其他结缔组织)与骨肉瘤。[1]Pisters PW, Casper ES, Mann GN. Soft-tissue sarcomas. In: Pazdur R, Coia LR, Hoskins WJ, et al, eds. Cancer management: a multidisciplinary approach. 9th ed. Melville, NY: PRR Inc.; 2005:585-614.[2]Cormier JN, Pollock RE. Soft tissue sarcomas. CA Cancer J Clin. 2004 Mar-Apr;54(2):94-109.
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[3]Jemal A, Siegel R, Xu J, et al. Cancer statistics, 2010. CA Cancer J Clin. 2010 Sep-Oct;60(5):277-300.
http://onlinelibrary.wiley.com/doi/10.3322/caac.20073/full
http://www.ncbi.nlm.nih.gov/pubmed/20610543?tool=bestpractice.com
[4]Pisters P. Staging and prognosis. In: Pollock RE, ed. American Cancer Society atlas of clinical oncology: soft tissue sarcomas. Hamilton, Ontario: BC Decker; 2002:80-8.[5]Clark MA, Fisher C, Judson I, et al. Soft tissue sarcomas in adults. N Engl J Med. 2005 Aug 18;353(7):701-11.
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[6]Coindre JM, Terrier P, Guillou L, et al. Predictive value of grade for metastasis development in the main histologic types of adult soft tissue sarcomas: a study of 1240 patients from the French Federation of Cancer Centers Sarcoma Group. Cancer. 2001 May 15;91(10):1914-26.
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[7]Dileo P, Demetri GD. Update on new diagnostic and therapeutic approaches for sarcomas. Clin Adv Hematol Oncol. 2005 Oct;3(10):781-91.
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[8]Borden EC, Baker LH, Bell RS, et al. Soft tissue sarcomas of adults: state of the translational science. Clin Cancer Res. 2003 Jun;9(6):1941-56.
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http://www.ncbi.nlm.nih.gov/pubmed/12796356?tool=bestpractice.com
目前已知的就有 50 多个不同的软组织肉瘤组织学亚型。[9]Fletcher CD, Gustafson P, Rydholm A, et al. Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification. J Clin Oncol. 2001 Jun 15;19(12):3045-50.
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[10]Fletcher CD, Bridge JA, Hogendoorn PCW, et al., eds. WHO/IARC classification of tumours: World Health Organization classification of tumours of soft tissue and bone. 4th ed (Vol 5). Lyon, France: IARC Press; 2013.