Lessnick Stephen L, Dei Tos A Paolo, Sorensen Poul H B, Dileo Palma, Baker Laurence H, Ferrari Stefano, Hall Kirsten Sundby
Huntsman Cancer Institute, Salt Lake City, UT, USA.
Semin Oncol. 2009 Aug;36(4):338-46. doi: 10.1053/j.seminoncol.2009.06.006.
Small round cell sarcomas are the most aggressive of the tumors morphologically and clinically encountered in children and adults, and in some ways the most leukemia- or lymphoma-like of sarcomas. Small round cell sarcomas often are associated with chromosomal translocations, like hematologic malignancies, and are usually more sensitive to chemotherapy than other sarcoma subtypes. They have a high risk of mortality, but chemotherapy (in addition to surgery and often radiation therapy) provides a good cure rate, although treatment is often long and intensive. The biology of these tumors is very telling in terms of some of the mechanisms of cancer cell survival and proliferation. Although there is some overlap of the discussion below with the section on translocation associated sarcomas, we have highlighted some of the key issues with these sarcomas below, with some ideas that may bear fruit both in terms of the management of these, other sarcomas, and other cancers alike.
小圆细胞肉瘤是儿童和成人中在形态学和临床上遇到的最具侵袭性的肿瘤，在某些方面也是最像白血病或淋巴瘤的肉瘤。小圆细胞肉瘤常与染色体易位有关，类似于血液系统恶性肿瘤，并且通常比其他肉瘤亚型对化疗更敏感。它们具有很高的死亡率，但化疗（除手术外，通常还包括放射治疗）可提供较高的治愈率，尽管治疗通常时间长且强度大。就癌细胞存活和增殖的一些机制而言，这些肿瘤的生物学特性非常能说明问题。尽管下面的讨论与易位相关肉瘤部分有一些重叠，但我们在下面突出了这些肉瘤的一些关键问题，以及一些可能在这些肉瘤、其他肉瘤和其他癌症的治疗方面都有成效的想法。
