“Nature often gives us hints to her profoundest secrets, and it is possible that she has given us a hint which, if we will but follow, may lead us on to the solution of this difficult problem.” – William B. Coley in “Contribution to the knowledge of sarcoma,” 1891.
Although soft tissue sarcomas (STS) are rare, accounting for only 1% of solid tumors in adults, surgical oncologists have played a unique role performing many of the pivotal clinical trials and studies that have defined the current management and biologic behavior of these malignancies. As surgery has remained the cornerstone of treatment in patients with STS, surgical oncologists have been responsible for developing and directing their multimodality care. STS arise from or within mesenchymal tissue (fat, nerves, muscle, etc.). While early mention of possible sarcoma of nerve origin occurred in Anatomy of the Human Body by William Cheselden published in 1740,1 sarcomas were not distinguished from other cancers until the early 19th century, when Richard Virchow published his work Die krankhaften Geschwülste in 1863.2 Further pathologic classification did not occur until 1948 when Arthur Purdy Stout at Columbia University published his histogenic classification of STS subtypes.3 This allowed recognition of the differences in biologic behavior between sarcoma subtypes, however pathologic misinterpretations led to the inability to reliably diagnose sarcoma subtypes, especially when dedifferentiated or high-grade.4 Therefore, the Task Force on Soft Tissue Sarcoma of the American Joint Committee for Cancer Staging and End Results Reporting was convened, composed of pathologists and surgeons, and formulated a standardized TNM classification system in 1977 stemming from the analysis of 1215 sarcoma patient records.5 The importance of tumor grade in determining patient stage and prognosis led to the addition of grade (G) as a unique qualifier in the staging system, not folded within the tumor (T) designation (Fig. 26-1).5
Survival curves by stage for 702 cases. (Reproduced with permission from Russell WO, Cohen J, Enzinger F, et al. A clinical and pathological staging system for soft tissue sarcomas. Cancer. October 1977;40(4):1562–1570.)
Historically, treatment for STS centered solely on surgical resection. Various adjuvant therapies were attempted, with only rare reports of success. Bloodroot, a common flowering plant in North America that produces benzylisoquinoline alkaloids, was reported as a treatment for STS in the New England Journal of Medicine and Surgery in 1819.6 However, it was not until after 1963, with the FDA approval of vincristine, that alkaloid agents were formally used in the treatment of sarcoma.7 In 1891, William B. Coley published his series of nine patients, mainly with head and neck STS, that were inoculated with streptococcus erysipelas and experienced regression of disease.8 Coley went on to inoculate more than 1000 cancer patients with mainly bone ...