SYMPOSIUM ON SOLID TUMORSSarcoma
Section snippets
HISTORY
Sarcomas have played an important role in the understanding of the nature of cancer. In 1909, Rous13 was given a Plymouth Rock hen bearing a spindle cell sarcoma. Rous found that he could transfer the tumor from one chicken to another, but not to all types of chickens. In his studies of the nature of the transmissible agent, Rous found that extracts of tumors that passed through a Berkefeld filter (which did not allow passage of particles of the size of known bacteria) could also transfer the
CYTOGENETIC CHANGES
Cytogenetic changes are common in sarcomas15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28 (Table 1) and can be divided into 2 broad categories. One group has specific characteristic cytogenetic changes and relatively simple karyotypes, such as a fusion gene or point mutation. The other group has nonspecific changes, often with very complex karyotypes.16, 29, 30, 31 In several cases, the observed genetic changes have been exploited as targets of therapy. A better understanding of the
ETIOLOGIC AGENTS AND RISK FACTORS
Although most sarcomas arise spontaneously, some risk factors have been identified. Exposure to ionizing radiation increases the incidence of sarcomas, typically more than 7 to 10 years after exposure, most commonly in patients treated with radiation therapy for breast and cervical cancer as well as lymphoma.32, 33 Patients treated with ionizing radiation for cancer have developed both osteosarcoma and STS, including angiosarcoma.34, 35, 36, 37, 38 Other risk factors include chronic lymphedema,
SOFT TISSUE SARCOMAS
Soft tissue sarcomas, which represent fewer than 1% of malignancies, may arise in skin or other organs as well as soft tissue. Because the Surveillance, Epidemiology, and End Results data on STS only include those arising in soft tissue, they underestimate their true incidence.54 For example, the 1993 US national estimate increases from approximately 6000 STS cases per year to approximately 11,400 cases after the inclusion of STSs that originated in organs.54, 55 Similarly, GIST, which was
PRIMARY SARCOMAS OF BONE
The most common primary bone tumors are osteosarcoma, EWS, and MFH of bone. Management of primary sarcomas of bone has improved dramatically in the past 3 decades. Most patients are able to undergo limb-sparing procedures, and survival has improved dramatically.
SURGICAL RESECTION OF METASTASES
Given the heterogeneity in the biological behavior of sarcoma, resection of metastatic disease, which often occurs with osteosarcoma but may occur with any sarcoma, is a reasonable option. The most commonly resected metastases are those of the lung. Factors that are weighed when deciding whether to resect metastases include the length of the disease-free interval, the number of metastases, and the growth rate of the metastases. Clearly, a short disease-free interval, the presence of a large
FUTURE DEVELOPMENTS
The development of optimal treatment strategies for sarcoma has been greatly complicated by the large number of subtypes, the heterogeneity in their biological behavior, and the small number of patients with particular subtypes enrolled in trials. Molecular techniques such as microarray-based gene expression profiles promise to improve our ability to predict both the probability of metastasis and overall clinical course and the probability of response to a particular treatment.7, 8, 80 Recent
CONCLUSION
Sarcomas comprise a heterogeneous group of neoplasms that can be grouped into 2 general categories, STSs and primary bone sarcomas, each with different staging and treatment approaches. The approach to a patient with a sarcoma begins with a biopsy that obtains adequate tissue for diagnosis without interfering with subsequent optimal definitive surgery. Subsequent treatment depends on the specific type of sarcoma. Because sarcomas are relatively uncommon and yet comprise a wide variety of
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Dr Skubitz has received grant support from Amgen, Bristol Myers, Cell Therapeutics, Johnson & Johnson, and Pfizer; is on the speakers' bureaus of Johnson & Johnson, Novartis, and Pfizer; owns publicly traded stock in Genentech and Johnson & Johnson; and has consulted for Amgen, Johnson & Johnson, Keryx, Novartis, and OSI. Dr D'Adamo is on the speakers' bureaus of Bayer, Novartis, and Pfizer.