Effective treatment of retroperitoneal sarcomas requires removal of all gross disease while sparing adjacent viscera not invaded by tumor. The prognosis for patients with high-grade retroperitoneal sarcomas is less favorable than for patients with tumors at other sites, partly because of the difficulty in completely resecting these tumors and the limitations placed on high-dose radiation therapy.[2,21]
Several prospective randomized trials have been unable to confirm conclusively whether doxorubicin-based adjuvant chemotherapy benefits adults with resectable soft tissue sarcomas. The majority of these studies accrued small numbers of patients and did not demonstrate a metastasis-free survival or an overall survival (OS) benefit for adjuvant chemotherapy. A small study of adjuvant chemotherapy showed a positive impact in disease-free survival and OS in patients treated with postoperative chemotherapy. There was wide interstudy variability among the numerous trials, including differences in therapeutic regimens, drug doses, sample size, tumor site, and histologic grade. A quantitative meta-analysis of updated data from 1,568 individual patients from 14 trials of doxorubicin-based adjuvant therapy showed an absolute benefit from adjuvant therapy of 6% for local relapse-free interval (95% confidence interval [CI], 1-10), 10% for distant relapse-free interval (95% CI, 5-15), and 10% for recurrence-free survival (95% CI, 5-15); however, there was no OS benefit at 10 years.[Level of evidence: 1iiDii] Patients with high-grade tumors (grades 3 or 4) larger than 5 cm in diameter have the greatest tendency for disease to metastasize and are eligible for prospective clinical trials of adjuvant chemotherapy.
With distant metastases (stage IV), surgery with curative intent is possible for patients selected for optimal underlying biologic behavior (i.e., patients with a limited number of metastases, with a long disease-free interval, and with slow clinical growth) with pulmonary metastases who have undergone or are undergoing complete resection of the primary tumor.[24,25,26] Doxorubicin alone or with dacarbazine is considered the best chemotherapeutic regimen for advanced sarcoma.[27,28,29] A randomized trial of 340 patients with advanced sarcoma showed a higher response rate (32% vs. 17%, P < .002) and longer time-to-progression (6 months vs. 4 months, P < .02) for doxorubicin, dacarbazine, ifosfamide, and mesna (MAID) versus doxorubicin and dacarbazine alone.[Level of evidence: 1iiDiii] The increased response rate of the MAID regimen may be justified in preoperative management of younger patients with borderline resectability, but the increased toxic effects argue against its use in older patients.
Complete surgical resection is often difficult for sarcomas of the retroperitoneum because of their large size before detection and anatomic location.[31,32] Prospective randomized trials have not shown improved survival with preoperative or postoperative adjuvant chemotherapy for this subgroup.
Note: Other PDQ summaries containing information about soft tissue sarcoma include:
- Childhood Soft Tissue Sarcoma Treatment
- Ewing Sarcoma Family of Tumors Treatment
- Gastrointestinal Stromal Tumors Treatment
- Kaposi Sarcoma Treatment
- Uterine Sarcoma Treatment
- American Cancer Society.: Cancer Facts and Figures 2011. Atlanta, Ga: American Cancer Society, 2011. Also available online. Last accessed July 27, 2011.
- Brennan MF, Singer S, Maki RG: Sarcomas of the soft tissue and bone. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. Vols. 1 & 2. 8th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2008, pp 1741-1833.
- Le Doussal V, Coindre JM, Leroux A, et al.: Prognostic factors for patients with localized primary malignant fibrous histiocytoma: a multicenter study of 216 patients with multivariate analysis. Cancer 77 (9): 1823-30, 1996.
- Le QT, Fu KK, Kroll S, et al.: Prognostic factors in adult soft-tissue sarcomas of the head and neck. Int J Radiat Oncol Biol Phys 37 (5): 975-84, 1997.
- 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 91 (10): 1914-26, 2001.
- Vraa S, Keller J, Nielsen OS, et al.: Prognostic factors in soft tissue sarcomas: the Aarhus experience. Eur J Cancer 34 (12): 1876-82, 1998.
- Collin CF, Friedrich C, Godbold J, et al.: Prognostic factors for local recurrence and survival in patients with localized extremity soft-tissue sarcoma. Semin Surg Oncol 4 (1): 30-7, 1988.
- Watson DI, Coventry BJ, Langlois SL, et al.: Soft-tissue sarcoma of the extremity. Experience with limb-sparing surgery. Med J Aust 160 (7): 412-6, 1994.
- Geer RJ, Woodruff J, Casper ES, et al.: Management of small soft-tissue sarcoma of the extremity in adults. Arch Surg 127 (11): 1285-9, 1992.
- Lohman RF, Nabawi AS, Reece GP, et al.: Soft tissue sarcoma of the upper extremity: a 5-year experience at two institutions emphasizing the role of soft tissue flap reconstruction. Cancer 94 (8): 2256-64, 2002.
- Fish FS: Soft tissue sarcomas in dermatology. Dermatol Surg 22 (3): 268-73, 1996.
- Marcove RC, Sheth DS, Healey J, et al.: Limb-sparing surgery for extremity sarcoma. Cancer Invest 12 (5): 497-504, 1994.
- Williard WC, Collin C, Casper ES, et al.: The changing role of amputation for soft tissue sarcoma of the extremity in adults. Surg Gynecol Obstet 175 (5): 389-96, 1992.
- Yang JC, Chang AE, Baker AR, et al.: Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol 16 (1): 197-203, 1998.
- Schmidt RA, Conrad EU 3rd, Collins C, et al.: Measurement and prediction of the short-term response of soft tissue sarcomas to chemotherapy. Cancer 72 (9): 2593-601, 1993.
- Temple WJ, Temple CL, Arthur K, et al.: Prospective cohort study of neoadjuvant treatment in conservative surgery of soft tissue sarcomas. Ann Surg Oncol 4 (7): 586-90, 1997 Oct-Nov.
- Valle AA, Kraybill WG: Management of soft tissue sarcomas of the extremity in adults. J Surg Oncol 63 (4): 271-9, 1996.
- Pollack A, Zagars GK, Goswitz MS, et al.: Preoperative vs. postoperative radiotherapy in the treatment of soft tissue sarcomas: a matter of presentation. Int J Radiat Oncol Biol Phys 42 (3): 563-72, 1998.
- Schoenfeld GS, Morris CG, Scarborough MT, et al.: Adjuvant radiotherapy in the management of soft tissue sarcoma involving the distal extremities. Am J Clin Oncol 29 (1): 62-5, 2006.
- O'Byrne K, Steward WP: The role of adjuvant chemotherapy in the treatment of adult soft tissue sarcomas. Crit Rev Oncol Hematol 27 (3): 221-7, 1998.
- Lewis JJ, Leung D, Woodruff JM, et al.: Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg 228 (3): 355-65, 1998.
- Frustaci S, Gherlinzoni F, De Paoli A, et al.: Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 19 (5): 1238-47, 2001.
- Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-analysis Collaboration. Lancet 350 (9092): 1647-54, 1997.
- van Geel AN, Pastorino U, Jauch KW, et al.: Surgical treatment of lung metastases: The European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group study of 255 patients. Cancer 77 (4): 675-82, 1996.
- Casson AG, Putnam JB, Natarajan G, et al.: Five-year survival after pulmonary metastasectomy for adult soft tissue sarcoma. Cancer 69 (3): 662-8, 1992.
- Putnam JB Jr, Roth JA: Surgical treatment for pulmonary metastases from sarcoma. Hematol Oncol Clin North Am 9 (4): 869-87, 1995.
- Santoro A, Tursz T, Mouridsen H, et al.: Doxorubicin versus CYVADIC versus doxorubicin plus ifosfamide in first-line treatment of advanced soft tissue sarcomas: a randomized study of the European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group. J Clin Oncol 13 (7): 1537-45, 1995.
- Zalupski M, Metch B, Balcerzak S, et al.: Phase III comparison of doxorubicin and dacarbazine given by bolus versus infusion in patients with soft-tissue sarcomas: a Southwest Oncology Group study. J Natl Cancer Inst 83 (13): 926-32, 1991.
- Borden EC, Amato DA, Rosenbaum C, et al.: Randomized comparison of three adriamycin regimens for metastatic soft tissue sarcomas. J Clin Oncol 5 (6): 840-50, 1987.
- Antman K, Crowley J, Balcerzak SP, et al.: An intergroup phase III randomized study of doxorubicin and dacarbazine with or without ifosfamide and mesna in advanced soft tissue and bone sarcomas. J Clin Oncol 11 (7): 1276-85, 1993.
- Heslin MJ, Lewis JJ, Nadler E, et al.: Prognostic factors associated with long-term survival for retroperitoneal sarcoma: implications for management. J Clin Oncol 15 (8): 2832-9, 1997.
- Jaques DP, Coit DG, Hajdu SI, et al.: Management of primary and recurrent soft-tissue sarcoma of the retroperitoneum. Ann Surg 212 (1): 51-9, 1990.