Stage IV Adult Soft Tissue Sarcoma
Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Nodal Disease
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Stage IV sarcomas are tumors that have metastatic involvement of regional lymph nodes or have spread to distant organs. Soft tissue sarcomas that more commonly spread to lymph nodes include synovial cell sarcomas, epithelioid sarcomas, and rhabdomyosarcomas. For stage IV sarcomas, local control of the primary tumor is probably best obtained by resection with negative margins, lymphadenectomy when appropriate, and postoperative external-beam radiation therapy.[1]
Standard treatment options:
- Surgical resection and lymphadenectomy for patients with clinically positive lymph nodes with or without postoperative radiation to the primary site may be used.
- In some centers, radiation therapy may be used prior to and following surgical extirpation.[2]
- Adjuvant chemotherapy may be considered for patients eligible for clinical trials.[3,4,5,6]
Visceral Disease
With distant metastases, surgery with curative intent is possible for patients with limited pulmonary metastases who are also undergoing or have undergone complete resection of the primary tumor.[7,8,9] The role of adjuvant therapy for pulmonary nodules has been clinically evaluated in trials such as the EORTC-62933 trial.
The value of resection of hepatic metastases is unclear. Doxorubicin alone or with dacarbazine is considered one of the most frequently used chemotherapeutic regimens for advanced sarcoma.[10,11,12] When used as single agents, only doxorubicin and ifosfamide show greater than 20% response rates; less active drugs include dacarbazine, cisplatin, methotrexate, and vinorelbine.[13] 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 versus doxorubicin and dacarbazine alone.[14][Level of evidence: 1iiDiii] For older patients, sequential use of single agents with each recurrence is a better strategy for palliation. High-dose chemotherapy (with or without transplantation) has not influenced disease-free survival or overall survival in published studies so far, but it remains under clinical evaluation for patients with metastatic disease in first complete remission, after resection of pulmonary nodules, or for inoperable large primaries.[15][Level of evidence: 3iiiDiv]
Standard treatment options:
- Surgical resection of the primary tumor with radiation therapy. Resection of pulmonary lesions may be performed following definitive therapy of the primary tumor.[7,8,9]
- Surgical excision with negative tissue margins may be used.
- If the tumor is resectable but wide margins cannot be obtained, radiation therapy may be added.
- If the tumor is unresectable, high-dose radiation therapy may be used, often with chemotherapy.
- For tumors of the retroperitoneum, trunk, and head and neck, surgical resection with preoperative and/or postoperative radiation therapy, and sometimes chemotherapy, may be used.
- For palliation of patients with unresectable visceral disease, chemotherapy with the following agents may be used:
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