Recurrent/Progressive Childhood Soft Tissue Sarcoma

Current Clinical Trials

Decisions about treatment options for recurrent or progressive childhood soft tissue sarcoma are based on many factors, including the following:

• Site of recurrence.
• Tumor biologic characteristics.
• Prior therapies.
• Individual patient considerations.

Standard treatment options for recurrent or progressive disease include the following:

• Surgical excision of local recurrence or isolated pulmonary recurrence.

• Surgical excision of local recurrence followed by radiation therapy or brachytherapy (if no prior radiation therapy was given).

• Limb amputation (only for some children with extremity sarcomas that have already received radiation therapy).

• A clinical trial of new chemotherapeutic regimens.

With the possible exception of infants with congenital fibrosarcoma, the prognosis for patients with recurrent or progressive disease is poor. Resection is the standard treatment for recurrent pediatric nonrhabdomyosarcomatous soft tissue sarcomas. If the patient has not yet received radiation therapy, adjuvant radiation should be considered after local excision of the recurrent tumor. Limb-sparing procedures with adjuvant brachytherapy have been evaluated in adults but have not been studied extensively in children. For some children with extremity sarcomas who have received previous radiation therapy, amputation may be the only therapeutic option. No prospective trial has been able to prove that enhanced local control of pediatric soft tissue sarcomas will ultimately improve survival. Therefore, treatment should be individualized for the site of recurrence and biologic characteristics (e.g., grade, invasiveness, and size) of the tumor. Pulmonary metastasectomy may achieve prolonged disease control for some patients.[1] A large, retrospective analysis of patients with recurrent soft tissue sarcoma showed that isolated local relapse had a better prognosis and that resection of pulmonary metastases improved the probability of survival.[2] All patients with recurrent tumors should be offered enrollment in current drug studies.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood soft tissue sarcoma ¹. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site ².

References

1. Belal A, Salah E, Hajjar W, et al.: Pulmonary metastatectomy for soft tissue sarcomas: is it valuable? J Cardiovasc Surg (Torino) 42 (6): 835-40, 2001.  [PUBMED Abstract]
   
   
2. Zagars GK, Ballo MT, Pisters PW, et al.: Prognostic factors for disease-specific survival after first relapse of soft-tissue sarcoma: analysis of 402 patients with disease relapse after initial conservative surgery and radiotherapy. Int J Radiat Oncol Biol Phys 57 (3): 739-47, 2003.  [PUBMED Abstract]