With the possible exception of infants with infantile fibrosarcoma, the prognosis for patients with recurrent or progressive disease is poor. No prospective trial has been able to prove that enhanced local control of pediatric soft tissue sarcomas (STSs) 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.
Decisions about treatment options for progressive or recurrent childhood STS are based on many factors, including the following:
Cancell is a trademarked name of a liquid mixture long promoted as a treatment for people with cancer and other diseases (see Question 1).
The U.S. Food and Drug Administration (FDA) has listed the ingredients of Cancell as the chemicals inositol, nitric acid, sodium sulfite, potassium hydroxide, sulfuric acid, and catechol (see Question 3).
Cantron and Protocel are other products that are said to be similar to Cancell.
None of the common chemicals in these products is known to be effective...
A phase I trial of pazopanib reported one partial response in a patient with desmoplastic small round cell tumor and prolonged disease stabilization in eight patients with recurrent sarcoma.[Level of evidence: 2Diii] Pazopanib has been approved for use in recurrent soft tissue sarcoma. The clinical trial that was used to obtain approval was limited to adults and demonstrated disease stabilization and prolonged time to progression; it did not demonstrate improved overall survival.
A clinical trial of new chemotherapeutic regimens.
Resection is the standard treatment for recurrent pediatric nonrhabdomyosarcomatous STSs. 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.
Pulmonary metastasectomy may achieve prolonged disease control for some patients. A large, retrospective analysis of patients with recurrent STS showed that isolated local relapse had a better prognosis and that resection of pulmonary metastases improved the probability of survival. In 31 children and adolescents younger than 23 years with pulmonary metastases from synovial sarcoma, complete resection of lung metastases appeared to prolong survival when compared with ten other patients who were not considered candidates for metastasectomy.[Level of evidence: 3iiiA] All patients with recurrent tumors should be considered for current clinical trials.