Metastatic Childhood Soft Tissue Sarcoma
Standard Treatment Options
Treatment Options Under Clinical Evaluation
Current Clinical Trials
The prognosis for children with metastatic soft tissue sarcomas is poor,[1-6]
and these children should receive combined treatment with chemotherapy,
radiation therapy, and surgical resection of pulmonary metastases. In a
prospective randomized trial, chemotherapy with vincristine, dactinomycin,
doxorubicin, and cyclophosphamide with or without dacarbazine led to tumor
responses in one-third of patients with unresectable or metastatic disease.
The estimated 4-year survival rate, however, was poor, with fewer than one-third
of the children surviving.[6-8]
Standard Treatment Options
Children with isolated pulmonary metastases should undergo exploratory
thoracotomy in an attempt to resect all gross disease. The estimated 5-year
survival rate after thoracotomy for pulmonary metastasectomy has ranged from
10% to 58% in adult studies. Formal segmentectomy, lobectomy, and mediastinal
lymph node dissection are unnecessary.[9]
Treatment Options Under Clinical Evaluation
Vincristine, doxorubicin, and ifosfamide with granulocyte colony-stimulating
factor have been used in patients with unresected or metastatic tumors. The Pediatric
Oncology Group evaluated the combination of doxorubicin and
ifosfamide in children with unresected or metastatic soft tissue sarcomas
because several adult trials have suggested that ifosfamide-based regimens may
be superior to other chemotherapeutic regimens for soft tissue sarcomas.[10]
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic childhood soft tissue sarcoma. 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.
References
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Demetri GD, Elias AD: Results of single-agent and combination chemotherapy for advanced soft tissue sarcomas. Implications for decision making in the clinic. Hematol Oncol Clin North Am 9 (4): 765-85, 1995.
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Elias A, Ryan L, Sulkes A, et al.: Response to mesna, doxorubicin, ifosfamide, and dacarbazine in 108 patients with metastatic or unresectable sarcoma and no prior chemotherapy. J Clin Oncol 7 (9): 1208-16, 1989.
[PUBMED Abstract]
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Edmonson JH, Ryan LM, Blum RH, et al.: Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas. J Clin Oncol 11 (7): 1269-75, 1993.
[PUBMED Abstract]
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Rao BN: Nonrhabdomyosarcoma in children: prognostic factors influencing survival. Semin Surg Oncol 9 (6): 524-31, 1993 Nov-Dec.
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deCou JM, Rao BN, Parham DM, et al.: Malignant peripheral nerve sheath tumors: the St. Jude Children's Research Hospital experience. Ann Surg Oncol 2 (6): 524-9, 1995.
[PUBMED Abstract]
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Pappo AS, Rao BN, Jenkins JJ, et al.: Metastatic nonrhabdomyosarcomatous soft-tissue sarcomas in children and adolescents: the St. Jude Children's Research Hospital experience. Med Pediatr Oncol 33 (2): 76-82, 1999.
[PUBMED Abstract]
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Pratt CB, Pappo AS, Gieser P, et al.: Role of adjuvant chemotherapy in the treatment of surgically resected pediatric nonrhabdomyosarcomatous soft tissue sarcomas: A Pediatric Oncology Group Study. J Clin Oncol 17 (4): 1219, 1999.
[PUBMED Abstract]
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Pratt CB, Maurer HM, Gieser P, et al.: Treatment of unresectable or metastatic pediatric soft tissue sarcomas with surgery, irradiation, and chemotherapy: a Pediatric Oncology Group study. Med Pediatr Oncol 30 (4): 201-9, 1998.
[PUBMED Abstract]
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Putnam JB Jr, Roth JA: Surgical treatment for pulmonary metastases from sarcoma. Hematol Oncol Clin North Am 9 (4): 869-87, 1995.
[PUBMED Abstract]
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Pappo AS, Devidas M, Jenkins J, et al.: Phase II trial of neoadjuvant vincristine, ifosfamide, and doxorubicin with granulocyte colony-stimulating factor support in children and adolescents with advanced-stage nonrhabdomyosarcomatous soft tissue sarcomas: a Pediatric Oncology Group Study. J Clin Oncol 23 (18): 4031-8, 2005.
[PUBMED Abstract]
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