Clear cell sarcoma of tendons and aponeuroses in pediatric patients
A report from the Italian and German Soft Tissue Sarcoma Cooperative Group
Version of Record online: 12 JUN 2002
Copyright © 2002 American Cancer Society
Volume 94, Issue 12, pages 3269–3276, 15 June 2002
How to Cite
Ferrari, A., Casanova, M., Bisogno, G., Mattke, A., Meazza, C., Gandola, L., Sotti, G., Cecchetto, G., Harms, D., Koscielniak, E., Treuner, J. and Carli, M. (2002), Clear cell sarcoma of tendons and aponeuroses in pediatric patients. Cancer, 94: 3269–3276. doi: 10.1002/cncr.10597
- Issue online: 12 JUN 2002
- Version of Record online: 12 JUN 2002
- Manuscript Accepted: 24 JAN 2002
- Manuscript Revised: 18 DEC 2001
- Manuscript Received: 12 SEP 2001
- Fondazione Città della Speranza
- clear cell sarcoma;
- malignant melanoma of soft part;
- pediatric tumors;
- soft tissue sarcomas
Clear cell sarcoma (CCS) of tendons and aponeuroses is extremely rare in childhood and little information is available on its clinical management. Originally believed to be a type of melanoma of soft tissue origin, CCS is now considered a distinct clinicopathologic entity that behaves like a high-grade soft tissue sarcoma. We report on a series of 28 pediatric patients treated from 1980 to 2000 by the Soft Tissue Sarcoma Italian Cooperative Group and the German Cooperative Group.
Patients were treated with a multimodality therapeutic approach. Surgical resection was complete in 17 patients (mutilating in 3), radiotherapy was administered to 8 patients, and 20 patients received chemotherapy.
After a median follow-up of 102 months (range, 19–238 months), the 5-year and event-free survival rates were 66.4% and 63.3%, respectively. Seventeen patients were alive in first remission, two were alive in second remission, and nine had died of disease. The response to chemotherapy in the 7 evaluable patients included one partial remission, one minor response, and five no responses. Radiotherapy contributed to achieving local control in four of six Intergroup Rhabdomyosarcoma Study (IRS) Group II patients. Statistically significant differences in outcome were evident according to IRS group, tumor size, and site.
Our study confirms the aggressive behavior of CCS. Complete surgical resection represents the mainstay of treatment, and even the only treatment for patients with small tumors. Radiotherapy may control microscopic residual disease after surgery. Chemotherapy is ineffective and the prognosis is unfavorable for patients with unresectable and large tumors. Cancer 2002;94:3269–76. © 2002 American Cancer Society.