Clear cell sarcoma of tendons and aponeuroses in pediatric patients: A report from the Italian and German Soft Tissue Sarcoma Cooperative Group

Andrea Ferrari, Michela Casanova, Gianni Bisogno, Adrian Mattke, Cristina Meazza, Lorenza Gandola, Guido Sotti, Giovanni Cecchetto, Dieter Harms, Eva Koscielniak, Jorn Treuner, Modesto Carli

Research output: Contribution to journalArticle

68 Citations (Scopus)

Abstract

BACKGROUND. 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. METHODS. 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. RESULTS. 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. CONCLUSIONS. 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.

Original languageEnglish
Pages (from-to)3269-3276
Number of pages8
JournalCancer
Volume94
Issue number12
DOIs
Publication statusPublished - Jun 15 2002

Fingerprint

Clear Cell Sarcoma
Sarcoma
Tendons
Pediatrics
Radiotherapy
Rhabdomyosarcoma
Drug Therapy
Aponeurosis
Neoplasms
Disease-Free Survival
Melanoma
Therapeutics
Survival Rate

Keywords

  • Clear cell sarcoma
  • Malignant melanoma of soft part
  • Pediatric tumors
  • Soft tissue sarcomas

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Clear cell sarcoma of tendons and aponeuroses in pediatric patients : A report from the Italian and German Soft Tissue Sarcoma Cooperative Group. / Ferrari, Andrea; Casanova, Michela; Bisogno, Gianni; Mattke, Adrian; Meazza, Cristina; Gandola, Lorenza; Sotti, Guido; Cecchetto, Giovanni; Harms, Dieter; Koscielniak, Eva; Treuner, Jorn; Carli, Modesto.

In: Cancer, Vol. 94, No. 12, 15.06.2002, p. 3269-3276.

Research output: Contribution to journalArticle

Ferrari, Andrea ; Casanova, Michela ; Bisogno, Gianni ; Mattke, Adrian ; Meazza, Cristina ; Gandola, Lorenza ; Sotti, Guido ; Cecchetto, Giovanni ; Harms, Dieter ; Koscielniak, Eva ; Treuner, Jorn ; Carli, Modesto. / Clear cell sarcoma of tendons and aponeuroses in pediatric patients : A report from the Italian and German Soft Tissue Sarcoma Cooperative Group. In: Cancer. 2002 ; Vol. 94, No. 12. pp. 3269-3276.
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abstract = "BACKGROUND. 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. METHODS. 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. RESULTS. 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. CONCLUSIONS. 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.",
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T1 - Clear cell sarcoma of tendons and aponeuroses in pediatric patients

T2 - A report from the Italian and German Soft Tissue Sarcoma Cooperative Group

AU - Ferrari, Andrea

AU - Casanova, Michela

AU - Bisogno, Gianni

AU - Mattke, Adrian

AU - Meazza, Cristina

AU - Gandola, Lorenza

AU - Sotti, Guido

AU - Cecchetto, Giovanni

AU - Harms, Dieter

AU - Koscielniak, Eva

AU - Treuner, Jorn

AU - Carli, Modesto

PY - 2002/6/15

Y1 - 2002/6/15

N2 - BACKGROUND. 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. METHODS. 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. RESULTS. 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. CONCLUSIONS. 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.

AB - BACKGROUND. 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. METHODS. 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. RESULTS. 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. CONCLUSIONS. 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.

KW - Clear cell sarcoma

KW - Malignant melanoma of soft part

KW - Pediatric tumors

KW - Soft tissue sarcomas

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