Synovial sarcoma in children and adolescents: Thirty three years of experience with multimodal therapy

M. Fatih Okcu, Simona Despa, Mary Choroszy, Su G. Berrak, Ayten Cangir, Norman Jaffe, R. Beverly Raney

Research output: Contribution to journalArticle

30 Citations (Scopus)

Abstract

Background. Synovial sarcoma (SS) is the most common type of non-rhabdomyosarcoma soft tissue sarcoma in childhood, with controversies about its prognosis and treatment. Procedure. We reviewed medical records of 42 children and adolescents with SS treated at our institution between 1966 and 1999 to determine treatment results and assess prognostic factors. Results. With a median follow-up duration of 7.8 years (range 0.2-22.4 years), 5-year progression free survival (PFS) and overall survival (OS) rates were 75.6% (95% Confidence Interval [Cl] 62-89.2%) and 87.7% (95% Cl 77.3-98.1%) respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. IRS grouping and tumor invasiveness were found to be significant prognostic indicators (P < 0.01 and = 0.02, respectively). Patients with initial gross total resection (IRS I and II) and non-invasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (<5 cm) (P = 0.09) or with monophasic histology (P = 0.14) had better PFS and OS. Conclusions. Achieving a complete resection or gross total resection with microscopic residual disease is vital for survival of patients with localized SS. Patients with localized disease who received radiotherapy had improved local control. Chemotherapy did not seem to impact PFS or OS. Future large multi-institutional trials are needed to address whether post-operative chemotherapy is necessary for patients with localized, surgically removed tumors, whether radiotherapy is necessary for patients with completely resected tumors, and to ascertain the order of importance of all the candidate prognostic markers.

Original languageEnglish (US)
Pages (from-to)90-96
Number of pages7
JournalMedical and Pediatric Oncology
Volume37
Issue number2
DOIs
StatePublished - 2001
Externally publishedYes

Fingerprint

Synovial Sarcoma
Disease-Free Survival
Survival
Neoplasms
Therapeutics
Radiotherapy
Drug Therapy
Sarcoma
Medical Records
Histology
Survival Rate
Confidence Intervals

Keywords

  • Childhood cancer
  • Soft tissue sarcoma
  • Synovial sarcoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Cancer Research

Cite this

Fatih Okcu, M., Despa, S., Choroszy, M., Berrak, S. G., Cangir, A., Jaffe, N., & Beverly Raney, R. (2001). Synovial sarcoma in children and adolescents: Thirty three years of experience with multimodal therapy. Medical and Pediatric Oncology, 37(2), 90-96. https://doi.org/10.1002/mpo.1175

Synovial sarcoma in children and adolescents : Thirty three years of experience with multimodal therapy. / Fatih Okcu, M.; Despa, Simona; Choroszy, Mary; Berrak, Su G.; Cangir, Ayten; Jaffe, Norman; Beverly Raney, R.

In: Medical and Pediatric Oncology, Vol. 37, No. 2, 2001, p. 90-96.

Research output: Contribution to journalArticle

Fatih Okcu, M, Despa, S, Choroszy, M, Berrak, SG, Cangir, A, Jaffe, N & Beverly Raney, R 2001, 'Synovial sarcoma in children and adolescents: Thirty three years of experience with multimodal therapy', Medical and Pediatric Oncology, vol. 37, no. 2, pp. 90-96. https://doi.org/10.1002/mpo.1175
Fatih Okcu, M. ; Despa, Simona ; Choroszy, Mary ; Berrak, Su G. ; Cangir, Ayten ; Jaffe, Norman ; Beverly Raney, R. / Synovial sarcoma in children and adolescents : Thirty three years of experience with multimodal therapy. In: Medical and Pediatric Oncology. 2001 ; Vol. 37, No. 2. pp. 90-96.
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abstract = "Background. Synovial sarcoma (SS) is the most common type of non-rhabdomyosarcoma soft tissue sarcoma in childhood, with controversies about its prognosis and treatment. Procedure. We reviewed medical records of 42 children and adolescents with SS treated at our institution between 1966 and 1999 to determine treatment results and assess prognostic factors. Results. With a median follow-up duration of 7.8 years (range 0.2-22.4 years), 5-year progression free survival (PFS) and overall survival (OS) rates were 75.6{\%} (95{\%} Confidence Interval [Cl] 62-89.2{\%}) and 87.7{\%} (95{\%} Cl 77.3-98.1{\%}) respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. IRS grouping and tumor invasiveness were found to be significant prognostic indicators (P < 0.01 and = 0.02, respectively). Patients with initial gross total resection (IRS I and II) and non-invasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (<5 cm) (P = 0.09) or with monophasic histology (P = 0.14) had better PFS and OS. Conclusions. Achieving a complete resection or gross total resection with microscopic residual disease is vital for survival of patients with localized SS. Patients with localized disease who received radiotherapy had improved local control. Chemotherapy did not seem to impact PFS or OS. Future large multi-institutional trials are needed to address whether post-operative chemotherapy is necessary for patients with localized, surgically removed tumors, whether radiotherapy is necessary for patients with completely resected tumors, and to ascertain the order of importance of all the candidate prognostic markers.",
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AU - Jaffe, Norman

AU - Beverly Raney, R.

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N2 - Background. Synovial sarcoma (SS) is the most common type of non-rhabdomyosarcoma soft tissue sarcoma in childhood, with controversies about its prognosis and treatment. Procedure. We reviewed medical records of 42 children and adolescents with SS treated at our institution between 1966 and 1999 to determine treatment results and assess prognostic factors. Results. With a median follow-up duration of 7.8 years (range 0.2-22.4 years), 5-year progression free survival (PFS) and overall survival (OS) rates were 75.6% (95% Confidence Interval [Cl] 62-89.2%) and 87.7% (95% Cl 77.3-98.1%) respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. IRS grouping and tumor invasiveness were found to be significant prognostic indicators (P < 0.01 and = 0.02, respectively). Patients with initial gross total resection (IRS I and II) and non-invasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (<5 cm) (P = 0.09) or with monophasic histology (P = 0.14) had better PFS and OS. Conclusions. Achieving a complete resection or gross total resection with microscopic residual disease is vital for survival of patients with localized SS. Patients with localized disease who received radiotherapy had improved local control. Chemotherapy did not seem to impact PFS or OS. Future large multi-institutional trials are needed to address whether post-operative chemotherapy is necessary for patients with localized, surgically removed tumors, whether radiotherapy is necessary for patients with completely resected tumors, and to ascertain the order of importance of all the candidate prognostic markers.

AB - Background. Synovial sarcoma (SS) is the most common type of non-rhabdomyosarcoma soft tissue sarcoma in childhood, with controversies about its prognosis and treatment. Procedure. We reviewed medical records of 42 children and adolescents with SS treated at our institution between 1966 and 1999 to determine treatment results and assess prognostic factors. Results. With a median follow-up duration of 7.8 years (range 0.2-22.4 years), 5-year progression free survival (PFS) and overall survival (OS) rates were 75.6% (95% Confidence Interval [Cl] 62-89.2%) and 87.7% (95% Cl 77.3-98.1%) respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. IRS grouping and tumor invasiveness were found to be significant prognostic indicators (P < 0.01 and = 0.02, respectively). Patients with initial gross total resection (IRS I and II) and non-invasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (<5 cm) (P = 0.09) or with monophasic histology (P = 0.14) had better PFS and OS. Conclusions. Achieving a complete resection or gross total resection with microscopic residual disease is vital for survival of patients with localized SS. Patients with localized disease who received radiotherapy had improved local control. Chemotherapy did not seem to impact PFS or OS. Future large multi-institutional trials are needed to address whether post-operative chemotherapy is necessary for patients with localized, surgically removed tumors, whether radiotherapy is necessary for patients with completely resected tumors, and to ascertain the order of importance of all the candidate prognostic markers.

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