Sheri L Spunt1, Nadine Francotte2, Gian Luca De Salvo3, Yueh-Yun Chi4, Ilaria Zanetti5, Andrea Hayes-Jordan6, Simon C Kao7, Daniel Orbach8, Bernadette Brennan9, Aaron R Weiss10, Max M van Noesel11, Lynn Million12, Rita Alaggio13, David M Parham14, Anna Kelsey15, R Lor Randall16, M Beth McCarville17, Gianni Bisogno5, Douglas S Hawkins18, Andrea Ferrari19. 1. Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA, USA. Electronic address: sspunt@stanford.edu. 2. Department of Pediatrics, CHC-Clinique de l'Esperance, rue Saint Nicolas, Montegnee, Belgium. 3. Clinical Research Unit, Veneto Institute of Oncology IOV - IRCCS, Padua, Italy. 4. Department of Biostatistics, University of Florida, Gainesville, FL, USA. 5. Hematology Oncology Division, Department of Woman's and Child's Health, University of Padova, Padova, Italy. 6. Department of Pediatric Surgery, UNC Lineberger Comprehensive Cancer Center, Chapel Hill, NC, USA. 7. Department of Radiology, University of Iowa Carver College of Medicine, Iowa City, IA, USA. 8. SIREDO Oncology Center (Care Innovation and Research for Children and AYA with Cancer), PSL Research University, Institut Curie, Paris, France. 9. Department of Pediatric Oncology, Royal Manchester Children's Hospital, Manchester, United Kingdom. 10. Department of Pediatrics, Maine Medical Center, Portland, ME, USA. 11. Princess Máxima Center for Pediatric Oncology, Utrecht, Netherlands. 12. Department of Radiation Oncology, Stanford University School of Medicine, Palo Alto, CA, USA. 13. Department of Pathology, University of Pittsburgh, Pittsburgh, PA, USA. 14. Department of Pathology and Laboratory Medicine, USC Keck School of Medicine and Children's Hospital of Los Angeles, Los Angeles, CA, USA. 15. Department of Paediatric Histopathology, Royal Manchester Children's Hospital, Manchester, United Kingdom. 16. Department of Orthopedic Surgery, University of California Davis School of Medicine, Sacramento, CA, USA. 17. Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN, USA. 18. Hematology/Oncology, Seattle Children's Hospital, University of Washington, Fred Hutchinson Cancer Research Center, Seattle, WA, USA. 19. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
Abstract
BACKGROUND: Data on the clinical features, optimal treatment and outcomes of paediatric patients with epithelioid sarcoma (ES) are limited and mostly retrospective. METHODS: A subset analysis of ES patients < 30 years of age enrolled on two international prospective clinical trials conducted between 7/2005 and 11/2015 was performed. Risk-adapted therapy was based on tumour diameter, histologic grade, extent of surgery and presence/absence of metastases and included surgery ± radiotherapy for all patients with the addition of ifosfamide/doxorubicin chemotherapy for intermediate-/high-risk patients. Response to therapy, event-free and overall survival and pattern and predictors of treatment failure were evaluated. RESULTS: Sixty-three ES patients (median age 13.1 years, 52% male) were eligible. Clinical features included the following: 68% extremity, median tumour diameter 3.5 cm, 56% high histologic grade, 14% nodal metastases, 14% distant metastases. Thirty-four low-risk patients underwent surgery (n = 30) or surgery/radiotherapy (n = 4); 16 intermediate-risk and 13 high-risk patients received chemotherapy ± surgery ± radiotherapy. Partial response was observed in 11/22 (50%) patients receiving neoadjuvant therapy. Events were local recurrence (n = 10) and distant recurrence (n = 15); estimated 5-year survival was 86.4%, 63.5% and 0%, respectively, for low-, intermediate- and high-risk patients. Locoregional nodal involvement, invasive tumour, high grade and lesser extent of resection predicted event-free survival in patients without metastases. CONCLUSIONS: Most low-risk ES patients who have undergone an adequate resection fare well without adjuvant therapy. Large tumour size, high histologic grade, tumour invasiveness, inadequate tumour resection and metastatic disease predict poorer outcomes in higher risk ES patients, for whom more effective therapies are needed. CLINICAL TRIAL REGISTRATION: COG ARST0332: ClinicalTrials.gov Identifier NCT00346164, EpSSG NRSTS 2005: European Union Drug Regulating Authorities Clinical Trials No. 2005-001139-31.
BACKGROUND: Data on the clinical features, optimal treatment and outcomes of paediatric patients with epithelioid sarcoma (ES) are limited and mostly retrospective. METHODS: A subset analysis of ES patients < 30 years of age enrolled on two international prospective clinical trials conducted between 7/2005 and 11/2015 was performed. Risk-adapted therapy was based on tumour diameter, histologic grade, extent of surgery and presence/absence of metastases and included surgery ± radiotherapy for all patients with the addition of ifosfamide/doxorubicin chemotherapy for intermediate-/high-risk patients. Response to therapy, event-free and overall survival and pattern and predictors of treatment failure were evaluated. RESULTS: Sixty-three ES patients (median age 13.1 years, 52% male) were eligible. Clinical features included the following: 68% extremity, median tumour diameter 3.5 cm, 56% high histologic grade, 14% nodal metastases, 14% distant metastases. Thirty-four low-risk patients underwent surgery (n = 30) or surgery/radiotherapy (n = 4); 16 intermediate-risk and 13 high-risk patients received chemotherapy ± surgery ± radiotherapy. Partial response was observed in 11/22 (50%) patients receiving neoadjuvant therapy. Events were local recurrence (n = 10) and distant recurrence (n = 15); estimated 5-year survival was 86.4%, 63.5% and 0%, respectively, for low-, intermediate- and high-risk patients. Locoregional nodal involvement, invasive tumour, high grade and lesser extent of resection predicted event-free survival in patients without metastases. CONCLUSIONS: Most low-risk ES patients who have undergone an adequate resection fare well without adjuvant therapy. Large tumour size, high histologic grade, tumour invasiveness, inadequate tumour resection and metastatic disease predict poorer outcomes in higher risk ES patients, for whom more effective therapies are needed. CLINICAL TRIAL REGISTRATION: COG ARST0332: ClinicalTrials.gov Identifier NCT00346164, EpSSG NRSTS 2005: European Union Drug Regulating Authorities Clinical Trials No. 2005-001139-31.
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