Synovial Sarcoma in Children, Adolescents, and Young Adults: A Report From the Children's Oncology Group ARST0332 Study
| العنوان: | Synovial Sarcoma in Children, Adolescents, and Young Adults: A Report From the Children's Oncology Group ARST0332 Study |
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| المؤلفون: | Wei Xue, Jennifer O. Black, Andrew Ostrenga, Simon C. Kao, Barry L. Shulkin, James R. Anderson, R. Lor Randall, Rajkumar Venkatramani, Suzanne L. Wolden, Dolores Lopez-Terrada, Alberto S. Pappo, Sheri L. Spunt |
| المصدر: | Journal of clinical oncology : official journal of the American Society of Clinical Oncology, vol 39, iss 35 J Clin Oncol |
| بيانات النشر: | eScholarship, University of California, 2021. |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | Male, Cancer Research, Prospective Studies, Young adult, Child, Cancer, Pediatric, screening and diagnosis, Soft tissue sarcoma, Soft tissue, Sarcoma, ORIGINAL REPORTS, Prognosis, Combined Modality Therapy, Synovial sarcoma, Neoadjuvant Therapy, 6.5 Radiotherapy and other non-invasive therapies, Survival Rate, Detection, Oncology, Child, Preschool, Treatment strategy, Female, Patient Safety, 4.2 Evaluation of markers and technologies, Adult, medicine.medical_specialty, Pediatric Research Initiative, Adolescent, Pediatric Cancer, Clinical Trials and Supportive Activities, Clinical Sciences, Oncology and Carcinogenesis, Sarcoma, Synovial, Young Adult, Rare Diseases, Clinical Research, Internal medicine, medicine, Humans, Oncology & Carcinogenesis, Preschool, Synovial, business.industry, Infant, Evaluation of treatments and therapeutic interventions, medicine.disease, Orphan Drug, Early adolescents, business, Follow-Up Studies |
| الوصف: | PURPOSE Synovial sarcoma (SS) is the second most common malignant soft tissue tumor in children. ARST0332 evaluated a risk-based treatment strategy for young patients with soft tissue sarcoma designed to limit therapy for low-risk (LR) disease and to test neoadjuvant chemoradiotherapy for unresected higher-risk disease. METHODS Newly diagnosed patients with SS age < 30 years were assigned to four treatment arms based on disease features: A (surgery only), B (55.8 Gy radiotherapy [RT]), C (ifosfamide and doxorubicin [ID] chemotherapy plus 55.8 Gy RT), and D (neoadjuvant ID and 45 Gy RT, then surgery and RT boost based on margins followed by adjuvant ID). Patients treated in Arms A and B were considered LR, arms C and D without metastases as intermediate-risk (IR), and those with metastases as high-risk (HR). RESULTS Of the 146 patients with SS enrolled, 138 were eligible and evaluable: LR (46), IR (71), and HR (21). Tumors were 80% extremity, 70% > 5 cm, 70% high-grade, 62% invasive, 95% deep, and 15% metastatic. Treatment was on arm A (29.7%), B (3.6%), C (16.7%), and D (50%). There were no toxic deaths and four unexpected grade 4 adverse events. By risk group, at a median follow-up of 6.8 years, estimated 5-year event-free survival was LR 82%, IR 70%, and HR 8%, and overall survival was LR 98%, IR 89%, and HR 13%. After accounting for the features that defined risk category, none of the other patient or disease characteristics (age, sex, tumor site, tumor invasiveness, and depth) improved the risk stratification model. CONCLUSION The risk-based treatment strategy used in ARST0332 produced favorable outcomes in patients with nonmetastatic SS relative to historical controls despite using RT less frequently and at lower doses. The outcome for metastatic SS remains unsatisfactory and new therapies are urgently needed. |
| وصف الملف: | application/pdf |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::f739b27bf97ada1362acb9704f7d5411 https://escholarship.org/uc/item/62b5r8q0 |
| Rights: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....f739b27bf97ada1362acb9704f7d5411 |
| قاعدة البيانات: | OpenAIRE |
| الوصف غير متاح. |