Veronique Minard-Colin1, David Walterhouse2, Gianni Bisogno3, Helene Martelli4, James Anderson5, David A Rodeberg6, Andrea Ferrari7, Meriel Jenney8, Suzanne Wolden9, Gianluca De Salvo10, Carola Arndt11, Johannes H M Merks12, Soledad Gallego13, Dominique Schwob14, Christine Haie-Meder15, Christophe Bergeron16, Michael C G Stevens17, Odile Oberlin1, Douglas Hawkins18,19. 1. Department of Pediatric and Adolescent Oncology, Gustave Roussy, Villejuif, France. 2. Division of Hematology, Oncology, and Stem Cell Transplantation, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois. 3. Pediatric Hematology and Oncology Division, Padova University, Padova, Italy. 4. Department of Pediatric Surgery, CHU Bicetre, Le Kremlin-Bicêtre, France. 5. Department of Oncology Clinical Research, Merck Research Laboratories, North Wales, Pennsylvania. 6. Department of Surgery, East Carolina University, Greenville, North Carolina. 7. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy. 8. Department of Pediatric Oncology, University of Wales, Cardiff, United Kingdom. 9. Department of Radiotherapy, Memorial Sloan Kettering Cancer Center, New York City, New York. 10. Clinical Trials and Biostatistics Unit, IRCCS Istituto Oncologico Veneto, Padova, Italy. 11. Department of Pediatric and Adolescent Medicine, Mayo Clinic and Foundation, Rochester, Minnesota. 12. Department of Pediatric Oncology, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands. 13. Department of Pediatric Oncology, Hospital Universitari Vall d'Hebron, Barcelona, Spain. 14. Department of Biostatistics, Gustave Roussy, Villejuif, France. 15. Department of Radiotherapy, Gustave Roussy, Villejuif, France. 16. Department of Pediatric and Adolescent Oncology, Centre Leon Berard, Lyon, France. 17. Department of Paediatric Oncology, Bristol Royal Hospital for Children, University of Bristol, Bristol, United Kingdom. 18. Division of Hematology/Oncology, Seattle Children's Hospital, University of Washington, Seattle, Washington. 19. Fred Hutchinson Cancer Research Center, University of Washington, Seattle, Washington.
Abstract
BACKGROUND: Vaginal/uterine rhabdomyosarcoma (VU RMS) is one of the most favorable RMS sites. To determine the optimal therapy, the experience of four cooperative groups (Children's Oncology Group [COG], International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumor Group [MMT], Italian Cooperative Soft Tissue Sarcoma Group [ICG], and European pediatric Soft tissue sarcoma Study Group [EpSSG]) was analyzed. PROCEDURE: From 1981 to 2009, 237 patients were identified. Median age (years) at diagnosis differed by tumor location; it was 1.9 for vagina (n = 160), 2.7 for uterus corpus (n = 26), and 13.5 for uterus cervix (n = 51). Twenty-eight percent of patients received radiation therapy (RT) as part of primary therapy (23% COG, 27% MMT, 46% ICG, and 42% EpSSG), with significant differences in the use of brachytherapy between the cooperative groups (23% COG, 76% MMT, 64% ICG, and 88% EpSSG). RESULTS: Ten-year event-free (EFS) and overall survival (OS) were 74% (95% CI, 67-79%) and 92% (95% CI, 88-96%), respectively. In univariate analysis, OS was inferior for patients with uterine RMS and for those with regional lymph node involvement. Although EFS was slightly lower in patients without initial RT (71% without RT vs. 81% with RT; P = 0.08), there was no difference in OS (94% without RT vs. 89% with RT; P = 0.18). Local control using brachytherapy was excellent (93%). Fifty-one (51.5%) of the 99 survivors with known primary therapy and treatment for relapse were cured with chemotherapy with or without conservative surgery. CONCLUSIONS: About half of all patients with VU RMS can be cured without systematic RT or radical surgery. When RT is indicated, modalities that limit sequelae should be considered, such as brachytherapy.
BACKGROUND: Vaginal/uterine rhabdomyosarcoma (VU RMS) is one of the most favorable RMS sites. To determine the optimal therapy, the experience of four cooperative groups (Children's Oncology Group [COG], International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumor Group [MMT], Italian Cooperative Soft Tissue Sarcoma Group [ICG], and European pediatric Soft tissue sarcoma Study Group [EpSSG]) was analyzed. PROCEDURE: From 1981 to 2009, 237 patients were identified. Median age (years) at diagnosis differed by tumor location; it was 1.9 for vagina (n = 160), 2.7 for uterus corpus (n = 26), and 13.5 for uterus cervix (n = 51). Twenty-eight percent of patients received radiation therapy (RT) as part of primary therapy (23% COG, 27% MMT, 46% ICG, and 42% EpSSG), with significant differences in the use of brachytherapy between the cooperative groups (23% COG, 76% MMT, 64% ICG, and 88% EpSSG). RESULTS: Ten-year event-free (EFS) and overall survival (OS) were 74% (95% CI, 67-79%) and 92% (95% CI, 88-96%), respectively. In univariate analysis, OS was inferior for patients with uterine RMS and for those with regional lymph node involvement. Although EFS was slightly lower in patients without initial RT (71% without RT vs. 81% with RT; P = 0.08), there was no difference in OS (94% without RT vs. 89% with RT; P = 0.18). Local control using brachytherapy was excellent (93%). Fifty-one (51.5%) of the 99 survivors with known primary therapy and treatment for relapse were cured with chemotherapy with or without conservative surgery. CONCLUSIONS: About half of all patients with VU RMS can be cured without systematic RT or radical surgery. When RT is indicated, modalities that limit sequelae should be considered, such as brachytherapy.
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