Courtney J Harris1,2, Irene Helenowski2, Andrew J Murphy3, Sara A Mansfield3, Michael P LaQuaglia4, Todd E Heaton4, Michele Cavalli4, Joseph T Murphy5, Erika Newman6, Richard E Overmen6, Tanvi T Kartal6, Jo Cooke-Barber7, Addison Donaher7, Marcus M Malek8, Ranjeet Kalsi8, Eugene S Kim9, Michael J Zobel9, Catherine J Goodhue9, Bindi J Naik-Mathuria10, Imory N Jefferson10, Jonathan P Roach11, Claudia Mata11, Nelson Piché12, Shahrzad Joharifard12, Serge Sultan13, Scott S Short14, Rebecka L Meyers14, Josh Bleicher14, Hau D Le15, Kevin Janek15, Andreana Bütter16, Jacob Davidson16, Jennifer H Aldrink17, Holden W Richards17, Elisabeth T Tracy18, Sarah J Commander18, Elizabeth A Fialkowski19, Misty Troutt7, Roshni Dasgupta7, Timothy B Lautz1,2. 1. Division of Pediatric Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois. 2. Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois. 3. Division of General Pediatric Surgery, Surgery Department, St Jude Children's Research Hospital, Memphis, Tennessee. 4. Division of Pediatric Surgery, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York. 5. Division of Pediatric Surgery, University of Texas Southwestern Medical Center, Dallas, Texas. 6. Section of Pediatric Surgery, Department of Surgery, University of Michigan School of Medicine, Ann Arbor MI. 7. Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. 8. Division of Pediatric General and Thoracic Surgery, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania. 9. Division of Pediatric Surgery, Department of Surgery, Children's Hospital Los Angeles, Los Angeles, California. 10. Division of Pediatric Surgery, Department of Surgery, Texas Children's Hospital, Houston, Texas. 11. Children's Hospital of Colorado, University of Colorado Anschutz Medical Campus, School of Medicine, Aurora, Colorado. 12. Division of Pediatric Surgery, Centre Hospitalier Universitaire Ste-Justine, Montreal, Quebec, Canada. 13. Department of Psychology and Pediatrics, University of Montreal, Centre Hospitalier Universitaire Ste-Justine Research Center, Montreal, Quebec, Canada. 14. Division of Pediatric Surgery, Primary Children's Hospital, University of Utah School of Medicine, Salt Lake City, Utah. 15. Division of Pediatric Surgery, American Family Children's Hospital, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin. 16. Division of Pediatric Surgery, Children's Hospital, London Health Sciences Centre, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada. 17. Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio. 18. Division of Pediatric Surgery, Department of Surgery, Duke University Medical Center, Durham, North Carolina. 19. Department of Surgery, Division of Pediatric Surgery, Oregon Health and Science University, Portland, Oregon.
Abstract
OBJECTIVE: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development and (3) patient-reported quality of life in children with sarcoma of the chest wall. SUMMARY BACKGROUND DATA: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. METHODS: A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008-2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. RESULTS: The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (IQR = 1-3), and number of ribs resected did not correlate with margin status (p = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor (HR 2.24, p = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posterior rib resection (HR 8.43; p = 0.003) and increased number of ribs resected (HR 1.78; p = 0.02). Overall, patient-reported quality of life is not impaired following chest wall tumor resection. CONCLUSIONS: Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.
OBJECTIVE: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development and (3) patient-reported quality of life in children with sarcoma of the chest wall. SUMMARY BACKGROUND DATA: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. METHODS: A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008-2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. RESULTS: The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (IQR = 1-3), and number of ribs resected did not correlate with margin status (p = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor (HR 2.24, p = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posterior rib resection (HR 8.43; p = 0.003) and increased number of ribs resected (HR 1.78; p = 0.02). Overall, patient-reported quality of life is not impaired following chest wall tumor resection. CONCLUSIONS: Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.
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