S Radaelli1, P Fossati2, S Stacchiotti3, T Akiyama4, J M Asencio5, S Bandiera6, A Boglione7, P Boland8, S Bolle9, Ø Bruland10, A Brunello11, P Bruzzi12, D Campanacci13, F Cananzi14, R Capanna15, R Casadei16, A Cordoba17, C Court18, A P Dei Tos19, T F DeLaney20, A De Paoli21, T M De Pas22, A Desai23, L Di Brina24, D M Donati16, N Fabbri8, M R Fiore25, A Frezza3, M Gambarotti26, A Gasbarrini6, P Georg2, G Grignani27, N Hindi28, E B Hug2, R Jones29, A Kawai30, A D Krol31, F Le Grange32, A Luzzati33, G Marquina34, J A Martin-Benlloch35, K Mazzocco36, F Navarria21, P Navarria24, P D Parchi15, S Patel37, E Pennacchioli38, M G Petrongari39, P Picci40, R Pollock41, L Porcu42, V Quagliuolo15, C Sangalli43, S Scheipl44, G M Scotto34, M Spalek45, T Steinmeier46, B Timmermann47, A Trama48, M Uhl49, C Valverde50, P P Varga51, R Verges52, D C Weber53, C Zoccali54, P G Casali55, J Sommer56, A Gronchi57. 1. Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. Electronic address: stefano.radaelli@istitutotumori.mi.it. 2. MedAustron Ion Therapy Center, Wiener Neustadt, Austria. 3. Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy. 4. Department of Orthopeadic Surgery, Saitama Medical Center, Jichi Medical University, Saitama, Japan. 5. General Surgery III Department and Liver Transplant Unit, Hospital General Universitario Gregorio Marañón, Madrid, Spain. 6. Department of Oncologic and Degenerative Spine Surgery, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy. 7. Medical Oncology Unit, Ospedale Humanitas, Gradenigo, Torino, Italy. 8. Orthopedic Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, USA. 9. Département de Radiothérapie, Gustave-Roussy Cancer Campus, Villejuif, France. 10. University of Oslo, Institute for Clinical Medicine and Department of Oncology, Oslo University Hospital-Norwegian Radium Hospital, Oslo, Norway. 11. Department of Clinical and Experimental Oncology, Medical Oncology 1st Unit, Istituto Oncologico Veneto IOV-IRCCS, Padova, Italy. 12. Dipartimento di Epidemiologia Clinica, IRCCS, AOU San Martino - IST, Genova, Italy. 13. Department of Orthopedic Oncology, Azienda Ospedaliero Universitaria Careggi, Firenze, Toscana, Italy. 14. Surgical Oncology Unit - Humanitas Clinical and Research Center, Rozzano, Italy. 15. 1st Orthopedic Division of Pisa University, Department of Translational Research and New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy. 16. Department of Orthopedics, Istituto Ortopedico Rizzoli, University of Bologna, Bologna, Italy. 17. Department of Radiotherapy, Oscar Lambret Comprehensive Cancer Center, Lille, France. 18. Orthopaedic and Traumatology Department, Spine and Tumor Unit, Bicetre University Hospital, AP-HP Paris, Univ. Paris-Sud Orsay, France. 19. Department of Pathology and Molecular Genetics, Treviso General Hospital, Treviso, Italy; Department of Medicine, University of Padova School of Medicine, Padova, Italy. 20. Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA, USA. 21. Department of Radiation Oncology, Centro di Riferimento Oncologico, National Cancer Institute, Aviano, Italy. 22. Division of Medical Oncology for Melanoma & Sarcoma, IEO, European Institute of Oncology IRCCS, Milan, Italy. 23. Department of Sarcoma and General Surgery, Midlands Abdominal and Retroperitoneal Sarcoma Unit, University Hospital Birmingham, NHS Foundation Trust, Birmingham, UK. 24. Radiotherapy and Radiosurgery, Humanitas Clinical and Research Center, Rozzano, Milano, Italy. 25. Radiotherapy Unit, National Center of Oncological Hadrontherapy (CNAO) Pavia, Italy. 26. Department of Pathology, IRCCS Rizzoli Orthopaedic Institute, Bologna, Italy. 27. Medical Oncology-Sarcoma Unit, Istituto di Candiolo-Fondazione del Piemonte per L'Oncologia, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Candiolo, Italy. 28. Instituto de Biomedicina (IBIS), Medical Oncology Department, Hospital Universitario Virgen del Rocio, Sevilla, Spain. 29. Royal Marsden Hospital and Institute of Cancer Research, London, UK. 30. Department of Musculoskeletal Oncology, National Cancer Center, Tokyo, Japan. 31. Department of Radiation Oncology, Leiden University Medical Center, Leiden, the Netherlands. 32. University College London Hospitals NHS Foundation Trust and the London Sarcoma Service, UK. 33. Centro di Chirurgia Ortopedica Oncologica e Ricostruttiva del Rachide, IRCCS Istituto Ortopedico Galeazzi, Milano, Italy. 34. Department of Medical Oncology, Hospital Clinico San Carlos, Madrid, Spain. 35. Department of Orthopaedic Surgery, Hospital Clinico Universitario de Valencia, Valencia, Spain. 36. Applied Research Division for Cognitive and Psychological Science, European Institute of Oncology, Department of Oncology and Hemato-Oncology, University of Milan, Milano, Lombardia, Italy. 37. Department of Sarcoma Medical Oncology, Division of Cancer Medicine, University of Texas M.D. Anderson Cancer Center, Houston, USA. 38. Division of Melanoma, Soft Tissue Sarcomas and Rare Tumors, European Institute of Oncology, Milan, Italy. 39. Department of Radiation Oncology, Regina Elena National Cancer Institute, Rome, Italy. 40. Laboratory of Experimental Oncology, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy. 41. Royal National Orthopaedic Hospital NHS Trust, Brockley Hill, Stanmore, Middlesex, UK. 42. Laboratory of Methodology for Clinical Research, Department of Oncology, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Italy. 43. Department of Radiation Therapy, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. 44. Department of Orthopaedics and Trauma, Medical University of Graz, Graz, Austria. 45. Department of Radiotherapy I, Maria Sklodowska-Curie Institute-Oncology Center, Warsaw, Poland. 46. Department of Particle Therapy, University Hospital Essen, West German Proton Therapy Centre Essen (WPE), West German Cancer Center (WTZ), Germany. 47. Department of Particle Therapy, University Hospital Essen, West German Proton Therapy Centre Essen (WPE), West German Cancer Center (WTZ), German Cancer Consortium (DKTK), Germany. 48. Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale Tumori di Milano, Milan, Italy. 49. Department of Radiation Oncology, University of Heidelberg, Heidelberg, Germany. 50. Department of Medical Oncology, Vall d'Hebron University Hospital, Barcelona, Spain. 51. National Center for Spinal Disorders, Budapest, Hungary. 52. Radiation Oncology Department, Vall d'Hebron University Hospital, Barcelona, Spain. 53. Centre for Proton Therapy, Paul Scherrer Institut, Villigen PSI, Switzerland. 54. Orthopedic Oncology Unit, Department of Experimental Clinical Oncology, Regina Elena National Cancer Institute, Rome, Italy. 55. Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy; Department of Medical Oncology and Haemato-Oncology, University of Milan, Milan, Italy. 56. Chordoma Foundation, Durham, NC, USA. 57. Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Abstract
OBJECTIVE: Aim of the manuscript is to discuss how to improve margins in sacral chordoma. BACKGROUND: Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery. METHODS: A multidisciplinary meeting of the "Chordoma Global Consensus Group" was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies. This review aims to report on the outcome of the consensus meeting and to provide a summary of the most recent evidence in this field. Possible new ways forward, including on-going international clinical studies, are discussed. RESULTS: En-bloc tumor-sacrum resection is the cornerstone of treatment of primary sacral chordoma, aiming to achieve negative microscopic margins. Radical definitive particle therapy seems to offer a similar outcome compared to surgery, although confirmation in comparative trials is lacking; besides there is still a certain degree of technical variability across institutions, corresponding to different fields of treatment and different tumor coverage. To address some of these questions, a prospective, randomized international study comparing surgery versus definitive high-dose RT is ongoing. Available data do not support the routine use of any medical therapy as (neo)adjuvant/cytoreductive treatment. CONCLUSION: Given the significant influence of margins status on local control in patients with primary localized sacral chordoma, the clear definition of adequate margins and a standard local approach across institutions for both surgery and particle RT is vital for improving the management of these patients.
OBJECTIVE: Aim of the manuscript is to discuss how to improve margins in sacral chordoma. BACKGROUND:Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery. METHODS: A multidisciplinary meeting of the "Chordoma Global Consensus Group" was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies. This review aims to report on the outcome of the consensus meeting and to provide a summary of the most recent evidence in this field. Possible new ways forward, including on-going international clinical studies, are discussed. RESULTS: En-bloc tumor-sacrum resection is the cornerstone of treatment of primary sacral chordoma, aiming to achieve negative microscopic margins. Radical definitive particle therapy seems to offer a similar outcome compared to surgery, although confirmation in comparative trials is lacking; besides there is still a certain degree of technical variability across institutions, corresponding to different fields of treatment and different tumor coverage. To address some of these questions, a prospective, randomized international study comparing surgery versus definitive high-dose RT is ongoing. Available data do not support the routine use of any medical therapy as (neo)adjuvant/cytoreductive treatment. CONCLUSION: Given the significant influence of margins status on local control in patients with primary localized sacral chordoma, the clear definition of adequate margins and a standard local approach across institutions for both surgery and particle RT is vital for improving the management of these patients.
Authors: Joaquim Soares do Brito; Miguel Esperança-Martins; André Abrunhosa-Branquinho; Cecilia Melo-Alvim; Raquel Lopes-Brás; João Janeiro; Dolores Lopez-Presa; Isabel Fernandes; José Portela; Luis Costa Journal: Cancers (Basel) Date: 2022-05-22 Impact factor: 6.575