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Management of Primary Retroperitoneal Sarcoma (RPS) in the Adult: An Updated Consensus Approach from the Transatlantic Australasian RPS Working Group

  • Sarcoma
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Retroperitoneal soft tissue sarcomas comprise a heterogeneous group of rare tumors of mesenchymal origin that include several well-defined histologic subtypes. In 2015, the Transatlantic Australasian RPS Working Group (TARPSWG) published consensus recommendations for the best management of primary retroperitoneal sarcoma (RPS). Since then, through international collaboration, new evidence and knowledge have been generated, creating the need for an updated consensus document.

Methods

The primary aim of this study was to critically evaluate the current evidence and develop an up-to-date consensus document on the approach to these difficult tumors. The resulting document applies to primary RPS that is non-visceral in origin, with exclusion criteria as previously described. The relevant literature was evaluated and an international group of experts consulted to formulate consensus statements regarding the best management of primary RPS. A level of evidence and grade of recommendation were attributed to each new/updated recommendation.

Results

Management of primary RPS was considered from diagnosis to follow-up. This rare and complex malignancy is best managed by an experienced multidisciplinary team in a specialized referral center. The best chance of cure is at the time of primary presentation, and an individualized management plan should be made based on the 29 consensus statements included in this article, which were agreed upon by all of the authors. Whenever possible, patients should be enrolled in prospective trials and studies.

Conclusions

Ongoing international collaboration is critical to expand upon current knowledge and further improve outcomes of patients with RPS. In addition, prospective data collection and participation in multi-institution trials are strongly encouraged.

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Authors and Affiliations

  1. Department of Surgical Oncology, Princess Margaret Cancer Centre/Mount Sinai Hospital, Toronto, ON, Canada

    Carol J. Swallow MD, PhD, Rebecca A. Gladdy MD, PhD & Deanna Ng MBBS

  2. Department of Surgery, University of Toronto, Toronto, ON, Canada

    Carol J. Swallow MD, PhD, Rebecca A. Gladdy MD, PhD & Deanna Ng MBBS

  3. Sarcoma Unit, Department of Academic Surgery, Royal Marsden Hospital, Royal Marsden NHS Foundation Trust, London, UK

    Dirk C. Strauss MD, Andrew Hayes MA, PhD & Myles J. Smith MB, BCh, BAO, PhD

  4. Department of Surgical Oncology, Institut Curie, PSL University, Paris, France

    Sylvie Bonvalot MD, PhD, Dimitri Tzanis MD, PhD & Toufik Bouhadiba MD

  5. Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland

    Piotr Rutkowski MD, PhD & Jacek Skoczylas MD

  6. Department of Surgery, University Hospitals Birmingham, Birmingham, UK

    Anant Desai MD & Samuel J. Ford MB, ChB, PhD

  7. Sarcoma Department, H. Lee Moffitt Cancer Center, Tampa, FL, USA

    Ricardo Gonzalez MD & John E. Mullinax MD

  8. Department of Cancer Surgery, Peter MacCallum Cancer Centre, Melbourne, VIC, Australia

    David E. Gyorki MBBS, MD & Hayden Snow MBBS

  9. Department of Surgery, Brigham and Women’s Hospital, Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA

    Mark Fairweather MD & Chandrajit P. Raut MD MSc

  10. Department of Surgical Oncology, The Netherlands Cancer Institute, Amsterdam, The Netherlands

    Winan J. van Houdt MD, PhD

  11. Department of Surgery, Institut Bergonie, Bordeaux, France

    Eberhard Stoeckle MD

  12. Department of Surgery, Samsung Medical Center, Seoul, South Korea

    Jae Berm Park MD, PhD

  13. Department of General, Visceral and Transplantation Surgery, Ludwig-Maximilians-Universität, University Hospital, Munich, Germany

    Markus Albertsmeier PD, Dr.med.

  14. Department of Surgery, University of Ottawa, Ottawa, ON, Canada

    Carolyn Nessim MD, MSc

  15. Department of Surgery, Winship Cancer Institute, Emory University, Atlanta, GA, USA

    Kenneth Cardona MD

  16. Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

    Marco Fiore MD, Dario Callegaro MD & Alessandro Gronchi MD

  17. Department of Radiation Oncology, The Netherlands Cancer Institute, Amsterdam, The Netherlands

    Rick L. Haas MD, PhD

  18. Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

    Silvia Stacchiotti MD

  19. Department of Medical Oncology, Royal Marsden NHS Foundation Trust, London, UK

    Robin L. Jones MBBS, MD(Res)

  20. Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Thomas DeLaney MD

  21. Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Christina L. Roland MD

Authors
  1. Carol J. Swallow MD, PhD
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  2. Dirk C. Strauss MD
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  3. Sylvie Bonvalot MD, PhD
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  4. Piotr Rutkowski MD, PhD
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  5. Anant Desai MD
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  6. Rebecca A. Gladdy MD, PhD
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  7. Ricardo Gonzalez MD
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  8. David E. Gyorki MBBS, MD
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Consortia

on behalf of the Transatlantic Australasian RPS Working Group (TARPSWG)

Corresponding authors

Correspondence to Carol J. Swallow MD, PhD, Dirk C. Strauss MD or Alessandro Gronchi MD.

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Disclosure

Piotr Rutkowski has received honoraria for lectures and advisory boards from Novartis, BMS, MSD, Pierre Fabre, Blueprin Medicines, Pfizer, Sanofi, and Merck outside the scope of this manuscript. Silvia Stacchiotti reports personal financial interests (honoraria, consultancy or advisory role) from Adaptimmune, Bayer, Epizyme, Eli Lilly, Daiichi Sankyo, Deciphera, Immunedesign, Karyopharm, Maxivax, Novartis, Pharmamar, and Takeda; and institutional financial interests from Advenchen, Amgen Dompé, Bayer, Epizyme, Eli Lilly, Daiichi Sankyo, Karyopharm, Novartis, Pfizer, Pharmamar, and Springwork. Robin Jones is a consultant and/or advisor for Adaptimmune Therapeutics plc.; Athenex Inc.; Bayer; Blueprint Medicines; Boehringer Ingelheim Clinigen Group plc.; Daiichi Sankyo Company, Limited; Deciphera Pharmaceuticals Ltd.; Eisai Co., Ltd.; Eli Lilly and Company; Epizyme, Inc.; Immune Design Corp; Merck Sharp & Dohme; Pharma Mar S.A; and UpToDate, Inc. Carol J. Swallow, Dirk Strauss, Sylvie Bonvalot, Anant Desai, Rebecca A. Gladdy, Ricardo Gonzalez, David E. Gyorki, Mark Fairweather , Winan J. van Houdt, Eberhard Stoeckle, Jae Berm Park, Markus Albertsmeier, Carolyn Nessim, Kenneth Cardona, Marco Fiore, Andrew Hayes, Dimitri Tzanis, Jacek Skoczylas, Samuel J. Ford, Deanna Ng, John E. Mullinax, Hayden Snow, Rick L. Haas, Dario Callegaro, Myles J. Smith, Toufik Bouhadiba, Thomas DeLaney, Christina L. Roland, Chandrajit P. Raut, and Alessandro Gronchi have no conflicts of interest to declare.

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Swallow, C.J., Strauss, D.C., Bonvalot, S. et al. Management of Primary Retroperitoneal Sarcoma (RPS) in the Adult: An Updated Consensus Approach from the Transatlantic Australasian RPS Working Group. Ann Surg Oncol 28, 7873–7888 (2021). https://doi.org/10.1245/s10434-021-09654-z

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