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

• Authors: Carol J Swallow, Mount Sinai Hospital; Dirk C Strauss, Royal Marsden NHS Foundation Trust; Sylvie Bonvalot, PSL University; Piotr Rutkowski, Maria Sklodowska-Curie National Research Institute of Oncology; Anant Desai, University Hospitals Birmingham; Rebecca A Gladdy, Mount Sinai Hospital; Ricardo Gonzalez, H. Lee Moffitt Cancer Center; David E Gyorki, Peter MacCallum Cancer Centre; Mark Fairweather, Harvard Medical School; Winan J van Houdt, Netherlands Cancer Institute; Eberhard Stoeckle, Institut Bergonie; Jae Berm Park, Samsung Medical Center; Markus Albertsmeier, Ludwig-Maximilians-Universität; Carolyn Nessim, University of Ottawa; Kenneth Cardona, Emory University; Marco Fiore, Fondazione IRCCS Istituto Nazionale dei Tumori; Andrew Hayes, Royal Marsden Hospital, Royal Marsden NHS Foundation Trust; Dimitri Tzanis, PSL University; Jacek Skoczylas, Maria Sklodowska-Curie National Research Institute of Oncology; Samuel J Ford, University Hospitals Birmingham; Deanna Ng, Mount Sinai Hospital; John E Mullinax, H. Lee Moffitt Cancer Center; Hayden Snow, Peter MacCallum Cancer Centre; Rick L Haas, Netherlands Cancer Institute; Dario Callegaro, Fondazione IRCCS Istituto Nazionale dei Tumori; Myles J Smith, Royal Marsden NHS Foundation Trust; Toufik Bouhadiba, PSL University; Silvia Stacchiotti, Fondazione IRCCS Istituto Nazionale dei Tumori; Robin L Jones, Royal Marsden NHS Foundation Trust; Thomas DeLaney, Harvard Medical School; Christina L Roland, The University of Texas MD Anderson Cancer Center; Chandrajit P Raut, Harvard Medical School; Alessandro Gronchi, Fondazione IRCCS Istituto Nazionale dei Tumori
• Journal Title: ANNALS OF SURGICAL ONCOLOGY
• Volume: Volume 28, Number 12
• Publisher: SPRINGER | 2021-04-14, Pages 7873-7888
• Type of Work: Article | Post-print: After Peer Review
• Publisher DOI: http://doi.org/10.1245/s10434-021-09654-z
• Subjects: Health Sciences, Oncology; Health Sciences, Medicine and Surgery
• Keywords: Science & Technology; Life Sciences & Biomedicine; Oncology; Surgery; SOFT-TISSUE SARCOMA; IMPROVES RISK STRATIFICATION; RADIATION-THERAPY; SURGICAL RESECTION; POSTOPERATIVE NOMOGRAM; ADJUVANT CHEMOTHERAPY; INTERMEDIATE-GRADE; PROGNOSTIC-FACTORS; BEAM RADIOTHERAPY; OPEN-LABEL

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.