Skip to main content

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

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.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  1. Porter GA, Baxter NN, Pisters PW. Retroperitoneal sarcoma: a population-based analysis of epidemiology, surgery, and radiotherapy. Cancer. 2006;106(7):1610–6.

    PubMed  Article  Google Scholar 

  2. Ferrari A, et al. Soft tissue sarcoma across the age spectrum: a population-based study from the Surveillance Epidemiology and End Results database. Pediatr Blood Cancer. 2011;57(6):943–9.

    PubMed  PubMed Central  Article  Google Scholar 

  3. Cormier JN, Gronchi A, Pollock RE. Soft tissue sarcomas. In: Brunicardi FC, Andersen DK, Billiar TR, Dunn DL, Hunter JG, Matthews JB, et al. (eds). Schwartz’s principles of surgery 10th edition. New York: McGraw-Hill; 2014.

  4. Dei Tos AP, Marino-Enriquez A, Pedeutour F. Soft Tissue Tumours. In: WHO Classification of Tumours Editorial Board (eds). WHO Classification of Tumours of Soft Tissue and Bone. Fifth Edition, Volume 3. Lyon: IARC Publications; 2020.

  5. Lewis JJ, et al. Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg. 1998;228(3):355–65.

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  6. Stoeckle E, et al. Prognostic factors in retroperitoneal sarcoma: a multivariate analysis of a series of 165 patients of the French Cancer Center Federation Sarcoma Group. Cancer. 2001;92(2):359–68.

    CAS  PubMed  Article  Google Scholar 

  7. Gronchi A, et al. Retroperitoneal soft tissue sarcomas: patterns of recurrence in 167 patients treated at a single institution. Cancer. 2004;100(11):2448–55.

    PubMed  Article  Google Scholar 

  8. Hassan I, et al. Operative management of primary retroperitoneal sarcomas: a reappraisal of an institutional experience. Ann Surg. 2004;239(2):244–50.

    PubMed  PubMed Central  Article  Google Scholar 

  9. van Dalen T, et al. Long-term prognosis of primary retroperitoneal soft tissue sarcoma. Eur J Surg Oncol. 2007;33(2):234–8.

    PubMed  Article  Google Scholar 

  10. Lehnert T, et al. Primary and locally recurrent retroperitoneal soft-tissue sarcoma: local control and survival. Eur J Surg Oncol. 2009;35(9):986–93.

    CAS  PubMed  Article  Google Scholar 

  11. Anaya DA, et al. Multifocality in retroperitoneal sarcoma: a prognostic factor critical to surgical decision-making. Ann Surg. 2009;249(1):137–42.

    PubMed  Article  Google Scholar 

  12. Strauss DC, et al. Surgical management of primary retroperitoneal sarcoma. Br J Surg. 2010;97(5):698–706.

    CAS  PubMed  Article  Google Scholar 

  13. Bonvalot S, et al. Primary retroperitoneal sarcomas: a multivariate analysis of surgical factors associated with local control. J Clin Oncol. 2009;27(1):31–7.

    PubMed  Article  Google Scholar 

  14. Gronchi A, et al. Aggressive surgical policies in a retrospectively reviewed single-institution case series of retroperitoneal soft tissue sarcoma patients. J Clin Oncol. 2009;27(1):24–30.

    PubMed  Article  Google Scholar 

  15. Bonvalot S, et al. Aggressive surgery in retroperitoneal soft tissue sarcoma carried out at high-volume centers is safe and is associated with improved local control. Ann Surg Oncol. 2010;17(6):1507–14.

    PubMed  Article  Google Scholar 

  16. Gronchi A, et al. Frontline extended surgery is associated with improved survival in retroperitoneal low- to intermediate-grade soft tissue sarcomas. Ann Oncol. 2012;23(4):1067–73.

    CAS  PubMed  Article  Google Scholar 

  17. Singer S, et al. Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma. Ann Surg. 2003;238(3):358–70.

    PubMed  PubMed Central  Article  Google Scholar 

  18. Dalal KM, et al. Subtype specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk. Ann Surg. 2006;244(3):381–91.

    PubMed  PubMed Central  Article  Google Scholar 

  19. Canter RJ, et al. Why do patients with low-grade soft tissue sarcoma die? Ann Surg Oncol. 2008;15(12):3550–60.

    PubMed  PubMed Central  Article  Google Scholar 

  20. Almond LM, et al. Neoadjuvant and adjuvant strategies in retroperitoneal sarcoma. Eur J Surg Oncol. 2018;44(5):571–9.

    PubMed  Article  Google Scholar 

  21. Bonvalo S, et al. Preoperative radiotherapy plus surgery versus surgery alone for patients with primary retroperitoneal sarcoma (EORTC-62092: STRASS): a multicentre, open-label, randomised, phase 3 trial. Lancet Oncol. 2020;21(10):1366–77.

    Article  Google Scholar 

  22. Bonvalot S, et al. Survival Benefit of the Surgical Management of Retroperitoneal Sarcoma in a Reference Center: A Nationwide Study of the French Sarcoma Group from the NetSarc Database. Ann Surg Oncol. 2019;26(7):2286–93.

    CAS  PubMed  Article  Google Scholar 

  23. Putt ME. Is surgery for retroperitoneal sarcoma at “low-volume” hospitals a bad idea? Cancer. 2018;124(23):4447–51. https://doi.org/10.1002/cncr.31750.

    Article  PubMed  Google Scholar 

  24. Raut CP, Bonvalot S, Gronchi A. A call to action: Why sarcoma surgery needs to be centalized. Cancer. 2018;124(23):4452–4.

    PubMed  Article  Google Scholar 

  25. Gronchi A, Pollock R. Surgery in retroperitoneal soft tissue sarcoma: a call for a consensus between Europe and North America. Ann Surg Oncol. 2011;18(8):2107–10.

    PubMed  Article  Google Scholar 

  26. van Houdt WJ, et al. New research strategies in retroperitoneal sarcoma. The case of TARPSWG, STRASS and RESAR: making progress through collaboration. Curr Opin Oncol. 2019;31(4):310-316.

  27. Management of primary retroperitoneal sarcoma (RPS) in the adult: a consensus approach from the Trans-Atlantic RPS Working Group. Ann Surg Oncol. 2015;22(1):256-63.

  28. Management of Recurrent Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach from the Trans-Atlantic RPS Working Group. Ann Surg Oncol. 2016;23(11):3531-3540.

  29. Management of metastatic retroperitoneal sarcoma. a consensus approach from the Trans-Atlantic Retroperitoneal Sarcoma Working Group (TARPSWG). Ann Oncol. 2018;29(4):857–71.

    Article  Google Scholar 

  30. Gutierrez JC, et al. Should soft tissue sarcomas be treated at high-volume centers? An analysis of 4205 patients. Ann Surg. 2007;245(6):952–8.

    PubMed  PubMed Central  Article  Google Scholar 

  31. Heudel PE, et al. Territorial inequalities in management and conformity to clinical guidelines for sarcoma patients: an exhaustive population-based cohort analysis in the Rhone-Alpes region. Int J Clin Oncol. 2014;19(4):744–52.

    PubMed  Google Scholar 

  32. Keung EZ, et al. Treatment at low-volume hospitals is associated with reduced short-term and long-term outcomes for patients with retroperitoneal sarcoma. Cancer. 2018;124(23):4495–503.

    PubMed  Article  Google Scholar 

  33. Toulmonde M, et al. Retroperitoneal sarcomas: patterns of care at diagnosis, prognostic factors and focus on main histological subtypes: a multicenter analysis of the French Sarcoma Group. Ann Oncol. 2014;25(3):735–42.

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  34. Villano AM, et al. Identifying the Minimum Volume Threshold for Retroperitoneal Soft Tissue Sarcoma Resection: Merging National Data with Consensus Expert Opinion. J Am Coll Surg. 2020;230(1):151-160.e2.

    PubMed  Article  Google Scholar 

  35. Blay JY, et al. Surgery in reference centers improves survival of sarcoma patients: a nationwide study. Ann Oncol. 2019;30(7):1143–53.

    PubMed  PubMed Central  Article  Google Scholar 

  36. Andritsch E, et al. ECCO Essential Requirements for Quality Cancer Care: Soft Tissue Sarcoma in Adults and Bone Sarcoma. A critical review. Crit Rev Oncol Hematol. 2017; 110:94-105.

  37. Messiou C, Morosi C. Imaging in retroperitoneal soft tissue sarcoma. J Surg Oncol. 2018;117(1):25–32.

    PubMed  Article  Google Scholar 

  38. Messiou C, et al. Primary retroperitoneal soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic algorithm. Eur J Surg Oncol. 2017;43(7):1191–8.

    CAS  PubMed  Article  Google Scholar 

  39. Kim DB, et al. Effect of nephrectomy for retroperitoneal sarcoma on post-operative renal function. J Surg Oncol. 2018;117(3):425–9.

    PubMed  Article  Google Scholar 

  40. Fendler WP, et al. Evaluation of several FDG PET parameters for prediction of soft tissue tumour grade at primary diagnosis and recurrence. Eur Radiol. 2015;25(8):2214–21.

    PubMed  Article  Google Scholar 

  41. von Mehren M, et al. Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2018;16(5):536-563.

  42. Casali PG, et al. Soft tissue and visceral sarcomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018;29 Suppl 4:iv51–iv67.

  43. Miah AB, et al. Optimal management of primary retroperitoneal sarcoma: an update. Expert Rev Anticancer Ther. 2014;14(5):565–79.

    CAS  PubMed  Article  Google Scholar 

  44. Wilkinson MJ, et al. Percutaneous core needle biopsy in retroperitoneal sarcomas does not influence local recurrence or overall survival. Ann Surg Oncol. 2015;22(3):853–8.

    CAS  PubMed  Article  Google Scholar 

  45. Thway K, et al. Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors. Am J Surg Pathol. 2012;36(3):462–9.

    PubMed  Article  Google Scholar 

  46. Lee ATJ, et al. Clinical and Molecular Spectrum of Liposarcoma. J Clin Oncol. 2018;36(2):151–9.

    CAS  PubMed  Article  Google Scholar 

  47. Sirvent N, et al. Detection of MDM2-CDK4 amplification by fluorescence in situ hybridization in 200 paraffin-embedded tumor samples: utility in diagnosing adipocytic lesions and comparison with immunohistochemistry and real-time PCR. Am J Surg Pathol. 2007;31(10):1476–89.

    PubMed  Article  Google Scholar 

  48. Demicco EG, et al. Extensive survey of STAT6 expression in a large series of mesenchymal tumors. Am J Clin Pathol. 2015;143(5):672–82.

    CAS  PubMed  Article  Google Scholar 

  49. Trojani M, et al. Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer. 1984;33(1):37–42.

    CAS  PubMed  Article  Google Scholar 

  50. Lahat G, et al. Computed tomography scan-driven selection of treatment for retroperitoneal liposarcoma histologic subtypes. Cancer. 2009;115(5):1081–90.

    PubMed  Article  Google Scholar 

  51. Berger-Richardson D, et al. Early and Late Complications of Percutaneous Core Needle Biopsy of Retroperitoneal Tumors at Two Tertiary Sarcoma Centers. Ann Surg Oncol. 2019;26(13):4692–8.

    PubMed  Article  Google Scholar 

  52. Van Houdt WJ, et al. Needle tract seeding following core biopsies in retroperitoneal sarcoma. Eur J Surg Oncol. 2017;43(9):1740–5.

    PubMed  Article  Google Scholar 

  53. Gronchi A, et al. Outcome prediction in primary resected retroperitoneal soft tissue sarcoma: histology-specific overall survival and disease-free survival nomograms built on major sarcoma center data sets. J Clin Oncol. 2013;31(13):1649–55.

    PubMed  Article  Google Scholar 

  54. Martin Broto J, Le Cesne A, Reichardt P. The importance of treating by histological subtype in advanced soft tissue sarcoma. Future Oncol. 2017;13(1s):23–31.

    CAS  PubMed  Article  Google Scholar 

  55. Kirov KM, et al. Role of nutritional status in the early postoperative prognosis of patients operated for retroperitoneal liposarcoma (RLS): A single center experience. Eur J Surg Oncol. 2019;45(2):261–7.

    PubMed  Article  Google Scholar 

  56. Previtali P, et al. Malnutrition and Perioperative Nutritional Support in Retroperitoneal Sarcoma Patients: Results from a Prospective Study. Ann Surg Oncol. 2019;17(10):019–08121.

    Google Scholar 

  57. Bonvalot S, et al. Technical considerations in surgery for retroperitoneal sarcomas: position paper from E-Surge, a master class in sarcoma surgery, and EORTC-STBSG. Ann Surg Oncol. 2012;19(9):2981–91.

    PubMed  Article  Google Scholar 

  58. Gronchi A, et al. Variability in Patterns of Recurrence After Resection of Primary Retroperitoneal Sarcoma (RPS): A Report on 1007 Patients From the Multi-institutional Collaborative RPS Working Group. Ann Surg. 2016;263(5):1002–9.

    PubMed  Article  Google Scholar 

  59. Dingley B, Fiore M, Gronchi A. Personalizing surgical margins in retroperitoneal sarcomas: an update. Expert Rev Anticancer Ther. 2019;19(7):613–31.

    CAS  PubMed  Article  Google Scholar 

  60. Fairweather M, et al. Current principles of surgery for retroperitoneal sarcomas. J Surg Oncol. 2018;117(1):33–41.

    PubMed  Article  Google Scholar 

  61. Tseng WW, et al. Pancreaticoduodenectomy in the surgical management of primary retroperitoneal sarcoma. Eur J Surg Oncol. 2018;44(6):810–5.

    PubMed  Article  Google Scholar 

  62. Ng D, et al. Why were non-metastatic primary Retroperitoneal Sarcomas not resected? Connective Tissue Oncology Society Meeting Final Program 2018. Abstract ID 3042575.

  63. Andraz P, et al. Inoperable Primary Retroperitoneal Sarcomas: clinical characteristics and reasons against resection at a single referral institution. Ann Surg Oncol. 2020. Submitted.

  64. Tseng WW, et al. Locoregional disease patterns in well-differentiated and dedifferentiated retroperitoneal liposarcoma: implications for the extent of resection? Ann Surg Oncol. 2014;21(7):2136–43.

    PubMed  Article  Google Scholar 

  65. Lyu HG, et al. Enhanced recovery after surgery pathway in patients with soft tissue sarcoma. Br J Surg. 2020;107(12):1667–72.

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  66. Pisters PW, O’Sullivan B. Retroperitoneal sarcomas: combined modality treatment approaches. Curr Opin Oncol. 2002;14(4):400–5.

    PubMed  Article  Google Scholar 

  67. Gilbeau L, et al. Surgical resection and radiotherapy for primary retroperitoneal soft tissue sarcoma. Radiother Oncol. 2002;65(3):137–43.

    PubMed  Article  Google Scholar 

  68. Pawlik TM, et al. Long-term results of two prospective trials of preoperative external beam radiotherapy for localized intermediate- or high-grade retroperitoneal soft tissue sarcoma. Ann Surg Oncol. 2006;13(4):508–17.

    PubMed  Article  Google Scholar 

  69. Feng M, et al. Long-term outcomes after radiotherapy for retroperitoneal and deep truncal sarcoma. Int J Radiat Oncol Biol Phys. 2007;69(1):103–10.

    PubMed  Article  Google Scholar 

  70. Ballo MT, et al. Retroperitoneal soft tissue sarcoma: an analysis of radiation and surgical treatment. Int J Radiat Oncol Biol Phys. 2007;67(1):158–63.

    PubMed  Article  Google Scholar 

  71. Paryani NN, et al. Multimodality local therapy for retroperitoneal sarcoma. Int J Radiat Oncol Biol Phys. 2012;82(3):1128–34.

    PubMed  Article  Google Scholar 

  72. Smith MJ, et al. Combined management of retroperitoneal sarcoma with dose intensification radiotherapy and resection: long-term results of a prospective trial. Radiother Oncol. 2014;110(1):165–71.

    PubMed  Article  Google Scholar 

  73. Pisters PW, et al. Phase I trial of preoperative concurrent doxorubicin and radiation therapy, surgical resection, and intraoperative electron-beam radiation therapy for patients with localized retroperitoneal sarcoma. J Clin Oncol. 2003;21(16):3092–7.

    CAS  PubMed  Article  Google Scholar 

  74. Gronchi A, et al. Preoperative chemo-radiation therapy for localised retroperitoneal sarcoma: a phase I-II study from the Italian Sarcoma Group. Eur J Cancer. 2014;50(4):784–92.

    CAS  PubMed  Article  Google Scholar 

  75. Angele MK, et al. Effectiveness of regional hyperthermia with chemotherapy for high-risk retroperitoneal and abdominal soft-tissue sarcoma after complete surgical resection: a subgroup analysis of a randomized phase-III multicenter study. Ann Surg. 2014;260(5):749–54.

    PubMed  Article  Google Scholar 

  76. Issels RD, et al. Effect of Neoadjuvant Chemotherapy Plus Regional Hyperthermia on Long-term Outcomes Among Patients With Localized High-Risk Soft Tissue Sarcoma: The EORTC 62961-ESHO 95 Randomized Clinical Trial. JAMA Oncol. 2018;4(4):483–92.

    PubMed  PubMed Central  Article  Google Scholar 

  77. Radaelli S, et al. Emerging therapies for adult soft tissue sarcoma. Expert Rev Anticancer Ther. 2014;14(6):689–704.

    CAS  PubMed  Article  Google Scholar 

  78. Alektiar KM, et al. High-dose-rate intraoperative radiation therapy (HDR-IORT) for retroperitoneal sarcomas. Int J Radiat Oncol Biol Phys. 2000;47(1):157–63.

    CAS  PubMed  Article  Google Scholar 

  79. Gieschen HL, et al. Long-term results of intraoperative electron beam radiotherapy for primary and recurrent retroperitoneal soft tissue sarcoma. Int J Radiat Oncol Biol Phys. 2001;50(1):127–31.

    CAS  PubMed  Article  Google Scholar 

  80. Yoon SS, et al. Proton-beam, intensity-modulated, and/or intraoperative electron radiation therapy combined with aggressive anterior surgical resection for retroperitoneal sarcomas. Ann Surg Oncol. 2010;17(6):1515–29.

    PubMed  PubMed Central  Article  Google Scholar 

  81. McBride SM, et al. Locoregional recurrence after preoperative radiation therapy for retroperitoneal sarcoma: adverse impact of multifocal disease and potential implications of dose escalation. Ann Surg Oncol. 2013;20(7):2140–7.

    PubMed  Article  Google Scholar 

  82. Roeder F, et al. A clinical phase I/II trial to investigate preoperative dose-escalated intensity-modulated radiation therapy (IMRT) and intraoperative radiation therapy (IORT) in patients with retroperitoneal soft tissue sarcoma. BMC Cancer. 2012;12:287.

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  83. DeLaney TF, et al. Phase 1 trial of preoperative image guided intensity modulated proton radiation therapy with simultaneously integrated boost to the high risk margin for retroperitoneal sarcomas. Adv Radiat Oncol. 2017;2(1):85–93.

    PubMed  PubMed Central  Article  Google Scholar 

  84. Le Pechoux C, et al. Should adjuvant radiotherapy be administered in addition to front-line aggressive surgery (FAS) in patients with primary retroperitoneal sarcoma? Ann Oncol. 2013;24(3):832–7.

    PubMed  Article  Google Scholar 

  85. Haas RL, et al. Radiation therapy in retroperitoneal sarcoma management. J Surg Oncol. 2018;117(1):93–8.

    PubMed  Article  Google Scholar 

  86. Fairweather M, et al. Safety and efficacy of radiation dose delivered via iodine-125 brachytherapy mesh implantation for deep cavity sarcomas. Ann Surg Oncol. 2015;22(5):1455–63.

    PubMed  Article  Google Scholar 

  87. Dziewirski W, et al. Surgery combined with intraoperative brachytherapy in the treatment of retroperitoneal sarcomas. Ann Surg Oncol. 2006;13(2):245–52.

    PubMed  Article  Google Scholar 

  88. Raut C, et al. Why Primary Retroperitoneal Sarcoma (PRPS) Patients Undergoing Treatment At STRASS Institutions Did Not Enroll In STRASS: The STREXIT Study From EORTC STBSG And TARPSWG. Connective Tissue Oncology Society Meeting Final Program 2018. Abstract ID: 3042602.

  89. Pervaiz N, et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113(3):573–81.

    PubMed  Article  Google Scholar 

  90. Woll PJ, et al. Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicentre randomised controlled trial. Lancet Oncol. 2012;13(10):1045–54.

    CAS  PubMed  Article  Google Scholar 

  91. Gronchi A, et al. Histotype-tailored neoadjuvant chemotherapy versus standard chemotherapy in patients with high-risk soft-tissue sarcomas (ISG-STS 1001): an international, open-label, randomised, controlled, phase 3, multicentre trial. Lancet Oncol. 2017;18(6):812–22.

    CAS  PubMed  Article  Google Scholar 

  92. Canter RJ, et al. A synovial sarcoma-specific preoperative nomogram supports a survival benefit to ifosfamide-based chemotherapy and improves risk stratification for patients. Clin Cancer Res. 2008;14(24):8191–7.

    CAS  PubMed  PubMed Central  Article  Google Scholar 

  93. Haas RL, et al. Radiation Therapy as Sole Management for Solitary Fibrous Tumors (SFT): A Retrospective Study From the Global SFT Initiative in Collaboration With the Sarcoma Patients EuroNet. Int J Radiat Oncol Biol Phys. 2018;101(5):1226–33.

    PubMed  Article  Google Scholar 

  94. Gamboa AC, et al. Lung Surveillance Strategy for High-Grade Soft Tissue Sarcomas: Chest X-Ray or CT Scan? J Am Coll Surg. 2019;229(5):449–57.

    PubMed  PubMed Central  Article  Google Scholar 

  95. Yahya Zaidi M, Cardona K. Post-operative surveillance in soft tissue sarcoma: using tumor-specific recurrence patterns to direct approach. Chin Clin Oncol. 2018;7(4):03.

    Google Scholar 

  96. Wong P, et al. Long-Term Quality of Life of Retroperitoneal Sarcoma Patients Treated with Pre-Operative Radiotherapy and Surgery. Cureus. 2017;9(10):e1764.

    PubMed  PubMed Central  Google Scholar 

  97. Callegaro D, et al. Long-term morbidity after multivisceral resection for retroperitoneal sarcoma. Br J Surg. 2015;102(9):1079–87.

    CAS  PubMed  Article  Google Scholar 

  98. Kattan MW, Leung DH, Brennan MF. Postoperative nomogram for 12-year sarcoma-specific death. J Clin Oncol. 2002;20(3):791–6.

    PubMed  Article  Google Scholar 

  99. Anaya DA, et al. Postoperative nomogram for survival of patients with retroperitoneal sarcoma treated with curative intent. Ann Oncol. 2010;21(2):397–402.

    CAS  PubMed  Article  Google Scholar 

  100. Raut CP, et al. External validation of a multi-institutional retroperitoneal sarcoma nomogram. Cancer. 2016;122(9):1417–24.

    PubMed  Article  Google Scholar 

  101. Tan MC, et al. Histology-based Classification Predicts Pattern of Recurrence and Improves Risk Stratification in Primary Retroperitoneal Sarcoma. Ann Surg. 2016;263(3):593–600.

    PubMed  Article  Google Scholar 

  102. Dario Callegaro, C.P.R, Deanna Ng, Dirk C Strauss, Charles Honoré, Eberhard Stoeckle, MD Sylvie Bonvalot, Rick L Haas, Nikolaos Vassos, Lorenzo Conti, Rebecca Gladdy, Mark Fairweather, Winan van Houdt, Yvonne Schrage, Frits van Coevorden, Piotr Rutkowski, Rosalba Miceli, Alessandro Gronchi, Carol Swallow. Has the outcome of patients who undergo resection of primary retroperitoneal sarcoma changed over time? A time-trends study over the past 15 years. Ann Surg Oncol. 2020 (Submitted)

  103. Khan AR, et al. Quality and strength of evidence of the Infectious Diseases Society of America clinical practice guidelines. Clin Infect Dis. 2010;51(10):1147–56.

    PubMed  Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Consortia

Corresponding authors

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

Ethics declarations

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.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

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

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1245/s10434-021-09654-z