Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Nov;28(12):7873-7888.
doi: 10.1245/s10434-021-09654-z. Epub 2021 Apr 14.

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

Carol J Swallow  1   2 , Dirk C Strauss  3 , Sylvie Bonvalot  4 , Piotr Rutkowski  5 , Anant Desai  6 , Rebecca A Gladdy  7   8 , Ricardo Gonzalez  9 , David E Gyorki  10 , Mark Fairweather  11 , Winan J van Houdt  12 , Eberhard Stoeckle  13 , Jae Berm Park  14 , Markus Albertsmeier  15 , Carolyn Nessim  16 , Kenneth Cardona  17 , Marco Fiore  18 , Andrew Hayes  19 , Dimitri Tzanis  4 , Jacek Skoczylas  5 , Samuel J Ford  6 , Deanna Ng  7   8 , John E Mullinax  9 , Hayden Snow  10 , Rick L Haas  20 , Dario Callegaro  18 , Myles J Smith  19 , Toufik Bouhadiba  4 , Silvia Stacchiotti  21 , Robin L Jones  22 , Thomas DeLaney  23 , Christina L Roland  24 , Chandrajit P Raut  11 , Alessandro Gronchi  25 ; Transatlantic Australasian RPS Working Group (TARPSWG)
Affiliations

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

Carol J Swallow et al. Ann Surg Oncol. 2021 Nov.

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.

PubMed Disclaimer

Figures

FIG. 1
FIG. 1
CT-guided percutaneous core needle biopsy of a large lipomatous tumor arising from the left retroperitoneum. CT guidance facilitated targeted sampling of the more solid area within the mass (a white arrow shows the location of this area on diagnostic CT; b, core needle is indicated by the white arrow), yielding a diagnosis of low grade dedifferentiated liposarcoma. The optimal treatment strategy was then discussed at Multidisciplinary Sarcoma Tumor Board. CT computed tomography
FIG. 2
FIG. 2
Dedifferentiated liposarcoma in a 76-year-old man. (a) Contrast-enhanced computed tomography scan of a right retroperitoneal mass. (b) Surgical specimen. The tumor was removed en bloc with the right kidney and right colon. (c) Surgical field after tumor removal.
FIG. 3
FIG. 3
EORTC62092 RCT – STRASS. Preoperative radiotherapy versus resection alone, study design. RCT randomized controlled trial, CRT conformal radiotherapy, IMRT intensity-modulated radiation therapy, max maximum
FIG. 4
FIG. 4
EORTC1809 RCT – STRASS2. Preoperative chemotherapy versus resection alone, study design. RCT randomized controlled trial, DD LPS dedifferentiated liposarcoma, LMS leiomyosarcoma, Preop preoperative
See this image and copyright information in PMC

Comment in

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
    1. 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. - PMC - PubMed
    1. 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.
    1. 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.
    1. 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. - PMC - PubMed

LinkOut - more resources