Abstract
Background
Retroperitoneal soft tissue sarcomas (RPS) are rare tumors that include several well-defined histologic subtypes. Although surgery is the mainstay of curative therapy, no universally accepted recommendations concerning the best management have been developed to date. Optimization of the initial approach is critical for maximizing patient outcomes.
Methods
An RPS Trans-Atlantic Working Group was established in 2013. The primary aim was to evaluate the current evidence critically and to develop a consensus document on the approach to this difficult disease. The outcome applies to primary RPS that is nonvisceral in origin. The evaluation included sarcomas of major veins (inferior vena cava, renal vein, ovarian/testicular vein), undifferentiated pleomorphic sarcoma of the psoas, and ureteric leiomyosarcoma (LMS). It excluded desmoid, lipoma and angiomyolipoma, gastrointestinal stromal tumors, visceral sarcomas such as those arising from the gut or its mesentery, uterine LMS, prostatic sarcoma, paratesticular/spermatic cord sarcoma, Ewing’s sarcoma, alveolar/embryonal rhabdomyosarcoma, primitive peripheral neuro-ectodermal tumor, sarcoma arising from teratoma, carcinosarcoma, sarcomatoid carcinoma, clear cell sarcoma, radiation-induced sarcoma, paraganglioma, and malignant pheochromocytoma.
Results
Management of RPS was evaluated from diagnosis to follow-up, and a level of evidence was attributed to each statement. 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 statements included in this article.
Conclusions
International collaboration is critical for adding to the current knowledge. A prospective registry will be set up.
Similar content being viewed by others
References
Cormier JN, Gronchi A, Pollock RE. Soft tissue sarcomas. In: Brunicardi F, Andersen D, Billiar T, Dunn D, Hunter J, Matthews J, Pollock RE (eds) Schwartz’s principles of surgery. New York: McGraw-Hill; 2014.
Lewis JJ, Leung D, Woodruff JM. Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg. 1998;228:355–65.
Stoeckle E, Coindre JM, Bonvalot S, 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;92:359–68.
Gronchi A, Casali PG, Fiore M, et al. Retroperitoneal soft tissue sarcomas: patterns of recurrence in 167 patients treated at a single institution. Cancer. 2004;100:2448–55.
Hassan I, Park SZ, Donohue JH, et al. Operative management of primary retroperitoneal sarcomas: a reappraisal of an institutional experience. Ann Surg. 2004; 239:244–50.
van Dalen T, Plooij JM, van Coevorden F, et al. Long-term prognosis of primary retroperitoneal soft tissue sarcoma. Eur J Surg Oncol. 2007;33:234–8.
Lehnert T, Cardona S, Hinz U, et al. Primary and locally recurrent retroperitoneal soft-tissue sarcoma: local control and survival. Eur J Surg Oncol. 2009;35:986–93.
Anaya DA, Lahat G, Liu J, et al. Multifocality in retroperitoneal sarcoma: a prognostic factor critical to surgical decision making. Ann Surg. 2009;249:137–42.
Strauss DC, Hayes AJ, Thway K, et al. Surgical management of primary retroperitoneal sarcoma. Br J Surg. 2010;101:520–3.
Bonvalot S, Rivoire M, Castaing M, et al. Primary retroperitoneal sarcomas: a multivariate analysis of surgical factors associated with local control. J Clin Oncol. 2009;27:31–7.
Gronchi A, Lo Vullo S, Fiore M, et al. Aggressive surgical policies in a retrospectively reviewed single-institution case series of retroperitoneal soft tissue sarcoma patients. J Clin Oncol. 2009;27:24–30.
Bonvalot S, Miceli R, Berselli M, 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;17:1507–14.
Gronchi A, Miceli R, Colombo C, et al. Frontline extended surgery is associated with improved survival in retroperitoneal low-intermediate grade soft tissue sarcomas. Ann Oncol. 2012;23:1067–73.
Singer S, Antonescu CR, Riedel E, et al. Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma. Ann Surg. 2003;238:358–70.
Dalal KM, Kattan MW, Antonescu CR, et al. Subtype-specific prognostic nomogram for patients with primary liposarcoma of the retroperitoneum, extremity, or trunk. Ann Surg. 2006;244:381–91.
Canter RJ, Qin LX, Ferrone CR, et al. Why do patients with low-grade soft tissue sarcoma die? Ann Surg Oncol. 2008;15:3550–60.
http://clinicaltrials.gov/ct2/show/NCT01344018. Accessed 14 Feb 2014.
Pervaiz N, Colterjohn N, Farrokhyar F, et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113:573–81.
Woll PJ, Reichardt P, Le Cesne A, et al. Adjuvant chemotherapy with doxorubicin, ifosfamide, and lenograstim for resected soft-tissue sarcoma (EORTC 62931): a multicentre randomised controlled trial. Lancet Oncol. 2012;13:1045–54.
Gronchi A, Pollock RE. Surgery in retroperitoneal soft tissue sarcoma: a call for a consensus between Europe and North America. Ann Surg Oncol. 2011;18: 2107–10.
Gronchi A, Pollock RE. Quality of local treatment or biology of the tumor: which are the trump cards for loco-regional control of retroperitoneal sarcoma? Ann Surg Oncol. 2013;20:2111–13.
Dykewicz CA. Summary of the guidelines for preventing opportunistic infections among hematopoietic stem cell transplant recipients. Clin Infect Dis. 2001;33:139–44.
Gutierrez JC, Perez EA, et al. Should soft tissue sarcomas be treated at high-volume centers? An analysis of 4,205 patients. Ann Surg. 2007;245:952–8.
Heudel PE, Cousin P, Lurkin A, et al. Territorial inequalities in management and conformity to clinical guidelines for sarcoma patients: an exhaustive population-based cohort analysis in the Rhône-Alpes region. Int J Clin Oncol. 2014;19:744–52.
von Mehren M, Benjamin RS, Bui MM, et al. Soft tissue sarcoma, version 2.2012: featured updates to the NCCN guidelines. J Natl Compr Cancer Netw. 2012;10:951–60.
ESMO/European Sarcoma Network Working Group. Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012;23(Suppl 7):vii92–9.
Miah AB, Hannay J, Benson C, et al. Optimal management of primary retroperitoneal sarcoma: an update. Expert Rev Anticancer Ther. 2014;14:565–79.
Strauss DC, Hayes AJ, Thomas JM. Preoperative core needle biopsy is safe and does not affect oncological outcome in retroperitoneal sarcoma. Poster HH, p. 112, CTOS 17th Annual Meeting, November 2012.
Lahat G, Madewell JE, Anaya DA, et al. Computed tomography scan-driven selection of treatment for retroperitoneal liposarcoma histologic subtypes. Cancer. 2009;115:1081–90.
Gronchi A, Miceli R, Shurell E, 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;31:1649–55.
Toulmonde M, Bonvalot S, Méeus P, 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;25:735–42.
Bonvalot S, Raut CP, Pollock RE, 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;19:2981–91.
Pisters PW, O’Sullivan B. Retroperitoneal sarcomas: combined modality treatment approaches. Curr Opin Oncol. 2002;14:400–5.
Gilbeau L, Kantor G, Stoeckle E, et al. Surgical resection and radiotherapy for primary retroperitoneal soft tissue sarcoma. Radiother Oncol. 2002;65:137–43.
Pawlik TM, Pisters PW, Mikula L, 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;13:508–17.
Feng M, Murphy J, Griffith KA, et al. Long-term outcomes after radiotherapy for retroperitoneal and deep truncal sarcoma. Int J Radiat Oncol Biol Phys. 2007;69:103–10.
Ballo MT, Zagars GK, Pollock RE, et al. Retroperitoneal soft tissue sarcoma: an analysis of radiation and surgical treatment. Int J Radiat Oncol Biol Phys. 2007;67:158–63.
Paryani NN, Zlotecki RA, Swanson EL, et al. Multimodality local therapy for retroperitoneal sarcoma. Int J Radiat Oncol Biol Phys. 2012;82:1128–34.
Smith MJ, Ridgway PF, Catton CN, et al. Combined management of retroperitoneal sarcoma with dose intensification radiotherapy and resection: long-term results of a prospective trial. Radiother Oncol. 2014. doi:10.1016/j.radonc.2013.10.041.
Pisters PW, Ballo MT, Fenstermacher MJ, 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;21:3092–7.
Gronchi A, De Paoli A, Dani C, et al. Preoperative chemo-radiation therapy for localized retroperitoneal sarcoma: a phase I-II study from the Italian Sarcoma Group. Eur J Cancer. 2014;50:784–92.
Angele MK, Albertsmeier M, Prix NJ, et al. Effectiveness of regional hyperthermia with chemotherapy for high-risk retroperitoneal and abdominal soft-tissue sarcoma after complete surgical resection: a randomized phase-III multicenter study. Ann Surg. (in press).
Canter RJ, Qin LX, Maki RG, 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;14:8191–7.
Saynak M, Bayir-Angin G, Kocak Z, et al. Recurrent solitary fibrous tumor of the pleura: significant response to radiotherapy. Med Oncol. 2010;27:45–8.
Radaelli S, Stacchiotti S, Casali PG, et al. Emerging therapies for adult soft tissue sarcoma. Expert Rev Anticancer Ther. 2014;14:689–704.
Alektiar KM, Hu K, Anderson L, et al. High-dose-rate intraoperative radiation therapy (HDR-IORT) for retroperitoneal sarcomas. Int J Radiat Oncol Biol Phys. 2000;47:157–63.
Gieschen HL, Spiro IJ, Suit HD, 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;50:127–31.
Yoon SS, Chen YL, Kirsch DG, 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;17:1515–29.
McBride SM, Raut CP, Lapidus M, et al. Loco-regional recurrence after pre-operative radiation therapy for retroperitoneal sarcoma: adverse impact of multifocal disease and potential implications of dose escalation. Ann Surg Oncol. 2013;20:2140–7.
Roeder F, Schulz-Ertner D, Nikoghosyan AV, 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.
Le Péchoux C, Musat E, Baey 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;24:832–7.
Paryani NN1, Zlotecki RA, Swanson EL, et al. Multimodality local therapy for retroperitoneal sarcoma. Int J Radiat Oncol Biol Phys. 2012;82:1128–34.
Mohindra P, Neuman HB, Kozak KR. The role of radiation in retroperitoneal sarcomas. Curr Treat Options Oncol. 2013;14:425–41.
Smith MJ, Ridgway PF, Catton CN, et al. Combined management of retroperitoneal sarcoma with dose intensification radiotherapy and resection: long-term results of a prospective trial. Radiother Oncol. 2014;110:165–71.
Dziewirski W1, Rutkowski P, Nowecki ZI, et al. Surgery combined with intraoperative brachytherapy in the treatment of retroperitoneal sarcomas. Ann Surg Oncol. 2006;13:245–52.
Conflict of interest
There are no conflicts of interest.
Author information
Authors and Affiliations
Consortia
Additional information
Correspondence to: Alessandro Gronchi
Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy, e-mail: alessandro.gronchi@istitutotumori.mi.it
Rights and permissions
About this article
Cite this article
Trans-Atlantic RPS Working Group. Management of Primary Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach From the Trans-Atlantic RPS Working Group. Ann Surg Oncol 22, 256–263 (2015). https://doi.org/10.1245/s10434-014-3965-2
Received:
Published:
Issue Date:
DOI: https://doi.org/10.1245/s10434-014-3965-2