Retroperitoneal soft tissue sarcomas (RPS) are rare tumors. Surgery is the mainstay of curative therapy, but local recurrence is common. No recommendations concerning the best management of recurring disease have been developed so far. Although every effort should be made to optimize the initial approach, recommendations to treat recurring RPS will be helpful to maximize disease control at recurrence.
An RPS transatlantic working group was established in 2013. The goals of the group were to share institutional experiences, build large multi-institutional case series, and develop consensus documents on the approach to this difficult disease. The outcome of this document applies to recurrent RPS that is nonvisceral in origin. Included are sarcomas of major veins, undifferentiated pleomorphic sarcoma of psoas, ureteric leiomyosarcoma (LMS). Excluded are desmoids-type fibromatosis, angiomyolipoma, gastrointestinal stromal tumors, sarcomas arising from the gut or its mesentery, uterine LMS, prostatic sarcoma, paratesticular/spermatic cord sarcoma, Ewing sarcoma, alveolar/embryonal rhabdomyosarcoma, sarcoma arising from teratoma, carcinosarcoma, sarcomatoid carcinoma, clear cell sarcoma, radiation-induced sarcoma, paraganglioma, and malignant pheochromocytoma.
Recurrent RPS management was evaluated from diagnosis to follow-up. It is a rare and complex malignancy that 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, but some patients may experience prolonged disease control also at recurrence, when the approach is optimized and follows the recommendations contained herein.
International collaboration is critical for adding to the present knowledge. A transatlantic prospective registry has been established.
This is a preview of subscription content, access via your institution.
Buy single article
Instant access to the full article PDF.
Tax calculation will be finalised during checkout.
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. McGraw Hill, New York; 2015:1465–94.
Gronchi A, Strauss DC, Miceli R, 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;263:1002–9.
Gronchi A, Miceli R, Allard MA, et al. Personalizing the approach to retroperitoneal soft tissue sarcoma: histology-specific patterns of failure and post-relapse outcome after primary extended resection. Ann Surg Oncol 2015;22(5):1447–54.
Toulmonde M, Bonvalot S, Meeus 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, 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.
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.
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.
Strauss DC, Hayes AJ, Thway K, et al. Surgical management of primary retroperitoneal sarcoma. Br J Surg 2010;97:698–706.
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.
Lewis JJ, Leung D, Woodruff JM, et al. Retroperitoneal soft-tissue sarcoma: analysis of 500 patients treated and followed at a single institution. Ann Surg 1998;228:355–365.
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(4):1067–73.
Management of primary retroperitoneal sarcoma (RPS) in the adult: a consensus approach from the Trans-Atlantic RPS Working Group. Ann Surg Oncol 2015;22:256–63.
Heslin MJ, Lewis JJ, Nadler E, et al. Prognostic factors associated with long-term survival for retroperitoneal sarcoma: implications for management. J Clin Oncol 1997;15:2832–9.
Canter RJ, Qin LX, Ferrone CR, et al. Why do patients with low-grade soft tissue sarcoma die? Ann Surg Oncol 2008;15(12):3550–60.
Tan MC, Brennan MF, Kuk D, et al. Histology-based classification predicts pattern of recurrence and improves risk stratification in primary retroperitoneal sarcoma. Ann Surg 2016;263:593–600.
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; discussion 370–1.
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.
Anaya DA, Lahat G, Wang X, et al. Postoperative nomogram for survival of patients with retroperitoneal sarcoma treated with curative intent. Ann Oncol 2010;21:397–402.
Gyorki DE, Brennan MF. Management of recurrent retroperitoneal sarcoma. J Surg Oncol. 2014;109:53-9.
Dykewicz CA. Summary of the guidelines for preventing opportunistic infections among hematopoietic stem cell transplant recipients. Clin Infect Dis 2001;33:139–44.
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.
Morosi C, Stacchiotti S, Marchianò A, et al. Correlation between radiological assessment and histopathological diagnosis in retroperitoneal tumors: analysis of 291 consecutive patients at a tertiary reference sarcoma center. Eur J Surg Oncol 2014. 10.1016/j.ejso.2014.10.005.
Tzeng CW, Smith JK, Heslin MJ. Soft tissue sarcoma: preoperative and postoperative imaging for staging. Surg Oncol Clin N Am 2007;16:389–402.
Tseng WW, Madewell JE, Wei W, et al. Locoregional disease patterns in well-differentiated and dedifferentiated retroperitoneal liposarcoma: implications for the extent of resection? Ann Surg Oncol 2014;21:2136–43.
Shiraev T, Pasricha SS, Choong P, et al. Retroperitoneal sarcomas: a review of disease spectrum, radiological features, characterisation and management. J Med Imaging Radiat Oncol 2013;57:687–700.
Niccoli-Asabella A, Altini C, Notaristefano A, et al. A retrospective study comparing contrast-enhanced computed tomography with 18F-FDG-PET/CT in the early follow-up of patients with retroperitoneal sarcomas. Nucl Med Commun 2013;34:32–9.
Alford S, Choong P, Chander S, et al. Value of PET scan in patients with retroperitoneal sarcoma treated with preoperative radiotherapy. Eur J Surg Oncol 2012;38:176–80.
Grobmyer SR, Wilson JP, Apel B, et al. Recurrent retroperitoneal sarcoma: impact of biology and therapy on outcomes. J Am Coll Surg 2010;210:602–8, 608–10.
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 datasets. J Clin Oncol 2013;31:1649–55.
van Dalen T, Hoekstra HJ, van Geel AN, et al. Locoregional recurrence of retroperitoneal soft tissue sarcoma: second chance of cure for selected patients. Eur J Surg Oncol 2001;27:564–8.
Neuhaus SJ, Barry P, Clark MA, et al. Surgical management of primary and recurrent retroperitoneal liposarcoma. Br J Surg 2005;92:246–52.
Lochan R, French JJ, Manas DM. Surgery for retroperitoneal soft tissue sarcomas: aggressive re-resection of recurrent disease is possible. Ann R Coll Surg Engl 2011;93:39–43.
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.
Park JO, Qin LX, Prete FP, et al. Predicting outcome by growth rate of locally recurrent retroperitoneal liposarcoma: the one centimeter per month rule. Ann Surg 2009;250:977–82.
Yang JY, Kong SH, Ahn HS, et al. Prognostic factors for reoperation of recurrent retroperitoneal sarcoma: the role of clinicopathological factors other than histologic grade. J Surg Oncol 2015;111:165–72.
Toulmonde M, Bonvalot S, Ray-Coquard I, et al. Retroperitoneal sarcomas: patterns of care in advanced stages, prognostic factors and focus on main histological subtypes: a multicenter analysis of the French Sarcoma Group. Ann Oncol 2014;25:730–4.
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.
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.
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.
Tseng WH, Martinez SR, Do L, et al. Lack of survival benefit following adjuvant radiation in patients with retroperitoneal sarcoma: a SEER analysis. J Surg Res 2011;168:e173–80.
Roeder F, Ulrich A, Habl G, et al. 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: interim analysis. BMC Cancer 2014;14:617-2407-14-617.
Hayes-Jordan A, Green HL, Lin H, et al. Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor. Ann Surg Oncol 2014;21:220–4.
Hayes-Jordan A. Cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy in DSRCT: progress and pitfalls. Curr Oncol Rep 2015;17:38-015-0461-1.
Sugarbaker P, Ihemelandu C, Bijelic L. Cytoreductive surgery and HIPEC as a treatment option for laparoscopic resection of uterine leiomyosarcoma with morcellation: early results. Ann Surg Oncol 2015.
Baratti D, Pennacchioli E, Kusamura S, et al. Peritoneal sarcomatosis: is there a subset of patients who may benefit from cytoreductive surgery and hyperthermic intraperitoneal chemotherapy? Ann Surg Oncol 2010;17:3220–8.
Bonvalot S, Cavalcanti A, Le Pechoux C, et al. Randomized trial of cytoreduction followed by intraperitoneal chemotherapy versus cytoreduction alone in patients with peritoneal sarcomatosis. Eur J Surg Oncol 2005;31:917–23.
Rossi CR, Deraco M, De Simone M, et al. Hyperthermic intraperitoneal intraoperative chemotherapy after cytoreductive surgery for the treatment of abdominal sarcomatosis: clinical outcome and prognostic factors in 60 consecutive patients. Cancer 2004;100:1943–50.
Angele MK, Albertsmeier M, Prix NJ, Hohenberger P, 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.
Sanfilippo R, Bertulli R, Marrari A, et al. High-dose continuous-infusion ifosfamide in advanced well-differentiated/dedifferentiated liposarcoma. Clin Sarcoma Res 2014;4(1):16.
Martin-Liberal J, Alam S, Constantinidou A, et al. Clinical activity and tolerability of a 14-day infusional Ifosfamide schedule in soft-tissue sarcoma. Sarcoma. 2013;2013:868973. doi:10.1155/2013/868973.
Radaelli S, Stacchiotti S, Casali PG, et al. Emerging therapies for adult soft tissue sarcoma. Expert Rev Anticancer Ther 2014;14.6:689–704.
Correspondence to: Alessandro Gronchi, MD
Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy, e-mail: email@example.com
Correspondence to: Carol J. Swallow, MD, PhD
Department of Surgical Oncology, Mount Sinai Hospital and Princess Margaret Cancer Center, and Department of Surgery University of Toronto, Toronto, Canada, e-mail: firstname.lastname@example.org
An erratum to this article is available at http://dx.doi.org/10.1245/s10434-017-5889-0.
About this article
Cite this article
Trans-Atlantic RPS Working Group. Management of Recurrent Retroperitoneal Sarcoma (RPS) in the Adult: A Consensus Approach from the Trans-Atlantic RPS Working Group. Ann Surg Oncol 23, 3531–3540 (2016). https://doi.org/10.1245/s10434-016-5336-7
- Synovial Sarcoma
- Malignant Peripheral Nerve Sheath Tumor
- Clear Cell Sarcoma
- Netherlands Cancer Institute