Highlights
- •Histologic subtype of the retroperitoneal sarcoma determines the pattern of recurrence
- •Resection of local or oligometastatic recurrence improves survival but patient selection is crucial
- •Oncological outcomes of recurrence are inferior to those of primary disease
- •Six-year overall survival rates are 54% and 71% develop a second recurrence
- •Treatment morbidity and impact on patient's quality of life must be factored into treatment decisions
Abstract
Recurrent retroperitoneal sarcomas are rare, with patterns of recurrence determined
by the histologic subtype. A range of patient characteristics and treatment profiles
combined with a myriad of presentations and clinical courses of recurrences make this
diverse entity challenging to manage. Although surgical resection improves survival
in select patients, the oncological outcomes are inferior to that of primary retroperitoneal
sarcomas. Management options for unresectable disease include local ablative therapy,
radiation and systemic therapy, with palliative surgery indicated occasionally. Attempts
at disease control must be balanced with potential morbidity and impact on the patient's
quality of life. This review aims to offer insights into the current understanding
of recurrent retroperitoneal sarcomas and provide some guidance on management.
Keywords
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References
- Retroperitoneal sarcoma: a population-based analysis of epidemiology, surgery, and radiotherapy.Cancer. 2006; 106: 1610-1616
- Burden and centralised treatment in Europe of rare tumours: results of RARECAREnet—a population-based study.Lancet Oncol. 2017; 18: 1022-1039
- WCoTE Board Soft tissue and bone tumours. International Agency for Research on Cancer (IARC), Lyon (France)2020
- Has the outcome for patients who undergo resection of primary retroperitoneal sarcoma changed over time? A study of time trends during the past 15 years.Ann Surg Oncol. 2021; 28: 1700-1709
- Primary retroperitoneal sarcomas: a multivariate analysis of surgical factors associated with local control.J Clin Oncol. 2009; 27: 31-37
- Aggressive surgical policies in a retrospectively reviewed single-institution case series of retroperitoneal soft tissue sarcoma patients.J Clin Oncol. 2009; 27: 24-30
- 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: 2981-2991
- Frontline extended surgery is associated with improved survival in retroperitoneal low- to intermediate-grade soft tissue sarcomas.Ann Oncol. 2012; 23: 1067-1073
- 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: 1002-1009
- Histology-based classification predicts pattern of recurrence and improves risk stratification in primary retroperitoneal sarcoma.Ann Surg. 2016; 263: 593-600
- Post-relapse outcomes after primary extended resection of retroperitoneal sarcoma: a report from the Trans-Atlantic RPS Working Group.Cancer, 2017
- Patterns of recurrence and survival probability after second recurrence of retroperitoneal sarcoma: a study from TARPSWG.Cancer. 2020; 126: 4917-4925
- Management of recurrent retroperitoneal sarcoma (RPS) in the Adult: a consensus approach from the trans-Atlantic RPS working group.Ann Surg Oncol. 2016; 23: 3531-3540
- Management of locoregional recurrence after radical resection of a primary nonmetastatic retroperitoneal soft tissue sarcoma: the Gustave Roussy experience.J Surg Oncol. 2018; 118: 1318-1325
- Predicting outcome by growth rate of locally recurrent retroperitoneal liposarcoma: the one centimeter per month rule.Ann Surg. 2009; 250: 977-982
- Completely resected recurrent soft tissue sarcoma: primary anatomic site governs outcomes.J Am Coll Surg. 2002; 194: 436-447
- Personalizing the approach to retroperitoneal soft tissue sarcoma: histology-specific patterns of failure and postrelapse outcome after primary extended resection.Ann Surg Oncol. 2015; 22: 1447-1454
- Locoregional recurrence of retroperitoneal soft tissue sarcoma: second chance of cure for selected patients.Eur J Surg Oncol. 2001; 27: 564-568
- Outcome following resection of retroperitoneal sarcoma.Br J Surg. 2015; 102: 1698-1709
- Postoperative morbidity after resection of recurrent retroperitoneal sarcoma: a report from the transatlantic Australasian RPS working group (TARPSWG).Ann Surg Oncol. 2021; 28: 2705-2714
- Multifocality in retroperitoneal sarcoma: a prognostic factor critical to surgical decision-making.Ann Surg. 2009; 249: 137-142
- Locoregional disease patterns in well-differentiated and dedifferentiated retroperitoneal liposarcoma: implications for the extent of resection?.Ann Surg Oncol. 2014; 21: 2136-2143
- Resectable well-differentiated versus dedifferentiated liposarcomas: two different diseases possibly requiring different treatment approaches.Ann Surg Oncol. 2008; 15: 1585-1593
- Analysis of differentiation changes and outcomes at time of first recurrence of retroperitoneal liposarcoma by transatlantic Australasian retroperitoneal sarcoma working group (TARPSWG).Ann Surg Oncol. 2021; 28: 7854-7863
- Histologic subtype and margin of resection predict pattern of recurrence and survival for retroperitoneal liposarcoma.Ann Surg. 2003; 238 (discussion 70-1): 358-370
- Recurrent retroperitoneal sarcoma: impact of biology and therapy on outcomes.J Am Coll Surg. 2010; 210 (602-8): 8-10
- Imaging in retroperitoneal soft tissue sarcoma.J Surg Oncol. 2018; 117: 25-32
- Prediction of histologic subtype and FNCLCC grade by SUVmax measured on (18)F-FDG PET/CT in patients with retroperitoneal liposarcoma.Contrast Media Mol Imaging. 2021; (2021)7191363
- The role of 18F-FDG PET/CT in retroperitoneal sarcomas—a multicenter retrospective study.J Surg Oncol. 2021; 123: 1081-1087
- Utilization of positron emission tomography in the management of patients with sarcoma.Curr Opin Oncol. 2009; 21: 345-351
- Postoperative morbidity after radical resection of primary retroperitoneal sarcoma: a report from the transatlantic RPS working group.Ann Surg. 2018; 267: 959-964
- Results of resection for recurrent or residual retroperitoneal sarcoma after failed primary treatment.Ann Surg Oncol. 2017; 24: 211-218
- Surgery for retroperitoneal soft tissue sarcomas: aggressive re-resection of recurrent disease is possible.Ann R Coll Surg Engl. 2011; 93: 39-43
- Long-term follow-up and post-relapse outcome of patients with localized retroperitoneal sarcoma treated in the Italian sarcoma group-soft tissue sarcoma (ISG-STS) protocol 0303.Ann Surg Oncol. 2017; 24: 3872-3879
- Salvage surgery for recurrent retroperitoneal well-differentiated liposarcoma: early reoperation may not provide benefit.Ann Surg Oncol. 2018; 25: 2193-2200
- Prognostic factors for reoperation of recurrent retroperitoneal sarcoma: the role of clinicopathological factors other than histologic grade.J Surg Oncol. 2015; 111: 165-172
- Recurrence patterns after resection of retroperitoneal sarcomas: an eight-institution study from the US Sarcoma Collaborative.J Surg Oncol. 2019; 120: 340-347
- Predicting survival in patients undergoing resection for locally recurrent retroperitoneal sarcoma: a study and novel nomogram from TARPSWG.Clin Cancer Res. 2019; 25(8): 2664-2671
- Recurrence patterns of retroperitoneal leiomyosarcoma and impact of salvage surgery.J Surg Oncol. 2017; 116: 313-319
- Postoperative nomogram for survival of patients with retroperitoneal sarcoma treated with curative intent.Ann Oncol. 2010; 21: 397-402
- Longitudinal prognostication in retroperitoneal sarcoma survivors: development and external validation of two dynamic nomograms.Eur J Cancer. 2021; 157: 291-300
- 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
- Combined management of retroperitoneal sarcoma with dose intensification radiotherapy and resection: long-term results of a prospective trial.Radiother Oncol. 2014; 110: 165-171
- Lack of survival benefit following adjuvant radiation in patients with retroperitoneal sarcoma: a SEER analysis.J Surg Res. 2011; 168: e173-e180
- Analysis of perioperative radiation therapy in the surgical treatment of primary and recurrent retroperitoneal sarcoma.J Surg Oncol. 2015; 112: 352-358
- 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: 1366-1377
- Impact of chemotherapy on survival in surgically resected retroperitoneal sarcoma.Eur J Surg Oncol. 2015; 41: 1386-1392
- Role of chemotherapy in dedifferentiated liposarcoma of the retroperitoneum: defining the benefit and challenges of the standard.Sci Rep. 2017; 7
- Doxorubicin plus dacarbazine (DoDa), doxorubicin plus ifosfamide (DI) or doxorubicin alone (Do) as first line treatment for advanced leiomyosarcoma (LMS).A retrospective study from the EORTC Soft Tissue and Bone Sarcoma Group (STBSG). 2018; 3611574
- Randomized trial of cytoreduction followed by intraperitoneal chemotherapy versus cytoreduction alone in patients with peritoneal sarcomatosis.Eur J Surg Oncol. 2005; 31: 917-923
- Aggressive locoregional management of recurrent peritoneal sarcomatosis.J Surg Oncol. 2013; 107: 329-334
- Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for the treatment of peritoneal sarcomatosis.Ann Surg Oncol. 2012; 19: 1410-1415
- Peritoneal sarcomatosis: is there a subset of patients who may benefit from cytoreductive surgery and hyperthermic intraperitoneal chemotherapy?.Ann Surg Oncol. 2010; 17: 3220-3228
- Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) in peritoneal sarcomatosis-A systematic review and meta-analysis.Eur J Surg Oncol. 2022; 48(3): 640-648
- Is there a role for incomplete resection in the management of retroperitoneal liposarcomas?.J Am Coll Surg. 2001; 193: 373-379
- 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: 730-734
- Effectiveness of palliative procedures for intra-abdominal sarcomas.Ann Surg Oncol. 2005; 12: 1084-1089
- Outcomes of palliative-intent surgery in retroperitoneal sarcoma-Results from the US Sarcoma Collaborative.J Surg Oncol. 2020; 121: 1140-1147
- Conformal radiotherapy for unresectable retroperitoneal soft tissue sarcoma.Int J Radiat Oncol Biol Phys. 1992; 22: 333-341
- Definitive radiotherapy in the management of non-resectable or residual retroperitoneal sarcomas: institutional cohort analysis and systematic review.Cancer Control. 2021; 281073274820983028
- CT-guided (125)I seed implantation for inoperable retroperitoneal sarcoma: a technique for delivery of local tumor brachytherapy.Exp Ther Med. 2016; 12: 3843-3850
- Percutaneous radiofrequency ablation for treatment of recurrent retroperitoneal liposarcoma.Cardiovasc Intervent Radiol. 2008; 31: 213-216
- Percutaneous computed tomography-guided cryoablation for recurrent retroperitoneal soft tissue sarcoma: a study of safety and efficacy.Oncotarget. 2016; 7: 42639-42649
- Radiofrequency ablation of cancer.Cardiovasc Intervent Radiol. 2004; 27: 427-434
- Radiofrequency ablation for the treatment of recurrent bone and soft-tissue sarcomas in non-surgical candidates.Int J Clin Oncol. 2014; 19: 955-962
- Management of metastatic retroperitoneal sarcoma: a consensus approach from the trans-Atlantic retroperitoneal sarcoma working group (TARPSWG).Ann Oncol. 2018; 29: 857-871
- Defining oligometastatic disease from a radiation oncology perspective: an ESTRO-ASTRO consensus document.Radiother Oncol. 2020; 148: 157-166
- Surgical and non-surgical management of repeat pulmonary metastasis from sarcoma following first pulmonary metastasectomy.Surg Today. 2016; 46: 1296-1300
- A simple prognostic benefit scoring system for sarcoma patients with pulmonary metastases: sarcoma lung metastasis score.Ann Surg Oncol. 2020; 28: 3884-3890
- A novel preoperative risk score to guide patient selection for resection of soft tissue sarcoma lung metastases: an analysis from the United States Sarcoma Collaborative.J Surg Oncol. 2021; 124: 1477-1484
- Outcomes and clinical predictors of improved survival in a patients undergoing pulmonary metastasectomy for sarcoma.J Surg Oncol. 2015; 112: 103-106
- Liver resection for sarcoma metastases: a systematic review and experience from two European centres.Eur J Surg Oncol. 2020; 46: 1807-1813
- Multi-institutional phase I/II trial of stereotactic body radiation therapy for liver metastases.J Clin Oncol. 2009; 27: 1572-1578
- Multi-institutional analysis of stereotactic body radiotherapy for sarcoma pulmonary metastases: high rates of local control with favorable toxicity.J Surg Oncol. 2020; 122(5): 877-883
- Treatment of sarcoma lung metastases with stereotactic body radiotherapy.Sarcoma. 2018; (2018): 1-6
- Radiofrequency ablation is a feasible therapeutic option in the multi modality management of sarcoma.Eur J Surg Oncol. 2010; 36: 477-482
- Transarterial chemoembolization in soft-tissue sarcoma metastases to the liver - the use of imaging biomarkers as predictors of patient survival.Eur J Radiol. 2015; 84: 424-430
- Systemic therapy in retroperitoneal sarcoma management.J Surg Oncol. 2018; 117: 87-92
- Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up( ).Ann Oncol. 2021; 32: 1348-1365
- Doxorubicin alone versus intensified doxorubicin plus ifosfamide for first-line treatment of advanced or metastatic soft-tissue sarcoma: a randomised controlled phase 3 trial.Lancet Oncol. 2014; 15: 415-423
- Trabectedin for patients with advanced soft tissue sarcoma: a non-interventional, retrospective, multicenter study of the Italian sarcoma group.Cancers. 2021; 13
- Eribulin versus dacarbazine in previously treated patients with advanced liposarcoma or leiomyosarcoma: a randomised, open-label, multicentre, phase 3 trial.Lancet. 2016; 387: 1629-1637
- Randomized phase II study of gemcitabine and docetaxel compared with gemcitabine alone in patients with metastatic soft tissue sarcomas: results of sarcoma alliance for research through collaboration study 002 [corrected].J Clin Oncol. 2007; 25: 2755-2763
- Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial.Lancet. 2012; 379: 1879-1886
- Treatment of milademetan versus trabectedin in patient with dedifferentiated liposarcoma.([Available from:)
- Progression-free survival among patients with well-differentiated or dedifferentiated liposarcoma treated with CDK4 inhibitor Palbociclib: a phase 2 clinical trial.JAMA Oncol. 2016; 2: 937-940
- Nivolumab with or without ipilimumab treatment for metastatic sarcoma (Alliance A091401): two open-label, non-comparative, randomised, phase 2 trials.Lancet Oncol. 2018; 19: 416-426
- Assessment of doxorubicin and pembrolizumab in patients with advanced Anthracycline-Naive sarcoma: a phase 1/2 Nonrandomized clinical trial.JAMA Oncol. 2020; 6: 1778-1782
- Management of locally recurrent retroperitoneal sarcoma in the Adult: an updated consensus approach from the transatlantic Australasian retroperitoneal sarcoma working group.Ann Surg Oncol. 2022; (Article in press)
Article info
Publication history
Published online: June 09, 2022
Accepted:
June 6,
2022
Received in revised form:
June 2,
2022
Received:
December 8,
2021
Identification
Copyright
© 2022 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.