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Retroperitoneal sarcoma

MedGen UID:
107937
•Concept ID:
C0585129
•
Neoplastic Process
Synonyms: Retroperitoneal Sarcoma; retroperitoneal sarcoma; retroperitoneal space sarcoma; sarcoma of retroperitoneal space
SNOMED CT: Retroperitoneal sarcoma (307219002)
 
Monarch Initiative: MONDO:0001501

Definition

A sarcoma that occurs in the retroperitoneal region. [from NCI]

Professional guidelines

PubMed

Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up(☆).
Gronchi A, Miah AB, Dei Tos AP, Abecassis N, Bajpai J, Bauer S, Biagini R, Bielack S, Blay JY, Bolle S, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brennan B, Brodowicz T, Buonadonna A, De Álava E, Del Muro XG, Dufresne A, Eriksson M, Fagioli F, Fedenko A, Ferraresi V, Ferrari A, Frezza AM, Gasperoni S, Gelderblom H, Gouin F, Grignani G, Haas R, Hassan AB, Hecker-Nolting S, Hindi N, Hohenberger P, Joensuu H, Jones RL, Jungels C, Jutte P, Kager L, Kasper B, Kawai A, Kopeckova K, Krákorová DA, Le Cesne A, Le Grange F, Legius E, Leithner A, Lopez-Pousa A, Martin-Broto J, Merimsky O, Messiou C, Mir O, Montemurro M, Morland B, Morosi C, Palmerini E, Pantaleo MA, Piana R, Piperno-Neumann S, Reichardt P, Rutkowski P, Safwat AA, Sangalli C, Sbaraglia M, Scheipl S, Schöffski P, Sleijfer S, Strauss D, Strauss S, Sundby Hall K, Trama A, Unk M, van de Sande MAJ, van der Graaf WTA, van Houdt WJ, Frebourg T, Casali PG, Stacchiotti S; ESMO Guidelines Committee, EURACAN and GENTURIS. Electronic address: [email protected]
Ann Oncol 2021 Nov;32(11):1348-1365. Epub 2021 Jul 22 doi: 10.1016/j.annonc.2021.07.006. PMID: 34303806
Retroperitoneal tumors: Review of diagnosis and management.
Sassa N
Int J Urol 2020 Dec;27(12):1058-1070. Epub 2020 Sep 10 doi: 10.1111/iju.14361. PMID: 32914475
Overview of primary adult retroperitoneal tumours.
Improta L, Tzanis D, Bouhadiba T, Abdelhafidh K, Bonvalot S
Eur J Surg Oncol 2020 Sep;46(9):1573-1579. Epub 2020 May 21 doi: 10.1016/j.ejso.2020.04.054. PMID: 32600897

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Next questions in the management of retroperitoneal sarcoma.
Drohan A, Gronchi A
Curr Opin Oncol 2023 Jul 1;35(4):301-308. Epub 2023 May 9 doi: 10.1097/CCO.0000000000000954. PMID: 37222202
Management of Recurrent Retroperitoneal Sarcoma.
Jolissaint JS, Raut CP, Fairweather M
Curr Oncol 2023 Feb 25;30(3):2761-2769. doi: 10.3390/curroncol30030209. PMID: 36975422Free PMC Article
Histology-tailored multidisciplinary management of primary retroperitoneal sarcomas.
Bonvalot S, Roland C, Raut C, Le Péchoux C, Tzanis D, Frezza AM, Gronchi A
Eur J Surg Oncol 2023 Jun;49(6):1061-1067. Epub 2022 May 16 doi: 10.1016/j.ejso.2022.05.010. PMID: 35599138
Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up(☆).
Gronchi A, Miah AB, Dei Tos AP, Abecassis N, Bajpai J, Bauer S, Biagini R, Bielack S, Blay JY, Bolle S, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brennan B, Brodowicz T, Buonadonna A, De Álava E, Del Muro XG, Dufresne A, Eriksson M, Fagioli F, Fedenko A, Ferraresi V, Ferrari A, Frezza AM, Gasperoni S, Gelderblom H, Gouin F, Grignani G, Haas R, Hassan AB, Hecker-Nolting S, Hindi N, Hohenberger P, Joensuu H, Jones RL, Jungels C, Jutte P, Kager L, Kasper B, Kawai A, Kopeckova K, Krákorová DA, Le Cesne A, Le Grange F, Legius E, Leithner A, Lopez-Pousa A, Martin-Broto J, Merimsky O, Messiou C, Mir O, Montemurro M, Morland B, Morosi C, Palmerini E, Pantaleo MA, Piana R, Piperno-Neumann S, Reichardt P, Rutkowski P, Safwat AA, Sangalli C, Sbaraglia M, Scheipl S, Schöffski P, Sleijfer S, Strauss D, Strauss S, Sundby Hall K, Trama A, Unk M, van de Sande MAJ, van der Graaf WTA, van Houdt WJ, Frebourg T, Casali PG, Stacchiotti S; ESMO Guidelines Committee, EURACAN and GENTURIS. Electronic address: [email protected]
Ann Oncol 2021 Nov;32(11):1348-1365. Epub 2021 Jul 22 doi: 10.1016/j.annonc.2021.07.006. PMID: 34303806
Myeloid Sarcoma: Presentation, Diagnosis, and Treatment.
Almond LM, Charalampakis M, Ford SJ, Gourevitch D, Desai A
Clin Lymphoma Myeloma Leuk 2017 May;17(5):263-267. Epub 2017 Mar 7 doi: 10.1016/j.clml.2017.02.027. PMID: 28342811

Diagnosis

Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up(☆).
Gronchi A, Miah AB, Dei Tos AP, Abecassis N, Bajpai J, Bauer S, Biagini R, Bielack S, Blay JY, Bolle S, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brennan B, Brodowicz T, Buonadonna A, De Álava E, Del Muro XG, Dufresne A, Eriksson M, Fagioli F, Fedenko A, Ferraresi V, Ferrari A, Frezza AM, Gasperoni S, Gelderblom H, Gouin F, Grignani G, Haas R, Hassan AB, Hecker-Nolting S, Hindi N, Hohenberger P, Joensuu H, Jones RL, Jungels C, Jutte P, Kager L, Kasper B, Kawai A, Kopeckova K, Krákorová DA, Le Cesne A, Le Grange F, Legius E, Leithner A, Lopez-Pousa A, Martin-Broto J, Merimsky O, Messiou C, Mir O, Montemurro M, Morland B, Morosi C, Palmerini E, Pantaleo MA, Piana R, Piperno-Neumann S, Reichardt P, Rutkowski P, Safwat AA, Sangalli C, Sbaraglia M, Scheipl S, Schöffski P, Sleijfer S, Strauss D, Strauss S, Sundby Hall K, Trama A, Unk M, van de Sande MAJ, van der Graaf WTA, van Houdt WJ, Frebourg T, Casali PG, Stacchiotti S; ESMO Guidelines Committee, EURACAN and GENTURIS. Electronic address: [email protected]
Ann Oncol 2021 Nov;32(11):1348-1365. Epub 2021 Jul 22 doi: 10.1016/j.annonc.2021.07.006. PMID: 34303806
Retroperitoneal tumors: Review of diagnosis and management.
Sassa N
Int J Urol 2020 Dec;27(12):1058-1070. Epub 2020 Sep 10 doi: 10.1111/iju.14361. PMID: 32914475
Malignant Soft-Tissue Sarcomas.
Brownstein JM, DeLaney TF
Hematol Oncol Clin North Am 2020 Feb;34(1):161-175. Epub 2019 Oct 28 doi: 10.1016/j.hoc.2019.08.022. PMID: 31739942
Multiply recurrent retroperitoneal liposarcoma.
Bagaria SP, Gabriel E, Mann GN
J Surg Oncol 2018 Jan;117(1):62-68. Epub 2017 Dec 19 doi: 10.1002/jso.24929. PMID: 29266232
Myeloid Sarcoma: Presentation, Diagnosis, and Treatment.
Almond LM, Charalampakis M, Ford SJ, Gourevitch D, Desai A
Clin Lymphoma Myeloma Leuk 2017 May;17(5):263-267. Epub 2017 Mar 7 doi: 10.1016/j.clml.2017.02.027. PMID: 28342811

Therapy

Preoperative Radiotherapy in Patients With Primary Retroperitoneal Sarcoma: EORTC-62092 Trial (STRASS) Versus Off-trial (STREXIT) Results.
Callegaro D, Raut CP, Ajayi T, Strauss D, Bonvalot S, Ng D, Stoeckle E, Fairweather M, Rutkowski P, van Houdt WJ, Gelderblom H, Sangalli C, Hayes A, Honoré C, Gladdy RA, Fau M, Haas R, Tzanis D, Miah AB, Chung P, Baldini EH, Marreaud S, Litiere S, Swallow CJ, Gronchi A
Ann Surg 2023 Jul 1;278(1):127-134. Epub 2022 Jul 14 doi: 10.1097/SLA.0000000000005492. PMID: 35833413
Histology-tailored multidisciplinary management of primary retroperitoneal sarcomas.
Bonvalot S, Roland C, Raut C, Le Péchoux C, Tzanis D, Frezza AM, Gronchi A
Eur J Surg Oncol 2023 Jun;49(6):1061-1067. Epub 2022 May 16 doi: 10.1016/j.ejso.2022.05.010. PMID: 35599138
Preoperative radiotherapy plus surgery versus surgery alone for patients with primary retroperitoneal sarcoma (EORTC-62092: STRASS): a multicentre, open-label, randomised, phase 3 trial.
Bonvalot S, Gronchi A, Le Péchoux C, Swallow CJ, Strauss D, Meeus P, van Coevorden F, Stoldt S, Stoeckle E, Rutkowski P, Rastrelli M, Raut CP, Hompes D, De Paoli A, Sangalli C, Honoré C, Chung P, Miah A, Blay JY, Fiore M, Stelmes JJ, Dei Tos AP, Baldini EH, Litière S, Marreaud S, Gelderblom H, Haas RL
Lancet Oncol 2020 Oct;21(10):1366-1377. Epub 2020 Sep 14 doi: 10.1016/S1470-2045(20)30446-0. PMID: 32941794
Randomized Controlled Trials in Soft Tissue Sarcoma: We Are Getting There!
MacNeill AJ, Gupta A, Swallow CJ
Surg Oncol Clin N Am 2017 Oct;26(4):531-544. Epub 2017 Aug 4 doi: 10.1016/j.soc.2017.05.001. PMID: 28923218
Myeloid Sarcoma: Presentation, Diagnosis, and Treatment.
Almond LM, Charalampakis M, Ford SJ, Gourevitch D, Desai A
Clin Lymphoma Myeloma Leuk 2017 May;17(5):263-267. Epub 2017 Mar 7 doi: 10.1016/j.clml.2017.02.027. PMID: 28342811

Prognosis

Histology-tailored multidisciplinary management of primary retroperitoneal sarcomas.
Bonvalot S, Roland C, Raut C, Le Péchoux C, Tzanis D, Frezza AM, Gronchi A
Eur J Surg Oncol 2023 Jun;49(6):1061-1067. Epub 2022 May 16 doi: 10.1016/j.ejso.2022.05.010. PMID: 35599138
Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up(☆).
Gronchi A, Miah AB, Dei Tos AP, Abecassis N, Bajpai J, Bauer S, Biagini R, Bielack S, Blay JY, Bolle S, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brennan B, Brodowicz T, Buonadonna A, De Álava E, Del Muro XG, Dufresne A, Eriksson M, Fagioli F, Fedenko A, Ferraresi V, Ferrari A, Frezza AM, Gasperoni S, Gelderblom H, Gouin F, Grignani G, Haas R, Hassan AB, Hecker-Nolting S, Hindi N, Hohenberger P, Joensuu H, Jones RL, Jungels C, Jutte P, Kager L, Kasper B, Kawai A, Kopeckova K, Krákorová DA, Le Cesne A, Le Grange F, Legius E, Leithner A, Lopez-Pousa A, Martin-Broto J, Merimsky O, Messiou C, Mir O, Montemurro M, Morland B, Morosi C, Palmerini E, Pantaleo MA, Piana R, Piperno-Neumann S, Reichardt P, Rutkowski P, Safwat AA, Sangalli C, Sbaraglia M, Scheipl S, Schöffski P, Sleijfer S, Strauss D, Strauss S, Sundby Hall K, Trama A, Unk M, van de Sande MAJ, van der Graaf WTA, van Houdt WJ, Frebourg T, Casali PG, Stacchiotti S; ESMO Guidelines Committee, EURACAN and GENTURIS. Electronic address: [email protected]
Ann Oncol 2021 Nov;32(11):1348-1365. Epub 2021 Jul 22 doi: 10.1016/j.annonc.2021.07.006. PMID: 34303806
Soft-tissue sarcoma in adults: An update on the current state of histiotype-specific management in an era of personalized medicine.
Gamboa AC, Gronchi A, Cardona K
CA Cancer J Clin 2020 May;70(3):200-229. Epub 2020 Apr 10 doi: 10.3322/caac.21605. PMID: 32275330
Multiply recurrent retroperitoneal liposarcoma.
Bagaria SP, Gabriel E, Mann GN
J Surg Oncol 2018 Jan;117(1):62-68. Epub 2017 Dec 19 doi: 10.1002/jso.24929. PMID: 29266232
Retroperitoneal sarcoma.
Thomas JM
Br J Surg 2007 Sep;94(9):1057-8. doi: 10.1002/bjs.5967. PMID: 17701955

Clinical prediction guides

Preoperative Radiotherapy in Patients With Primary Retroperitoneal Sarcoma: EORTC-62092 Trial (STRASS) Versus Off-trial (STREXIT) Results.
Callegaro D, Raut CP, Ajayi T, Strauss D, Bonvalot S, Ng D, Stoeckle E, Fairweather M, Rutkowski P, van Houdt WJ, Gelderblom H, Sangalli C, Hayes A, Honoré C, Gladdy RA, Fau M, Haas R, Tzanis D, Miah AB, Chung P, Baldini EH, Marreaud S, Litiere S, Swallow CJ, Gronchi A
Ann Surg 2023 Jul 1;278(1):127-134. Epub 2022 Jul 14 doi: 10.1097/SLA.0000000000005492. PMID: 35833413
Preoperative radiotherapy plus surgery versus surgery alone for patients with primary retroperitoneal sarcoma (EORTC-62092: STRASS): a multicentre, open-label, randomised, phase 3 trial.
Bonvalot S, Gronchi A, Le Péchoux C, Swallow CJ, Strauss D, Meeus P, van Coevorden F, Stoldt S, Stoeckle E, Rutkowski P, Rastrelli M, Raut CP, Hompes D, De Paoli A, Sangalli C, Honoré C, Chung P, Miah A, Blay JY, Fiore M, Stelmes JJ, Dei Tos AP, Baldini EH, Litière S, Marreaud S, Gelderblom H, Haas RL
Lancet Oncol 2020 Oct;21(10):1366-1377. Epub 2020 Sep 14 doi: 10.1016/S1470-2045(20)30446-0. PMID: 32941794
Surgical morbidity in retroperitoneal sarcoma resection.
MacNeill AJ, Fiore M
J Surg Oncol 2018 Jan;117(1):56-61. Epub 2018 Jan 3 doi: 10.1002/jso.24902. PMID: 29314041
Multiply recurrent retroperitoneal liposarcoma.
Bagaria SP, Gabriel E, Mann GN
J Surg Oncol 2018 Jan;117(1):62-68. Epub 2017 Dec 19 doi: 10.1002/jso.24929. PMID: 29266232
Management of recurrent retroperitoneal sarcoma.
Gyorki DE, Brennan MF
J Surg Oncol 2014 Jan;109(1):53-9. Epub 2013 Oct 24 doi: 10.1002/jso.23463. PMID: 24155163

Recent systematic reviews

Aggressive surgical approach with vascular resection and reconstruction for retroperitoneal sarcomas: a systematic review.
Hu H, Guo Q, Zhao J, Huang B, Du X
BMC Surg 2023 Sep 12;23(1):275. doi: 10.1186/s12893-023-02178-1. PMID: 37700246Free PMC Article
Dealing with malignancy involving the inferior vena cava in the 21st century.
Baia M, Naumann DN, Wong CS, Mahmood F, Parente A, Bissacco D, Almond M, Ford SJ, Tirotta F, Desai A
J Cardiovasc Surg (Torino) 2022 Dec;63(6):664-673. Epub 2022 Oct 14 doi: 10.23736/S0021-9509.22.12408-0. PMID: 36239927
Neoadjuvant radiotherapy for retroperitoneal sarcoma: A systematic review.
Cheng H, Miura JT, Lalehzari M, Rajeev R, Donahue AE, Bedi M, Gamblin TC, Turaga KK, Johnston FM
J Surg Oncol 2016 May;113(6):628-34. Epub 2016 Mar 16 doi: 10.1002/jso.24221. PMID: 26990903
Meta-analysis of perioperative antibiotics in patients undergoing laparoscopic cholecystectomy.
Pasquali S, Boal M, Griffiths EA, Alderson D, Vohra RS; CholeS Study Group; West Midlands Research Collaborative
Br J Surg 2016 Jan;103(1):27-34; discussion 34. Epub 2015 Sep 2 doi: 10.1002/bjs.9904. PMID: 26331356
Pregnancy and genital sarcoma: a systematic review of the literature.
Matsuo K, Eno ML, Im DD, Rosenshein NB
Am J Perinatol 2009 Aug;26(7):507-18. Epub 2009 Mar 13 doi: 10.1055/s-0029-1215428. PMID: 19288396

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Consumer resources

    Reviews

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