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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: clinicalguidelines@esmo.org
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

A CT-based radiomics classification model for the prediction of histological type and tumour grade in retroperitoneal sarcoma (RADSARC-R): a retrospective multicohort analysis.
Arthur A, Orton MR, Emsley R, Vit S, Kelly-Morland C, Strauss D, Lunn J, Doran S, Lmalem H, Nzokirantevye A, Litiere S, Bonvalot S, Haas R, Gronchi A, Van Gestel D, Ducassou A, Raut CP, Meeus P, Spalek M, Hatton M, Le Pechoux C, Thway K, Fisher C, Jones R, Huang PH, Messiou C
Lancet Oncol 2023 Nov;24(11):1277-1286. doi: 10.1016/S1470-2045(23)00462-X. PMID: 37922931Free PMC Article
Current management of benign retroperitoneal tumors.
Tirotta F, Napolitano A, Noh S, Schmitz E, Nessim C, Patel D, Sicklick JK, Smith M, Thway K, van der Hage J, Ford SJ, Tseng WW
Eur J Surg Oncol 2023 Jun;49(6):1081-1090. Epub 2022 Jul 19 doi: 10.1016/j.ejso.2022.07.006. PMID: 35879135
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: clinicalguidelines@esmo.org
Ann Oncol 2021 Nov;32(11):1348-1365. Epub 2021 Jul 22 doi: 10.1016/j.annonc.2021.07.006. PMID: 34303806
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
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: clinicalguidelines@esmo.org
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
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
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
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
Systemic therapy in retroperitoneal sarcoma management.
Constantinidou A, Jones RL
J Surg Oncol 2018 Jan;117(1):87-92. Epub 2017 Nov 30 doi: 10.1002/jso.24933. PMID: 29194633
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
Radiation therapy for retroperitoneal sarcoma.
Tuan J, Vitolo V, Vischioni B, Iannalfi A, Fiore MR, Fossati P, Orecchia R
Radiol Med 2014 Oct;119(10):790-802. Epub 2014 Mar 18 doi: 10.1007/s11547-013-0350-3. PMID: 24638910

Prognosis

A CT-based radiomics classification model for the prediction of histological type and tumour grade in retroperitoneal sarcoma (RADSARC-R): a retrospective multicohort analysis.
Arthur A, Orton MR, Emsley R, Vit S, Kelly-Morland C, Strauss D, Lunn J, Doran S, Lmalem H, Nzokirantevye A, Litiere S, Bonvalot S, Haas R, Gronchi A, Van Gestel D, Ducassou A, Raut CP, Meeus P, Spalek M, Hatton M, Le Pechoux C, Thway K, Fisher C, Jones R, Huang PH, Messiou C
Lancet Oncol 2023 Nov;24(11):1277-1286. doi: 10.1016/S1470-2045(23)00462-X. PMID: 37922931Free PMC Article
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: clinicalguidelines@esmo.org
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.
Leffall LD Jr
Ann Surg 1991 Jul;214(1):1. PMID: 2064464Free PMC Article

Clinical prediction guides

A CT-based radiomics classification model for the prediction of histological type and tumour grade in retroperitoneal sarcoma (RADSARC-R): a retrospective multicohort analysis.
Arthur A, Orton MR, Emsley R, Vit S, Kelly-Morland C, Strauss D, Lunn J, Doran S, Lmalem H, Nzokirantevye A, Litiere S, Bonvalot S, Haas R, Gronchi A, Van Gestel D, Ducassou A, Raut CP, Meeus P, Spalek M, Hatton M, Le Pechoux C, Thway K, Fisher C, Jones R, Huang PH, Messiou C
Lancet Oncol 2023 Nov;24(11):1277-1286. doi: 10.1016/S1470-2045(23)00462-X. PMID: 37922931Free PMC Article
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

Recent systematic reviews

A systematic review of the role of chemotherapy in retroperitoneal sarcoma by the Australia and New Zealand sarcoma association clinical practice guidelines working party.
Zhou DD, Connolly EA, Mar J, Lazarakis S, Grimison PS, Connor J, Gyorki DE, Hong AM
Cancer Treat Rev 2024 Jan;122:102663. Epub 2023 Nov 25 doi: 10.1016/j.ctrv.2023.102663. PMID: 38039565
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
Radiotherapy and surgery-an indispensable duo in the treatment of retroperitoneal sarcoma.
Van De Voorde L, Delrue L, van Eijkeren M, De Meerleer G
Cancer 2011 Oct 1;117(19):4355-64. Epub 2011 Mar 28 doi: 10.1002/cncr.26071. PMID: 21446048
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

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    Clinical resources

    Practice guidelines

    • PubMed
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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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