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Endometrial stromal sarcoma(ESS)

MedGen UID:
60196
•Concept ID:
C0206630
•
Neoplastic Process
Synonyms: Endometrioid stromal sarcoma; ESS
SNOMED CT: Endometrial stromal sarcoma (1178986006); Endometrial sarcoma (1178986006)
 
Monarch Initiative: MONDO:0006745
Orphanet: ORPHA213711

Definition

A malignant mesenchymal neoplasm that affects the uterine corpus, and rarely, the ovaries, cervix, and vagina. In the uterine corpus it is classified as low grade or high grade endometrial stromal sarcoma. In the remainder of the anatomic sites it is classified as low grade endometrioid stromal sarcoma. [from NCI]

Professional guidelines

PubMed

Endometrial stromal tumors of the uterus: Epidemiology, pathological and biological features, treatment options and clinical outcomes.
Gadducci A, Multinu F, De Vitis LA, Cosio S, Carinelli S, Aletti GD
Gynecol Oncol 2023 Apr;171:95-105. Epub 2023 Feb 24 doi: 10.1016/j.ygyno.2023.02.009. PMID: 36842409
Systemic treatment in adult uterine sarcomas.
Desar IME, Ottevanger PB, Benson C, van der Graaf WTA
Crit Rev Oncol Hematol 2018 Feb;122:10-20. Epub 2017 Dec 14 doi: 10.1016/j.critrevonc.2017.12.009. PMID: 29458779
Systemic therapy for endometrial stromal sarcomas: current treatment options.
Serkies K, Pawłowska E, Jassem J
Ginekol Pol 2016;87(8):594-7. doi: 10.5603/GP.2016.0051. PMID: 27629136

Curated

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

Recent clinical studies

Etiology

Uterine sarcomas and rare uterine mesenchymal tumors with malignant potential. Diagnostic guidelines of the French Sarcoma Group and the Rare Gynecological Tumors Group.
Croce S, Devouassoux-Shisheboran M, Pautier P, Ray-Coquard I, Treilleux I, Neuville A, Arnould L, Just PA, Belda MALF, Averous G, Leroux A, Mery E, Loussouarn D, Weinbreck N, Le Guellec S, Mishellany F, Morice P, Guyon F, Genestie C
Gynecol Oncol 2022 Nov;167(2):373-389. Epub 2022 Sep 14 doi: 10.1016/j.ygyno.2022.07.031. PMID: 36114030
A nomogram for predicting overall survival in patients with low-grade endometrial stromal sarcoma: A population-based analysis.
Wu J, Zhang H, Li L, Hu M, Chen L, Xu B, Song Q
Cancer Commun (Lond) 2020 Jul;40(7):301-312. Epub 2020 Jun 18 doi: 10.1002/cac2.12067. PMID: 32558385Free PMC Article
BCOR involvement in cancer.
Astolfi A, Fiore M, Melchionda F, Indio V, Bertuccio SN, Pession A
Epigenomics 2019 May;11(7):835-855. Epub 2019 May 31 doi: 10.2217/epi-2018-0195. PMID: 31150281Free PMC Article
Molecular characteristics of uterine sarcomas.
Davidson B, Micci F
Expert Rev Mol Diagn 2017 May;17(5):515-522. Epub 2017 Apr 3 doi: 10.1080/14737159.2017.1311790. PMID: 28335657
Uterine sarcoma 2008.
Lin JF, Slomovitz BM
Curr Oncol Rep 2008 Nov;10(6):512-8. doi: 10.1007/s11912-008-0077-9. PMID: 18928666

Diagnosis

Endometrial stromal tumors of the uterus: Epidemiology, pathological and biological features, treatment options and clinical outcomes.
Gadducci A, Multinu F, De Vitis LA, Cosio S, Carinelli S, Aletti GD
Gynecol Oncol 2023 Apr;171:95-105. Epub 2023 Feb 24 doi: 10.1016/j.ygyno.2023.02.009. PMID: 36842409
Uterine sarcomas and rare uterine mesenchymal tumors with malignant potential. Diagnostic guidelines of the French Sarcoma Group and the Rare Gynecological Tumors Group.
Croce S, Devouassoux-Shisheboran M, Pautier P, Ray-Coquard I, Treilleux I, Neuville A, Arnould L, Just PA, Belda MALF, Averous G, Leroux A, Mery E, Loussouarn D, Weinbreck N, Le Guellec S, Mishellany F, Morice P, Guyon F, Genestie C
Gynecol Oncol 2022 Nov;167(2):373-389. Epub 2022 Sep 14 doi: 10.1016/j.ygyno.2022.07.031. PMID: 36114030
Update on Uterine Mesenchymal Neoplasms.
Kertowidjojo EC, Bennett JA
Surg Pathol Clin 2022 Jun;15(2):315-340. Epub 2022 May 20 doi: 10.1016/j.path.2022.02.008. PMID: 35715164
A nomogram for predicting overall survival in patients with low-grade endometrial stromal sarcoma: A population-based analysis.
Wu J, Zhang H, Li L, Hu M, Chen L, Xu B, Song Q
Cancer Commun (Lond) 2020 Jul;40(7):301-312. Epub 2020 Jun 18 doi: 10.1002/cac2.12067. PMID: 32558385Free PMC Article
Uterine sarcomas.
Moinfar F, Azodi M, Tavassoli FA
Pathology 2007 Feb;39(1):55-71. doi: 10.1080/00313020601136146. PMID: 17365823

Therapy

Hormonal therapy in uterine sarcomas.
Zang Y, Dong M, Zhang K, Gao C, Guo F, Wang Y, Xue F
Cancer Med 2019 Apr;8(4):1339-1349. Epub 2019 Mar 21 doi: 10.1002/cam4.2044. PMID: 30897294Free PMC Article
Low-Grade Endometrial Stromal Sarcoma - a Review.
Thiel FC, Halmen S
Oncol Res Treat 2018;41(11):687-692. Epub 2018 Oct 13 doi: 10.1159/000494225. PMID: 30317238
Systemic therapy for endometrial stromal sarcomas: current treatment options.
Serkies K, Pawłowska E, Jassem J
Ginekol Pol 2016;87(8):594-7. doi: 10.5603/GP.2016.0051. PMID: 27629136
Uterine sarcoma 2008.
Lin JF, Slomovitz BM
Curr Oncol Rep 2008 Nov;10(6):512-8. doi: 10.1007/s11912-008-0077-9. PMID: 18928666
Uterine sarcomas.
Moinfar F, Azodi M, Tavassoli FA
Pathology 2007 Feb;39(1):55-71. doi: 10.1080/00313020601136146. PMID: 17365823

Prognosis

Endometrial stromal tumors of the uterus: Epidemiology, pathological and biological features, treatment options and clinical outcomes.
Gadducci A, Multinu F, De Vitis LA, Cosio S, Carinelli S, Aletti GD
Gynecol Oncol 2023 Apr;171:95-105. Epub 2023 Feb 24 doi: 10.1016/j.ygyno.2023.02.009. PMID: 36842409
A nomogram for predicting overall survival in patients with low-grade endometrial stromal sarcoma: A population-based analysis.
Wu J, Zhang H, Li L, Hu M, Chen L, Xu B, Song Q
Cancer Commun (Lond) 2020 Jul;40(7):301-312. Epub 2020 Jun 18 doi: 10.1002/cac2.12067. PMID: 32558385Free PMC Article
Low-Grade Endometrial Stromal Sarcoma - a Review.
Thiel FC, Halmen S
Oncol Res Treat 2018;41(11):687-692. Epub 2018 Oct 13 doi: 10.1159/000494225. PMID: 30317238
Uterine sarcomas.
Mbatani N, Olawaiye AB, Prat J
Int J Gynaecol Obstet 2018 Oct;143 Suppl 2:51-58. doi: 10.1002/ijgo.12613. PMID: 30306577
Molecular characteristics of uterine sarcomas.
Davidson B, Micci F
Expert Rev Mol Diagn 2017 May;17(5):515-522. Epub 2017 Apr 3 doi: 10.1080/14737159.2017.1311790. PMID: 28335657

Clinical prediction guides

Uterine sarcomas and rare uterine mesenchymal tumors with malignant potential. Diagnostic guidelines of the French Sarcoma Group and the Rare Gynecological Tumors Group.
Croce S, Devouassoux-Shisheboran M, Pautier P, Ray-Coquard I, Treilleux I, Neuville A, Arnould L, Just PA, Belda MALF, Averous G, Leroux A, Mery E, Loussouarn D, Weinbreck N, Le Guellec S, Mishellany F, Morice P, Guyon F, Genestie C
Gynecol Oncol 2022 Nov;167(2):373-389. Epub 2022 Sep 14 doi: 10.1016/j.ygyno.2022.07.031. PMID: 36114030
A nomogram for predicting overall survival in patients with low-grade endometrial stromal sarcoma: A population-based analysis.
Wu J, Zhang H, Li L, Hu M, Chen L, Xu B, Song Q
Cancer Commun (Lond) 2020 Jul;40(7):301-312. Epub 2020 Jun 18 doi: 10.1002/cac2.12067. PMID: 32558385Free PMC Article
Molecular characteristics of uterine sarcomas.
Davidson B, Micci F
Expert Rev Mol Diagn 2017 May;17(5):515-522. Epub 2017 Apr 3 doi: 10.1080/14737159.2017.1311790. PMID: 28335657
Endometrial stromal tumours revisited: an update based on the 2014 WHO classification.
Ali RH, Rouzbahman M
J Clin Pathol 2015 May;68(5):325-32. Epub 2015 Jan 16 doi: 10.1136/jclinpath-2014-202829. PMID: 25595274
Endometrial stromal tumors: the new WHO classification.
Conklin CM, Longacre TA
Adv Anat Pathol 2014 Nov;21(6):383-93. doi: 10.1097/PAP.0000000000000046. PMID: 25299308

Recent systematic reviews

Extrauterine endometrial stromal sarcoma: A systematic review and outcome analysis.
Deb PQ, Heller DS
Ann Diagn Pathol 2022 Aug;59:151966. Epub 2022 May 10 doi: 10.1016/j.anndiagpath.2022.151966. PMID: 35569210
Endometrial stromal sarcoma: A review of rare mesenchymal uterine neoplasm.
Capozzi VA, Monfardini L, Ceni V, Cianciolo A, Butera D, Gaiano M, Berretta R
J Obstet Gynaecol Res 2020 Nov;46(11):2221-2236. Epub 2020 Aug 23 doi: 10.1111/jog.14436. PMID: 32830415
Uterine sarcoma part III-Targeted therapy: The Taiwan Association of Gynecology (TAG) systematic review.
Yen MS, Chen JR, Wang PH, Wen KC, Chen YJ, Ng HT; Taiwan Association of Gynecology Systematic Review Group
Taiwan J Obstet Gynecol 2016 Oct;55(5):625-634. doi: 10.1016/j.tjog.2016.07.001. PMID: 27751406
Uterine sarcoma Part II-Uterine endometrial stromal sarcoma: The TAG systematic review.
Horng HC, Wen KC, Wang PH, Chen YJ, Yen MS, Ng HT; Taiwan Association of Gynecology Systematic Review Group
Taiwan J Obstet Gynecol 2016 Aug;55(4):472-9. doi: 10.1016/j.tjog.2016.04.034. PMID: 27590366
Endometrial stromal sarcoma: a systematic review.
Rauh-Hain JA, del Carmen MG
Obstet Gynecol 2013 Sep;122(3):676-83. doi: 10.1097/AOG.0b013e3182a189ac. PMID: 23921879

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    Clinical resources

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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

    Reviews

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