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Stromal sarcoma

MedGen UID:
235091
•Concept ID:
C1370723
•
Neoplastic Process
Synonyms: Malignant stromal tumor; Stromal Sarcoma; stromal sarcoma; stromal sarcoma, malignant; stromal tumor, malignant
SNOMED CT: Stromal sarcoma (128753005); Malignant stromal tumor (253048008)
 
Monarch Initiative: MONDO:0044337

Definition

A malignant neoplasm characterized by the presence of atypical mesenchymal-stromal cells. Representative examples include endometrial stromal sarcoma and prostate stromal sarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVStromal sarcoma

Professional guidelines

PubMed

Current Treatment Options: Uterine Sarcoma.
Lewis D, Liang A, Mason T, Ferriss JS
Curr Treat Options Oncol 2024 Jul;25(7):829-853. Epub 2024 May 31 doi: 10.1007/s11864-024-01214-3. PMID: 38819624
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

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
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
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

Gynecological sarcomas: literature review of 2020.
Hanvic B, Ray-Coquard I
Curr Opin Oncol 2021 Jul 1;33(4):345-350. doi: 10.1097/CCO.0000000000000753. PMID: 34009140
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
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
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
Endometriosis-associated ovarian neoplasia.
Matias-Guiu X, Stewart CJR
Pathology 2018 Feb;50(2):190-204. Epub 2017 Dec 11 doi: 10.1016/j.pathol.2017.10.006. PMID: 29241974
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

Clinical characteristics and prognosis analysis of uterine sarcoma: a single-institution retrospective study.
Wang F, Dai X, Chen H, Hu X, Wang Y
BMC Cancer 2022 Oct 7;22(1):1050. doi: 10.1186/s12885-022-10129-x. PMID: 36207687Free PMC Article
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
Endometriosis-associated ovarian neoplasia.
Matias-Guiu X, Stewart CJR
Pathology 2018 Feb;50(2):190-204. Epub 2017 Dec 11 doi: 10.1016/j.pathol.2017.10.006. PMID: 29241974
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

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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