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Ovarian Low Grade Endometrioid Stromal Sarcoma

MedGen UID:
276498
•Concept ID:
C1518022
•
Neoplastic Process
Synonyms: Endometrioid Stromal Sarcoma of the Ovary; Low Grade Endometrioid Stromal Sarcoma of the Ovary; Ovarian Endometrioid Stromal Sarcoma

Definition

A rare invasive mesenchymal tumor that arises from the ovary. It is characterized by the absence of marked pleomorphism and nuclear atypia in the neoplastic mesenchymal cells. It may have a favorable clinical outcome. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Low Grade Endometrioid Stromal Sarcoma

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
Fertility after the treatment of gynecologic tumors.
Kesic V
Recent Results Cancer Res 2008;178:79-95. doi: 10.1007/978-3-540-71274-9_9. PMID: 18080446
Treatment options in stage I endometrial stromal sarcoma: a retrospective analysis of 53 cases.
Li N, Wu LY, Zhang HT, An JS, Li XG, Ma SK
Gynecol Oncol 2008 Feb;108(2):306-11. Epub 2007 Dec 3 doi: 10.1016/j.ygyno.2007.10.023. PMID: 18061249

Recent clinical studies

Etiology

Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study.
Dai Q, Xu B, Wu H, You Y, Li L
Int J Surg 2024 Apr 1;110(4):1919-1928. doi: 10.1097/JS9.0000000000001146. PMID: 38329091Free PMC Article
Ovarian preservation in gynecologic oncology: current indications and techniques.
Bizzarri N, Pavone M, Loverro M, Querleu D, Fagotti A, Scambia G
Curr Opin Oncol 2023 Sep 1;35(5):401-411. Epub 2023 Jul 13 doi: 10.1097/CCO.0000000000000969. PMID: 37498120
Beyond Smooth Muscle-Other Mesenchymal Neoplasms of the Uterus.
Dickson BC
Surg Pathol Clin 2019 Mar;12(1):107-137. Epub 2018 Dec 24 doi: 10.1016/j.path.2018.10.005. PMID: 30709439
Ovarian preservation for low-grade endometrial stromal sarcoma: a systematic review of the literature and meta-analysis.
Nasioudis D, Ko EM, Kolovos G, Vagios S, Kalliouris D, Giuntoli RL
Int J Gynecol Cancer 2019 Jan;29(1):126-132. doi: 10.1136/ijgc-2018-000063. PMID: 30640694
Low grade endometrial stromal sarcoma of uterine: review of 17 cases.
Behtash N, Akhavan S, Gilani MM, Mousavi A, Ghaemmaghami F, Mazhari MM
Acta Med Iran 2011;49(9):619-24. PMID: 22052146

Diagnosis

Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study.
Dai Q, Xu B, Wu H, You Y, Li L
Int J Surg 2024 Apr 1;110(4):1919-1928. doi: 10.1097/JS9.0000000000001146. PMID: 38329091Free PMC Article
Ovarian preservation in gynecologic oncology: current indications and techniques.
Bizzarri N, Pavone M, Loverro M, Querleu D, Fagotti A, Scambia G
Curr Opin Oncol 2023 Sep 1;35(5):401-411. Epub 2023 Jul 13 doi: 10.1097/CCO.0000000000000969. PMID: 37498120
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
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
Beyond Smooth Muscle-Other Mesenchymal Neoplasms of the Uterus.
Dickson BC
Surg Pathol Clin 2019 Mar;12(1):107-137. Epub 2018 Dec 24 doi: 10.1016/j.path.2018.10.005. PMID: 30709439

Therapy

An open-label, single-arm, prospective, multi-center, tandem two-stage designed phase II study to evaluate the efficacy of fulvestrant in women with recurrent/metastatic estrogen receptor-positive gynecological malignancies (FUCHSia study).
Trozzi R, Tuyaerts S, Annibali D, Herreros Pomares A, Boog L, Van Dam P, Leunen K, Deroose C, Trum H, Amant F
Int J Gynecol Cancer 2024 Aug 5;34(8):1217-1224. doi: 10.1136/ijgc-2023-005229. PMID: 38724237
Ovarian preservation for low-grade endometrial stromal sarcoma: a systematic review of the literature and meta-analysis.
Nasioudis D, Ko EM, Kolovos G, Vagios S, Kalliouris D, Giuntoli RL
Int J Gynecol Cancer 2019 Jan;29(1):126-132. doi: 10.1136/ijgc-2018-000063. PMID: 30640694
Estrogen therapy in gynecological cancer survivors.
Guidozzi F
Climacteric 2013 Dec;16(6):611-7. Epub 2013 Aug 16 doi: 10.3109/13697137.2013.806471. PMID: 23952524
Fertility-preserving surgical procedures for patients with gynecologic malignancies.
Rasool N, Rose PG
Clin Obstet Gynecol 2010 Dec;53(4):804-14. doi: 10.1097/GRF.0b013e3181f97d02. PMID: 21048447
Clear cell tumors of the female genital tract.
Matias-Guiu X, Lerma E, Prat J
Semin Diagn Pathol 1997 Nov;14(4):233-9. PMID: 9383823

Prognosis

Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study.
Dai Q, Xu B, Wu H, You Y, Li L
Int J Surg 2024 Apr 1;110(4):1919-1928. doi: 10.1097/JS9.0000000000001146. PMID: 38329091Free PMC Article
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
The prognosis of recurrent low-grade endometrial stromal sarcoma: a retrospective cohort study.
Dai Q, Xu B, Wu H, You Y, Wu M, Li L
Orphanet J Rare Dis 2021 Apr 7;16(1):160. doi: 10.1186/s13023-021-01802-8. PMID: 33827628Free PMC Article
Beyond Smooth Muscle-Other Mesenchymal Neoplasms of the Uterus.
Dickson BC
Surg Pathol Clin 2019 Mar;12(1):107-137. Epub 2018 Dec 24 doi: 10.1016/j.path.2018.10.005. PMID: 30709439
Ovarian preservation for low-grade endometrial stromal sarcoma: a systematic review of the literature and meta-analysis.
Nasioudis D, Ko EM, Kolovos G, Vagios S, Kalliouris D, Giuntoli RL
Int J Gynecol Cancer 2019 Jan;29(1):126-132. doi: 10.1136/ijgc-2018-000063. PMID: 30640694

Clinical prediction guides

Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study.
Dai Q, Xu B, Wu H, You Y, Li L
Int J Surg 2024 Apr 1;110(4):1919-1928. doi: 10.1097/JS9.0000000000001146. PMID: 38329091Free PMC Article
Endometrioid Borderline Ovarian Tumor: Clinical Characteristics, Prognosis, and Managements.
Ricotta G, Maulard A, Candiani M, Scherrier S, Genestie C, Pautier P, Leary A, Chargari C, Mangili G, Morice P, Gouy S
Ann Surg Oncol 2022 Sep;29(9):5894-5903. Epub 2022 May 19 doi: 10.1245/s10434-022-11893-7. PMID: 35590116
The prevalence of germline DICER1 pathogenic variation in cancer populations.
Kim J, Schultz KAP, Hill DA, Stewart DR
Mol Genet Genomic Med 2019 Mar;7(3):e555. Epub 2019 Jan 22 doi: 10.1002/mgg3.555. PMID: 30672147Free PMC Article
Ovarian preservation for low-grade endometrial stromal sarcoma: a systematic review of the literature and meta-analysis.
Nasioudis D, Ko EM, Kolovos G, Vagios S, Kalliouris D, Giuntoli RL
Int J Gynecol Cancer 2019 Jan;29(1):126-132. doi: 10.1136/ijgc-2018-000063. PMID: 30640694
Gynecologic Oncology Group studies with ifosfamide.
Sutton GP, Blessing JA, Manetta A, Homesley H, McGuire W
Semin Oncol 1992 Dec;19(6 Suppl 12):31-4. PMID: 1485172

Recent systematic reviews

Long-Term Survival Trend of Gynecological Cancer: A Systematic Review of Population-Based Cancer Registration Data.
Zhou XH, Yang DN, Zou YX, Tang DD, Chen J, Li ZY, Shen QM, Xu Q, Xiang YB
Biomed Environ Sci 2024 Aug 20;37(8):897-921. doi: 10.3967/bes2024.133. PMID: 39198254
Uterine lesions with sex cord-like architectures: a systematic review.
Jia M, Sun PL, Gao H
Diagn Pathol 2019 Nov 18;14(1):129. doi: 10.1186/s13000-019-0909-y. PMID: 31739799Free PMC Article
Ovarian preservation for low-grade endometrial stromal sarcoma: a systematic review of the literature and meta-analysis.
Nasioudis D, Ko EM, Kolovos G, Vagios S, Kalliouris D, Giuntoli RL
Int J Gynecol Cancer 2019 Jan;29(1):126-132. doi: 10.1136/ijgc-2018-000063. PMID: 30640694
Clinical characteristics and outcomes of uterine tumors resembling ovarian sex-cord tumors (UTROSCT): a systematic review of literature.
Blake EA, Sheridan TB, Wang KL, Takiuchi T, Kodama M, Sawada K, Matsuo K
Eur J Obstet Gynecol Reprod Biol 2014 Oct;181:163-70. Epub 2014 Aug 8 doi: 10.1016/j.ejogrb.2014.07.050. PMID: 25150955

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