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Low Grade Endometrioid Stromal Sarcoma

MedGen UID:
137771
•Concept ID:
C0334486
•
Neoplastic Process
Synonyms: Endolymphatic Stromal Myoses; Endolymphatic Stromal Myosis; Myoses, Endolymphatic Stromal; Myosis, Endolymphatic Stromal; Sarcoma, Endometrial Stromal, Low-Grade; Stromal Myoses, Endolymphatic; Stromal Myosis, Endolymphatic
SNOMED CT: Stromal myosis (128726006); Stromal endometriosis (128726006); Endometrial stromatosis (128726006); Endolymphatic stromal myosis (128726006); Endometrial stromal sarcoma, low grade (128726006); Low grade endometrial stromal sarcoma (699357004); Endometrioid stromal sarcoma, low grade (128726006)

Definition

An infiltrating mesenchymal tumor arising from the uterine corpus, cervix, vagina, and the ovary. It is characterized by the presence of oval to spindle-shape cells that resemble the cells of the endometrial stroma, without evidence of significant atypia and pleomorphism. Numerous small vessels are also present. It is usually characterized by indolent growth and late recurrences. [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
Current management of uterine sarcomas.
García-Martínez E, Egea Prefasi L, García-Donas J, Escolar-Pérez PP, Pastor F, González-Martín A
Clin Transl Oncol 2011 May;13(5):307-14. doi: 10.1007/s12094-011-0659-0. PMID: 21596658

Recent clinical studies

Diagnosis

Primary extrauterine cystic low-grade endometrioid stromal sarcoma mimicking stromal endometriosis. A case report emphasizing the differential diagnosis and its potential local aggressive behavior.
Stolnicu S, Tunde C, Szilard G, Podoleanu C, Rozsnyai F
Pol J Pathol 2020;71(3):277-280. doi: 10.5114/pjp.2020.99795. PMID: 33112119
Unusual Presentations of Gynecologic Tumors: Primary, Extrauterine, Low-Grade Endometrioid Stromal Sarcoma.
Masand RP
Arch Pathol Lab Med 2018 Apr;142(4):536-541. Epub 2018 Jan 2 doi: 10.5858/arpa.2017-0241-RA. PMID: 29293021

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