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Epithelioid leiomyosarcoma

MedGen UID:
61454
•Concept ID:
C0205815
•
Neoplastic Process
Synonyms: Epithelioid Leiomyosarcoma; Epithelioid Leiomyosarcomas; Leiomyosarcoma, Epithelioid; Leiomyosarcomas, Epithelioid
SNOMED CT: Epithelioid leiomyosarcoma (42392001)
 
Monarch Initiative: MONDO:0003356

Definition

A morphologic variant of leiomyosarcoma characterized by the presence of epithelioid round cells with eosinophilic to clear cytoplasm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpithelioid leiomyosarcoma

Professional guidelines

PubMed

Epithelioid Leiomyosarcoma of the Uterus: Modern Outcome-based Appraisal of Diagnostic Criteria in a Large Institutional Series.
Chapel DB, Nucci MR, Quade BJ, Parra-Herran C
Am J Surg Pathol 2022 Apr 1;46(4):464-475. doi: 10.1097/PAS.0000000000001795. PMID: 34419987
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):168-77. Epub 2015 Mar 26 doi: 10.1177/0394632015576029. PMID: 25816394
Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2.
Chokoeva AA, Tchernev G, Cardoso JC, Patterson JW, Dechev I, Valkanov S, Zanardelli M, Lotti T, Wollina U
Int J Immunopathol Pharmacol 2015 Jun;28(2):178-86. Epub 2015 Mar 26 doi: 10.1177/0394632015575977. PMID: 25816393

Recent clinical studies

Etiology

Epithelioid leiomyosarcoma: An unusual presentation.
Kalantri SH, Joseph LD, Priyathersini N, Elangovan B
J Cancer Res Ther 2023 Jan-Mar;19(2):490-492. doi: 10.4103/jcrt.jcrt_1178_21. PMID: 37313923
Analysis of Extended Resection of Limb Soft Tissue Leiomyosarcoma.
Liu Q, Tang L, Hu X, Ye J, Liang P, Panayi AC, Wang S, Deng J
Biomed Res Int 2021;2021:2106972. Epub 2021 Aug 3 doi: 10.1155/2021/2106972. PMID: 34395610Free PMC Article
Recurrent vaginal epithelioid leiomyosarcoma; a case report from Botswana and review of the literature.
Benti TM, Mercy N, Eshetu AB, Iwuh IE, Kassa MW
Afr J Reprod Health 2021 Feb;25(1):161-168. doi: 10.29063/ajrh2021/v25i1.17. PMID: 34077121
PGR Gene Fusions Identify a Molecular Subset of Uterine Epithelioid Leiomyosarcoma With Rhabdoid Features.
Chiang S, Samore W, Zhang L, Sung YS, Turashvili G, Murali R, Soslow RA, Hensley ML, Swanson D, Dickson BC, Stewart CJR, Oliva E, Antonescu CR
Am J Surg Pathol 2019 Jun;43(6):810-818. doi: 10.1097/PAS.0000000000001239. PMID: 30829727Free PMC Article
Epithelioid leiomyosarcoma of uterus.
Setia A, Kanotra S, Aggarwal R, Bhavthankar DP
BMJ Case Rep 2012 Apr 2;2012 doi: 10.1136/bcr.11.2011.5144. PMID: 22602833Free PMC Article

Diagnosis

Epithelioid leiomyosarcoma: An unusual presentation.
Kalantri SH, Joseph LD, Priyathersini N, Elangovan B
J Cancer Res Ther 2023 Jan-Mar;19(2):490-492. doi: 10.4103/jcrt.jcrt_1178_21. PMID: 37313923
Epithelioid leiomyosarcoma of the vulva: report of a rare case and literature review.
Lan H, Chai W, Gong F, Jia G
J Int Med Res 2022 Apr;50(4):3000605221090473. doi: 10.1177/03000605221090473. PMID: 35410510Free PMC Article
Incidental superficial soft tissue epithelioid angioleiomyoma.
Val-Bernal JF, Fontanil N, García-Montesinos B, Martino M
J Cutan Pathol 2022 Aug;49(8):731-735. Epub 2022 Apr 19 doi: 10.1111/cup.14233. PMID: 35357042
Epithelioid leiomyosarcoma of broad ligament harboring PGR-NR4A3 and UBR5-PGR gene fusions: a unique case report.
Ren H, Li Y, Yao Q, Lv H, Tang S, Zhou X, Yang W
Virchows Arch 2022 Apr;480(4):933-938. Epub 2021 Aug 5 doi: 10.1007/s00428-021-03169-4. PMID: 34351486
When the uterine mass is not a leiomyoma.
Pereira S, Sousa R, Constantino L, Abrantes C
BMJ Case Rep 2021 Aug 17;14(8) doi: 10.1136/bcr-2021-243845. PMID: 34404659Free PMC Article

Therapy

Recurrent vaginal epithelioid leiomyosarcoma; a case report from Botswana and review of the literature.
Benti TM, Mercy N, Eshetu AB, Iwuh IE, Kassa MW
Afr J Reprod Health 2021 Feb;25(1):161-168. doi: 10.29063/ajrh2021/v25i1.17. PMID: 34077121
Long-term response to first-line trabectedin in an elderly female patient with a metastatic leiomyosarcoma unfit for anthracycline.
Maruzzo M, Brunello A, Diminutto A, Rastrelli M, Basso U
Anticancer Drugs 2016 Mar;27(3):264-7. doi: 10.1097/CAD.0000000000000326. PMID: 26629769Free PMC Article
Primary multiple epithelioid leiomyosarcoma of the liver.
Cioffi U, Quattrone P, De Simone M, Bonavina L, Segalin A, Masini T, Montorsi M
Hepatogastroenterology 1996 Nov-Dec;43(12):1603-5. PMID: 8975973
Combined therapy in the treatment of gastric epithelioid leiomyosarcoma.
van der Vaart CH, Spoelstra P, Samsom JP, Klinkert P, de Koning J
Neth J Med 1990 Feb;36(1-2):69-73. PMID: 2314523
Gastric tumors in patients with pulmonary chondroma or extra-adrenal paraganglioma: an ultrastructural study.
Wick MR, Ruebner BH, Carney JA
Arch Pathol Lab Med 1981 Oct;105(10):527-31. PMID: 6895164

Prognosis

Epithelioid leiomyosarcoma: An unusual presentation.
Kalantri SH, Joseph LD, Priyathersini N, Elangovan B
J Cancer Res Ther 2023 Jan-Mar;19(2):490-492. doi: 10.4103/jcrt.jcrt_1178_21. PMID: 37313923
Clinically Aggressive Uterine Epithelioid Leiomyosarcoma with Rhabdomyoblastic Differentiation and High Proliferation Rate: A Case Report.
Kim JY, Kim B
Am J Case Rep 2023 May 16;24:e939349. doi: 10.12659/AJCR.939349. PMID: 37190674Free PMC Article
When the uterine mass is not a leiomyoma.
Pereira S, Sousa R, Constantino L, Abrantes C
BMJ Case Rep 2021 Aug 17;14(8) doi: 10.1136/bcr-2021-243845. PMID: 34404659Free PMC Article
Recurrent vaginal epithelioid leiomyosarcoma; a case report from Botswana and review of the literature.
Benti TM, Mercy N, Eshetu AB, Iwuh IE, Kassa MW
Afr J Reprod Health 2021 Feb;25(1):161-168. doi: 10.29063/ajrh2021/v25i1.17. PMID: 34077121
Leiomyosarcoma of the gastro-intestinal tract: general pattern of metastasis and recurrence.
Lee YT
Cancer Treat Rev 1983 Jun;10(2):91-101. doi: 10.1016/0305-7372(83)90007-5. PMID: 6347377

Clinical prediction guides

Incidental superficial soft tissue epithelioid angioleiomyoma.
Val-Bernal JF, Fontanil N, García-Montesinos B, Martino M
J Cutan Pathol 2022 Aug;49(8):731-735. Epub 2022 Apr 19 doi: 10.1111/cup.14233. PMID: 35357042
Epithelioid Leiomyosarcoma of the Uterus: Modern Outcome-based Appraisal of Diagnostic Criteria in a Large Institutional Series.
Chapel DB, Nucci MR, Quade BJ, Parra-Herran C
Am J Surg Pathol 2022 Apr 1;46(4):464-475. doi: 10.1097/PAS.0000000000001795. PMID: 34419987
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
Characteristics of mismatch repair deficiency in sarcomas.
Doyle LA, Nowak JA, Nathenson MJ, Thornton K, Wagner AJ, Johnson JM, Albrayak A, George S, Sholl LM
Mod Pathol 2019 Jul;32(7):977-987. Epub 2019 Feb 14 doi: 10.1038/s41379-019-0202-3. PMID: 30765880
Epithelioid leiomyosarcoma of the uterine cervix: a case report and review of the literature.
Toyoshima M, Okamura C, Niikura H, Ito K, Yaegashi N
Gynecol Oncol 2005 Jun;97(3):957-60. doi: 10.1016/j.ygyno.2005.02.028. PMID: 15890394

Recent systematic reviews

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

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