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Uterine Corpus Epithelioid Smooth Muscle Tumor of Uncertain Malignant Potential

MedGen UID:
1792896
•Concept ID:
C5556455
•
Neoplastic Process
Synonym: Uterine Corpus Epithelioid STUMP

Definition

A smooth muscle tumor of uncertain malignant potential that arises from the uterine corpus and is composed of neoplastic epithelioid cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUterine Corpus Epithelioid Smooth Muscle Tumor of Uncertain Malignant Potential

Professional guidelines

PubMed

TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis.
Schoolmeester JK, Dao LN, Sukov WR, Wang L, Park KJ, Murali R, Hameed MR, Soslow RA
Am J Surg Pathol 2015 Mar;39(3):394-404. doi: 10.1097/PAS.0000000000000349. PMID: 25517951Free PMC Article

Recent clinical studies

Etiology

Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases.
Fadare O
Adv Anat Pathol 2008 Mar;15(2):63-75. doi: 10.1097/PAP.0b013e31816613b0. PMID: 18418088
Perivascular epithelioid cell tumor ('PEComa') of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors.
Vang R, Kempson RL
Am J Surg Pathol 2002 Jan;26(1):1-13. doi: 10.1097/00000478-200201000-00001. PMID: 11756764

Diagnosis

NTRK-Fusion Sarcoma of the Uterine Cervix: Report of 2 Cases With Comparative Clinicopathologic Features.
Nilforoushan N, Wethington SL, Nonogaki H, Gross J, Vang R, Xing D
Int J Gynecol Pathol 2022 Nov 1;41(6):642-648. Epub 2021 Oct 29 doi: 10.1097/PGP.0000000000000834. PMID: 34723848Free PMC Article
EWSR1-WT1 gene fusions in neoplasms other than desmoplastic small round cell tumor: a report of three unusual tumors involving the female genital tract and review of the literature.
Schoolmeester JK, Folpe AL, Nair AA, Halling K, Sutton BC, Landers E, Karnezis AN, Dickson BC, Nucci MR, Kolin DL
Mod Pathol 2021 Oct;34(10):1912-1920. Epub 2021 Jun 7 doi: 10.1038/s41379-021-00843-5. PMID: 34099870
PNL2: A Useful Adjunct Biomarker to HMB45 in the Diagnosis of Uterine Perivascular Epithelioid Cell Tumor (PEComa).
Valencia-Guerrero A, Pinto A, Anderson WJ, Trevisan G, Nucci MR, Hirsch MS
Int J Gynecol Pathol 2020 Nov;39(6):529-536. doi: 10.1097/PGP.0000000000000653. PMID: 31851063
Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
Epithelioid malignant peripheral nerve sheath tumor of the uterine corpus.
Gulati N, Rekhi B, Suryavanshi P, Jambhekar NA
Ann Diagn Pathol 2011 Dec;15(6):441-5. Epub 2010 Aug 30 doi: 10.1016/j.anndiagpath.2010.06.002. PMID: 20952276

Therapy

Large cell neuroendocrine carcinoma with sarcomatous changes of the endometrium: a case report with immunohistochemical studies and molecular genetic study of KIT and PDGFRA.
Terada T
Pathol Res Pract 2010 Jun 15;206(6):420-5. Epub 2010 Feb 26 doi: 10.1016/j.prp.2009.12.008. PMID: 20189318

Prognosis

Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
Alveolar Soft Part Sarcoma of the Female Genital Tract: A Morphologic, Immunohistochemical, and Molecular Cytogenetic Study of 10 Cases With Emphasis on its Distinction From Morphologic Mimics.
Schoolmeester JK, Carlson J, Keeney GL, Fritchie KJ, Oliva E, Young RH, Nucci MR
Am J Surg Pathol 2017 May;41(5):622-632. doi: 10.1097/PAS.0000000000000796. PMID: 28009610
TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis.
Schoolmeester JK, Dao LN, Sukov WR, Wang L, Park KJ, Murali R, Hameed MR, Soslow RA
Am J Surg Pathol 2015 Mar;39(3):394-404. doi: 10.1097/PAS.0000000000000349. PMID: 25517951Free PMC Article
Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases.
Fadare O
Adv Anat Pathol 2008 Mar;15(2):63-75. doi: 10.1097/PAP.0b013e31816613b0. PMID: 18418088
Endometrioid carcinomas of the uterine corpus with sex cord-like formations, hyalinization, and other unusual morphologic features: a report of 31 cases of a neoplasm that may be confused with carcinosarcoma and other uterine neoplasms.
Murray SK, Clement PB, Young RH
Am J Surg Pathol 2005 Feb;29(2):157-66. doi: 10.1097/01.pas.0000149704.89463.05. PMID: 15644772

Clinical prediction guides

Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
Alveolar Soft Part Sarcoma of the Female Genital Tract: A Morphologic, Immunohistochemical, and Molecular Cytogenetic Study of 10 Cases With Emphasis on its Distinction From Morphologic Mimics.
Schoolmeester JK, Carlson J, Keeney GL, Fritchie KJ, Oliva E, Young RH, Nucci MR
Am J Surg Pathol 2017 May;41(5):622-632. doi: 10.1097/PAS.0000000000000796. PMID: 28009610
TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis.
Schoolmeester JK, Dao LN, Sukov WR, Wang L, Park KJ, Murali R, Hameed MR, Soslow RA
Am J Surg Pathol 2015 Mar;39(3):394-404. doi: 10.1097/PAS.0000000000000349. PMID: 25517951Free PMC Article

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