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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
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
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
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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