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Uterine corpus perivascular epithelioid cell tumor

MedGen UID:
276940
•Concept ID:
C1519862
•
Neoplastic Process
Synonyms: body of uterus neoplasm with perivascular epithelioid cell differentiation; Uterine Corpus PEComa; uterine corpus PEComa; Uterine Corpus Perivascular Epithelioid Cell Tumor; uterine corpus perivascular epithelioid cell tumor; uterine perivascular epithelioid cell tumor; uterine perivascular epithelioid cell tumour; uterus perivascular epithelioid cell tumor; uterus perivascular epithelioid cell tumour
 
Monarch Initiative: MONDO:0004221

Definition

A neoplasm with perivascular epithelioid cell differentiation arising from the uterine corpus wall. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUterine corpus perivascular epithelioid cell tumor

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
Predicting the Behavior of Perivascular Epithelioid Cell Tumors of the Uterine Corpus.
Acosta AM, Adley BP
Arch Pathol Lab Med 2017 Mar;141(3):463-469. doi: 10.5858/arpa.2016-0092-RS. PMID: 28234575
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
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

Perivascular epithelioid cell tumor of the uterine cervix identified on the liquid-based cytology: a case report.
Tang X, Feng M, Shen Y, Chen Q
Diagn Pathol 2023 Jan 16;18(1):7. doi: 10.1186/s13000-023-01290-3. PMID: 36647157Free PMC Article
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 of the uterine cervix identified on a conventional cervical smear.
Tajima S, Koda K
Diagn Cytopathol 2015 Dec;43(12):1011-6. Epub 2015 Sep 21 doi: 10.1002/dc.23369. PMID: 26390283
Perivascular epithelioid tumours (PEComas) of the gynaecological tract.
Conlon N, Soslow RA, Murali R
J Clin Pathol 2015 Jun;68(6):418-26. Epub 2015 Mar 6 doi: 10.1136/jclinpath-2015-202945. PMID: 25750268Free PMC Article
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

Prognosis

Perivascular epithelioid cell tumor of the uterine cervix identified on the liquid-based cytology: a case report.
Tang X, Feng M, Shen Y, Chen Q
Diagn Pathol 2023 Jan 16;18(1):7. doi: 10.1186/s13000-023-01290-3. PMID: 36647157Free PMC Article
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
Predicting the Behavior of Perivascular Epithelioid Cell Tumors of the Uterine Corpus.
Acosta AM, Adley BP
Arch Pathol Lab Med 2017 Mar;141(3):463-469. doi: 10.5858/arpa.2016-0092-RS. PMID: 28234575
Perivascular epithelioid tumours (PEComas) of the gynaecological tract.
Conlon N, Soslow RA, Murali R
J Clin Pathol 2015 Jun;68(6):418-26. Epub 2015 Mar 6 doi: 10.1136/jclinpath-2015-202945. PMID: 25750268Free PMC Article
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

Clinical prediction guides

Perivascular epithelioid cell tumor of the uterine cervix identified on the liquid-based cytology: a case report.
Tang X, Feng M, Shen Y, Chen Q
Diagn Pathol 2023 Jan 16;18(1):7. doi: 10.1186/s13000-023-01290-3. PMID: 36647157Free PMC Article
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
Predicting the Behavior of Perivascular Epithelioid Cell Tumors of the Uterine Corpus.
Acosta AM, Adley BP
Arch Pathol Lab Med 2017 Mar;141(3):463-469. doi: 10.5858/arpa.2016-0092-RS. PMID: 28234575
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
Heterologous and rare homologous sarcomas of the uterine corpus: a clinicopathologic review.
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

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