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Benign PEComa

MedGen UID:
825530
•Concept ID:
C3839685
•
Neoplastic Process
Synonyms: benign neoplasm with perivascular epithelioid cell differentiation; benign PEComa; Benign PEComa, NOS; benign PEComa, nos; Benign PEComa, Not Otherwise Specified; benign PEComa, not otherwise specified; neoplasm with perivascular epithelioid cell differentiation, benign; PEComa, benign; Perivascular epithelioid tumor, benign; Typical PEComa; typical PEComa
SNOMED CT: PEComa, benign (703604002); Perivascular epithelioid tumor, benign (703604002)
 
Monarch Initiative: MONDO:0020581

Definition

A tumor with perivascular epithelioid cell differentiation characterized by the absence of pleomorphism and scarcity or absence of mitotic figures. [from NCI]

Professional guidelines

PubMed

The Role of Interventional Radiology Techniques in the Management of Renal Angiomyolipomas.
Kiefer RM, Stavropoulos SW
Curr Urol Rep 2017 May;18(5):36. doi: 10.1007/s11934-017-0687-6. PMID: 28299630
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197
Diagnosis and management of solid benign liver lesions.
Belghiti J, Cauchy F, Paradis V, Vilgrain V
Nat Rev Gastroenterol Hepatol 2014 Dec;11(12):737-49. Epub 2014 Sep 2 doi: 10.1038/nrgastro.2014.151. PMID: 25178878

Recent clinical studies

Etiology

Clinical and ultrasound features of uterine perivascular epithelioid cell tumors: case series and literature review.
Wang R, Luo H, Cao W
Ultrasound Obstet Gynecol 2024 Nov;64(5):687-695. Epub 2024 Oct 12 doi: 10.1002/uog.29116. PMID: 39395194

Diagnosis

Clinical and ultrasound features of uterine perivascular epithelioid cell tumors: case series and literature review.
Wang R, Luo H, Cao W
Ultrasound Obstet Gynecol 2024 Nov;64(5):687-695. Epub 2024 Oct 12 doi: 10.1002/uog.29116. PMID: 39395194
Perivascular epithelioid cell tumor (PEComa) of the cystic duct.
Okamoto T, Sasaki T, Takahashi Y, Takamatsu M, Kanda H, Hiratsuka M, Matsuyama M, Ozaka M, Sasahira N
Clin J Gastroenterol 2023 Feb;16(1):87-95. Epub 2022 Oct 30 doi: 10.1007/s12328-022-01730-5. PMID: 36309916
Recurrence of retroperitoneal localized perivascular epithelioid cell tumor two years after initial diagnosis: case report.
Cihan YB, Kut E, Koç A
Sao Paulo Med J 2019 Mar-Apr;137(2):206-208. Epub 2018 Jan 15 doi: 10.1590/1516-3180.2017.0120050717. PMID: 29340502Free PMC Article

Clinical prediction guides

Clinical and ultrasound features of uterine perivascular epithelioid cell tumors: case series and literature review.
Wang R, Luo H, Cao W
Ultrasound Obstet Gynecol 2024 Nov;64(5):687-695. Epub 2024 Oct 12 doi: 10.1002/uog.29116. PMID: 39395194

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