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A Primary Adrenal Epithelioid Angiomyolipoma (PEComa) in a Patient with Tuberous Sclerosis Complex: Report of a Case and Review of the Literature
Joint Authors
Torres Luna, Nancy
Mosquera, Jorge Esteban
Comba, Isin Yagmur
Kinaan, Mustafa
Otoya, Jorge
Source
Issue
Vol. 2020, Issue 2020 (31 Dec. 2020), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2020-03-17
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
Epithelioid angiomyolipomas (EAMLs) are mesenchymal tumors that are part of the family of the perivascular epithelioid cell neoplasms (PEComas).
These tumors portray a potential aggressive behavior with metastatic lesions found in around 30% of reported cases.
EAMLs might present sporadically or in association with the tuberous sclerosis complex (TSC).
They typically involve the kidneys, liver, and lungs.
It is extremely rare for these tumors to arise from other organs.
The present report describes an unusual case of an adult patient with a history of TSC who developed EAML of the adrenal gland.
Moreover, he presented with metastatic disease to the liver, a feature rarely described.
The diagnosis of EAMLs can be challenging as they are hard to distinguish from other adrenal or renal tumors without a thorough histopathologic and immunohistochemical evaluation.
Due to the potential aggressive behavior of these malignancies, timely diagnosis is extremely important and has significant therapeutic and prognostic implications.
American Psychological Association (APA)
Torres Luna, Nancy& Mosquera, Jorge Esteban& Comba, Isin Yagmur& Kinaan, Mustafa& Otoya, Jorge. 2020. A Primary Adrenal Epithelioid Angiomyolipoma (PEComa) in a Patient with Tuberous Sclerosis Complex: Report of a Case and Review of the Literature. Case Reports in Medicine،Vol. 2020, no. 2020, pp.1-4.
https://search.emarefa.net/detail/BIM-1148509
Modern Language Association (MLA)
Torres Luna, Nancy…[et al.]. A Primary Adrenal Epithelioid Angiomyolipoma (PEComa) in a Patient with Tuberous Sclerosis Complex: Report of a Case and Review of the Literature. Case Reports in Medicine No. 2020 (2020), pp.1-4.
https://search.emarefa.net/detail/BIM-1148509
American Medical Association (AMA)
Torres Luna, Nancy& Mosquera, Jorge Esteban& Comba, Isin Yagmur& Kinaan, Mustafa& Otoya, Jorge. A Primary Adrenal Epithelioid Angiomyolipoma (PEComa) in a Patient with Tuberous Sclerosis Complex: Report of a Case and Review of the Literature. Case Reports in Medicine. 2020. Vol. 2020, no. 2020, pp.1-4.
https://search.emarefa.net/detail/BIM-1148509
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1148509