A Primary Adrenal Epithelioid Angiomyolipoma (PEComa) in a Patient with Tuberous Sclerosis Complex: Report of a Case and Review of the Literature

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...

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Bibliographic Details
Published in:Case reports in medicine 2020, Vol.2020 (2020), p.1-4
Main Authors: Kinaan, Mustafa, Comba, Isin Yagmur, Mosquera, Jorge Esteban, Torres Luna, Nancy, Otoya, Jorge
Format: Article
Language:English
Subjects:
Abdomen
Adrenal glands
Angiomyolipoma
Back pain
Case Report
Case reports
Diagnosis
Health aspects
Literature reviews
Liver
Liver diseases
Lymphatic system
Magnetic resonance imaging
Medical prognosis
Melanoma
Mesenchyme
Metastases
Metastasis
Patients
Smooth muscle
Stem cells
Tuberous sclerosis
Tumors
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Summary: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.
ISSN:1687-9627
1687-9635
DOI:10.1155/2020/5131736