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Review
. 2024 Jun;15(3):372-381.
doi: 10.14740/wjon1794. Epub 2024 Apr 15.

Comprehensive Insights Into Renal Perivascular Epithelioid Cell Neoplasms: From Molecular Mechanisms to Clinical Practice

Bao Nan Dong  1 , Hui Zhan  1 , Ting Luan  1 , Jian Song Wang  1
Affiliations
Review

Comprehensive Insights Into Renal Perivascular Epithelioid Cell Neoplasms: From Molecular Mechanisms to Clinical Practice

Bao Nan Dong et al. World J Oncol. 2024 Jun.

Abstract

Perivascular epithelioid cell neoplasms (PEComas) are a rare category of mesenchymal tissue tumors, manifesting across various tissues and organs such as the kidneys, liver, lungs, pancreas, uterus, ovaries, and gastrointestinal tract. They predominantly affect females more than males. PEComas characteristically express both melanocytic and smooth muscle markers, making immunohistochemistry vital for their diagnosis. Renal angiomyolipoma (AML) represents a common variant of PEComas, typically marked by favorable prognoses. Nonetheless, only a small fraction of subtypes, especially epithelioid AML, possess the capacity to be malignant. Renal PEComas usually appear as asymptomatic masses accompanied by vague imaging characteristics. The main methods for diagnosis are histopathological analysis and the application of immunohistochemical stains. Presently, a uniform treatment plan for renal PEComas is absent. Strategies for management include active surveillance, selective arterial embolization, surgical procedures, and drug-based treatments. The focus of this review is on renal PEComas, shedding light on their pathogenesis, pathological characteristics, clinical presentations, diagnosis, and treatment modalities, and incorporating a clinical case study.

Keywords: Angiomyolipoma; Etiology; Immunohistochemistry; Perivascular epithelioid cell neoplasms; Therapeutics.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
The computed tomography (CT) and magnetic resonance imaging (MRI) of the patient.
Figure 2
Figure 2
The radical nephrectomy of the right kidney. (a) Adhesion of the right kidney to the descending portion of the duodenum. (b) The kidney and the duodenum after adhesion were released. (c) Adhesion of the right kidney to the inferior vena cava. (d) The kidney and the inferior vena cava after adhesion were released. (e) The lower lobe of the liver after adhesion was released.
Figure 3
Figure 3
The histopathology (a-e) and immunohistochemistry (f-h) of the right renal mass. (f) HMB45 positive, (g) melan-A positive, (h) TFE3 negative.
Figure 4
Figure 4
The case history.
See this image and copyright information in PMC

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