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Multinucleated Giants: Unveiling Pediatric Renal Epithelioid Pecoma Publisher Pubmed



Azariyam A1 ; Vasei M2 ; Safavi M1
Authors
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Authors Affiliations
  1. 1. Division of Molecular Pathology and Cytogenetics, Pathology Department, Children’s Medical Center, Faculty of Medicine, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Gene Therapy Research Center, Digestive Disease Research Institute, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran

Source: Fetal and Pediatric Pathology Published:2025


Abstract

Perivascular epithelioid cell tumors (PEComas) of the kidney are rare mesenchymal tumors that rarely occur in children. Individuals with tuberous sclerosis (TS) are at increased risk for these tumors. While classic PEComas are benign, the epithelioid variant could have malignant potential and metastasis capacity. We report a case of right kidney epithelioid PEComa with uncertain malignant potential in a 3-year-old girl who had no personal or family history of tuberous sclerosis. The tumor was found through ultrasound imaging. Sections from the radical nephrectomy specimen showed epithelioid and spindle cells with abundant granular eosinophilic or clear cytoplasm, mildly pleomorphic vesicular nuclei and distinctive perivascular arrangement. Abundant multinucleated giant cells were seen. No further therapy was suggested by the oncologist. The patient is doing well ten months post-surgery. We reviewed the literature and analyzed the features of pediatric renal PEComas reported so far. © 2025 Taylor & Francis Group, LLC.