Multinucleated Giants: Unveiling Pediatric Renal Epithelioid Pecoma Publisher Pubmed

Summary: Rare kidney tumors can arise even in children without TS. A 3-year-old's case reveals uncertainties in management. Are we prepared to tackle such anomalies? Monitoring ongoing post-surgery. #PediatricOncology #RareDiseases

Azariyam A¹ ; Vasei M² ; Safavi M¹
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.