Multinucleated Giants: Unveiling Pediatric Renal Epithelioid PEComa.

• Tác giả: Aileen Azari-Yam, Moeinadin Safavi, Mohammad Vasei
• Ngôn ngữ: eng
• Ký hiệu phân loại: 025.3177 Bibliographic analysis and control
• Thông tin xuất bản: England : Fetal and pediatric pathology , 2025
• Bộ sưu tập: NCBI
• ID: 493360

Tóm tắt

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.