Multinucleated Giants: Unveiling Pediatric Renal Epithelioid PEComa

Area of Science:

• Oncology
• Pediatric Pathology
• Nephrology

Background:

• Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms.
• PEComas of the kidney are exceptionally rare, particularly in pediatric populations.
• Tuberous sclerosis (TS) is associated with an increased risk of PEComas, but this case occurred in a patient without TS.

Purpose of the Study:

• To report a rare case of epithelioid PEComa of the kidney in a pediatric patient.
• To analyze the histological features and clinical presentation of this rare entity.
• To review and contribute to the understanding of pediatric renal PEComas.

Main Methods:

• Case report of a 3-year-old girl diagnosed with right kidney epithelioid PEComa.
• Tumor identification via ultrasound imaging.
• Histopathological examination of radical nephrectomy specimen, including cell morphology and arrangement.
• Literature review of pediatric renal PEComa cases.

Main Results:

• The epithelioid PEComa presented with a distinctive perivascular arrangement of epithelioid and spindle cells, abundant eosinophilic or clear cytoplasm, and multinucleated giant cells.
• The tumor exhibited uncertain malignant potential.
• The patient, despite lacking a history of tuberous sclerosis, is recovering well ten months after nephrectomy with no further therapy indicated.

Conclusions:

• Pediatric renal epithelioid PEComas are rare and can occur in patients without tuberous sclerosis.
• Histopathological features, including the presence of multinucleated giant cells, are important for diagnosis.
• Complete surgical resection appears to be an effective treatment for pediatric renal PEComas with uncertain malignant potential.