Discussion:
Member of the metanephric tumor family, which also includes metanephric adenofibroma and metanephric stromal tumor
Most tumors have BRAF gene mutation
Benign tumor composed of cytologically bland embryonic looking epithelium with frequent psammomatous calcifications
Can be seen in patients from pediatric to older adult age
Considered to arise from different forms of maturation arrested embryonal rests, persistent blastema or maturing nephroblastoma due to its resemblance to nephrogenic rests / maturing Wilms tumor
Usually discovered incidentally with no presenting symptoms
If presenting with symptoms, it can demonstrate hematuria, pyrexia, flank pain, abdominal mass and polycythemia
Positive Stains: WT1, CD57, CD56 (Focal) and PAX8
Negative Stains: AMACR, CK7