Features

Architecture

• Highly cellular
• Large 3D clusters
• Papillary with dense hyaline cores
• Rare single cells

Cellular features

• Clear cells and granular cells
• Abundant cytoplasm, vacuolated or granular
• Well-defined cell borders
• May have intracytoplasmic inclusion bodies

Nuclear features

• Enlarged, eccentrically located nuclei
• Irregular nuclear membranes
• Prominent nucleoli
• May have intranuclear cytoplasmic inclusions

Background

• May have necrosis
• May have psammoma bodies

Ancillary studies

• IHC: Expresses TFE3 (D/D other RCCs), vimentin, CD10, AMACR, RCC, HMB-45, Melan A; cytokeratin and EMA weak to absent
• Translocation t(X;17) i.e. Xp11.2 translocation resulting in TFE3 gene fusions including ASPL:TFE3 (same as alveolar soft part sarcoma). May have t(X;1) involving PRCC:TFE3 fusion protein