CT scan Image

cut section Image

tumor normal interface Image

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Suspect when variable morphology, difficult to classify Biphasic: clear cell /epithelioid cells with small cell secondary population around hyaline BM deposits Image

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ihc Image

pax2 Image

TFE3 (left) and TFEB (right) Image

this tumor on left, epithelioid angiomyolipoma on right Image

t(6;11) TFEB-ALPHA

Rare (~20 reported cases)

Epidemiology not well characterized – may be similar to Xp11 cases

Most cases low stage at diagnosis, without recurrence following resection