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Index case displaying a distinctive pseudo-alveolar pattern was tested for TFE3 immunohistochemistry and gene rearrangements (EHE2). (A, B) Morphologic appearance showing mature vessel lumen formation lined by epithelioid endothelial cells with moderate amount of densely to lightly eosinophilic cytoplasm, focal intra-cytoplasmic vacuoles and stromal inflammation including eosinophils. (C) CD31 and (D) TFE3 strong reactivity.
