VCL-ALKRenal Cell Carcinoma in Children With Sickle-cell Trait

We report the third case of a renal cell carcinoma bearing a fusion of the vinculin (VCL) and anaplastic lymphoma kinase (ALK) genes. Like the 2 other reported cases, this neoplasm occurred in a young patient (6 y old) with sickle-cell trait and demonstrated distinctive morphologic features including medullary epicenter, discohesive polygonal or spindle-shaped cells with prominent cytoplasmic vacuoles, and prominent lymphocytic infiltrate. The neoplastic cells demonstrated focal membranous labeling for ALK protein by immunohistochemistry, ALKgene rearrangement by fluorescence in situ hybridization, and a specific VCL-ALKgene fusion by reverse transcriptase polymerase chain reaction. VCL-ALKrenal cell carcinoma may represent the eighth sickle-cell nephropathy.