VCL-ALK renal cell carcinoma in children with sickle-cell trait: the eighth sickle-cell nephropathy?
case_report · Level V
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- Record sourced from PubMed, PMID 24698962.
- Also identified by DOI 10.1097/PAS.0000000000000179 and PMC identifier 4352307.
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Abstract
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, ALK gene rearrangement by fluorescence in situ hybridization, and a specific VCL-ALK gene fusion by reverse transcriptase polymerase chain reaction. VCL-ALK renal cell carcinoma may represent the eighth sickle-cell nephropathy.
Medical subject headings
- Biomarkers, Tumor
- Carcinoma, Renal Cell
- Gene Fusion
- Kidney Neoplasms
- Receptor Protein-Tyrosine Kinases
- Sickle Cell Trait
- Vinculin