TY - JOUR
T1 - VCL-ALK renal cell carcinoma in children with sickle-cell trait
T2 - The eighth sickle-cell nephropathy?
AU - Smith, Nathaniel E.
AU - Deyrup, Andrea T.
AU - Mariño-Enriquez, Adrian
AU - Fletcher, Jonathan A.
AU - Bridge, Julia A.
AU - Illei, Peter B.
AU - Netto, George J.
AU - Argani, Pedram
PY - 2014
Y1 - 2014
N2 - 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.
AB - 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.
KW - Anaplastic lymphoma kinase
KW - Renal cell carcinoma
KW - Vinculin
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U2 - 10.1097/PAS.0000000000000179
DO - 10.1097/PAS.0000000000000179
M3 - Article
C2 - 24698962
AN - SCOPUS:84900847567
SN - 0147-5185
VL - 38
SP - 858
EP - 863
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
IS - 6
ER -