TY - JOUR
T1 - Translocation renal cell carcinomas in adults
T2 - A single-institution experience
AU - Zhong, Minghao
AU - De Angelo, Patricia
AU - Osborne, Lisa
AU - Paniz-Mondolfi, Alberto E.
AU - Geller, Matthew
AU - Yang, Youfeng
AU - Linehan, W. Marston
AU - Merino, Maria J.
AU - Cordon-Cardo, Carlos
AU - Cai, Dongming
PY - 2012/5
Y1 - 2012/5
N2 - Translocation renal cell carcinoma is a newly recognized subtype of renal cell carcinoma (RCC) with chromosomal translocations involving TFE3 (Xp11.2) or, less frequently, TFEB (6p21). Xp11 translocation RCC was originally described as a pediatric neoplasm representing 20% to 40% of pediatric RCCs, with a much lower frequency in the adult population. TFEB translocation RCC is very rare, with approximately 10 cases reported in the literature. Here, we describe the clinicopathologic features of adult translocation RCC from a single institution. Using tissue microarray, immunohistochemistry, cytogenetic examination, and fluorescence in situ hybridization, we identified 6 (∼5%) cases of TFE3 translocation RCC and 1 (<1%) case of TFEB translocation RCC in 121 consecutive adult RCC cases between 2001 and 2009. Our results suggest that weak TFE3 staining of a significant proportion of RCC cases may be because of expression of the full-length TFE3 protein rather than the chimeric fusion protein resulting from chromosomal translocation.
AB - Translocation renal cell carcinoma is a newly recognized subtype of renal cell carcinoma (RCC) with chromosomal translocations involving TFE3 (Xp11.2) or, less frequently, TFEB (6p21). Xp11 translocation RCC was originally described as a pediatric neoplasm representing 20% to 40% of pediatric RCCs, with a much lower frequency in the adult population. TFEB translocation RCC is very rare, with approximately 10 cases reported in the literature. Here, we describe the clinicopathologic features of adult translocation RCC from a single institution. Using tissue microarray, immunohistochemistry, cytogenetic examination, and fluorescence in situ hybridization, we identified 6 (∼5%) cases of TFE3 translocation RCC and 1 (<1%) case of TFEB translocation RCC in 121 consecutive adult RCC cases between 2001 and 2009. Our results suggest that weak TFE3 staining of a significant proportion of RCC cases may be because of expression of the full-length TFE3 protein rather than the chimeric fusion protein resulting from chromosomal translocation.
KW - TFE3
KW - TFEB
KW - translocation renal cell carcinoma
UR - http://www.scopus.com/inward/record.url?scp=84862804721&partnerID=8YFLogxK
U2 - 10.1097/PAS.0b013e31824f24a6
DO - 10.1097/PAS.0b013e31824f24a6
M3 - Article
C2 - 22446944
AN - SCOPUS:84862804721
SN - 0147-5185
VL - 36
SP - 654
EP - 662
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
IS - 5
ER -