PMID- 23426439 OWN - NLM STAT- MEDLINE DCOM- 20131119 LR - 20161214 IS - 1699-5848 (Electronic) IS - 0213-3911 (Linking) VI - 28 IP - 6 DP - 2013 Jun TI - Review of renal carcinoma with t(6;11)(p21;q12) with focus on clinical and pathobiological aspects. PG - 685-90 LID - 10.14670/HH-28.685 [doi] AB - Recently, a new category of MiTF/TFE family translocation carcinomas of the kidney has been proposed. This category includes Xp11.2 renal cell carcinoma (RCC) and the t(6;11) RCC. These tumors share clinical, morphological, immunohistochemical and molecular genetic features. In this article, we review t(6;11) RCC. This tumor predominantly affects children and young adults. Macroscopically, the tumor generally forms a well circumscribed mass. Satellite nodules may be observed. Histologically, the tumor comprises large cells and small cells surrounded by basement membrane material. Immunohistochemically, tumor cells show nuclear immunolabeling for TFEB and usually express Cathepsin-K in the cytoplasm. Karyotyping detects the rearrangement between chromosome 6p21 and chromosome 11q12. Alpha-TFEB fusion can be detected by reverse transcriptase polymerase chain reaction (RT-PCR) or fluorescence in situ hybridization (FISH). Most cases affecting children and young adults seem to be indolent, but some adult cases have presented with metastasis or caused death. As previously reported cases remain limited to date, further examination in a large scale study will be needed in order to elucidate clinical behavior and molecular characteristics. FAU - Kuroda, Naoto AU - Kuroda N AD - Department of Diagnostic Pathology, Kochi Red Cross Hospital, Kochi, Japan. kurochankochi@yahoo.co.jp FAU - Tanaka, Azusa AU - Tanaka A FAU - Sasaki, Naomi AU - Sasaki N FAU - Ishihara, Akira AU - Ishihara A FAU - Matsuura, Keiko AU - Matsuura K FAU - Moriyama, Masatsugu AU - Moriyama M FAU - Nagashima, Yoji AU - Nagashima Y FAU - Inoue, Keiji AU - Inoue K FAU - Petersson, Fredrik AU - Petersson F FAU - Martignoni, Guido AU - Martignoni G FAU - Michal, Michal AU - Michal M FAU - Hes, Ondrej AU - Hes O LA - eng PT - Journal Article PT - Review DEP - 20130221 PL - Spain TA - Histol Histopathol JT - Histology and histopathology JID - 8609357 RN - 0 (Basic Helix-Loop-Helix Leucine Zipper Transcription Factors) RN - 0 (TFEB protein, human) RN - EC 3.4.22.38 (Cathepsin K) SB - IM MH - Basic Helix-Loop-Helix Leucine Zipper Transcription Factors/genetics MH - Carcinoma, Renal Cell/genetics/metabolism/*pathology MH - Cathepsin K/metabolism MH - Cell Nucleus/metabolism/pathology MH - *Chromosomes, Human, Pair 11 MH - *Chromosomes, Human, Pair 6 MH - Gene Fusion MH - Gene Rearrangement MH - Humans MH - In Situ Hybridization, Fluorescence MH - Kidney Neoplasms/genetics/metabolism/*pathology MH - Reverse Transcriptase Polymerase Chain Reaction MH - *Translocation, Genetic EDAT- 2013/02/22 06:00 MHDA- 2013/11/20 06:00 CRDT- 2013/02/22 06:00 PHST- 2013/02/22 06:00 [entrez] PHST- 2013/02/22 06:00 [pubmed] PHST- 2013/11/20 06:00 [medline] AID - HH-11-295 [pii] AID - 10.14670/HH-28.685 [doi] PST - ppublish SO - Histol Histopathol. 2013 Jun;28(6):685-90. doi: 10.14670/HH-28.685. Epub 2013 Feb 21.