From Cell Biology to Tissue Engineering



MiT family translocation renal cell carcinomas: A 15th anniversary update

Jatin S. Gandhi1*, Faizan Malik1*, Mahul B. Amin1, Pedram Argani2 and Armita Bahrami1,3

1Department of Pathology, University of Tennessee Health Science Center, Memphis, TN, 2Department of Pathology, John Hopkins University, Baltimore and 3Departments of Pathology and Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA
*These authors have contributed equally to this work

Offprint requests to: Armita Bahrami, MD, Associate Professor, Pathology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, MS 250, Memphis, TN 38105-3678, USA. e-mail: armita.bahrami@stjude.org

Summary. Microphthalmia (MiT) family translocation renal cell carcinomas (RCCs) are a heterogeneous category of renal tumors which all express MiT transcription factors, typically from chromosomal translocation and rarely from gene amplification. This tumor family has two major subtypes [i.e., Xp11 translocation RCC and t(6;11) RCC] and several related neoplasms (i.e., TFEB amplification RCC and melanotic Xp11 translocation renal cancers). Increased understanding of the clinical, pathological, molecular and prognostic heterogeneity of these tumors, since their official recognition in 2004, provides the opportunity to identify prognostic biomarkers and to understand the reasons for tumor aggression. We will review the literature from the past 15 years and highlight the need for a greater understanding of the molecular mechanisms underpinning heterogeneous tumor behavior. Histol Histopathol 35, 125-136 (2020)

Key words: Renal cell carcinoma, Translocation renal cell carcinoma, TFE3, TFEB

DOI: 10.14670/HH-18-159