Synonyms
t(6;11) renal cell carcinoma; Xp11 translocation renal cell carcinoma
Definition
The new category of MiT family translocation renal cell carcinoma has been included into the World Health Organization (WHO) classification in 2016. The MiT family renal cell carcinoma comprises two different entities: Xp11 translocation renal cell carcinoma harboring TFE3 gene fusions and t(6;11) renal cell carcinoma harboring a MALAT1-TFEB gene fusion.
Clinical Features
Incidence
Xp11 translocation renal cell carcinoma comprises 20–75% of childhood renal cell carcinoma and 1–4% of adult renal cell carcinoma. T (6;11) renal cell carcinoma is an extremely rare variant, less common than the Xp11 renal cell carcinomas; approximately 60 cases documented in the literature.
Age
Although the initial descriptions were in children, Xp11 translocation and t(6,11) renal cell carcinoma may occur in adults in the age range of other renal cell carcinomas.
Sex
There is no gender predilection.
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Argani, P. (2015). MiT family translocation renal cell carcinoma. Seminars in Diagnostic Pathology, 32, 103–113.
Argani, P., Olgac, S., Tickoo, S. K., et al. (2007). Xp11 translocation renal cell carcinoma in adults: Expanded clinical, pathologic, and genetic spectrum. The American Journal of Surgical Pathology, 31, 1149–1160.
Caliò, A., Brunelli, M., Segala, D., et al. (2018). t(6;11) renal cell carcinoma: A study of seven cases including two with aggressive behavior, and utility of CD68 (PG-M1) in the differential diagnosis with pure epithelioid PEComa/epithelioid angiomyolipoma. Modern Pathology, 31, 474–487.
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Caliò, A., Segala, D., Martignoni, G. (2020). MiT Family Translocation Renal Cell Carcinoma. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4847
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DOI: https://doi.org/10.1007/978-3-030-41894-6_4847
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