2020 Edition
| Editors: Maria Rosaria Raspollini, Antonio Lopez-Beltran

MiT Family Translocation Renal Cell Carcinoma

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_4847


t(6;11) renal cell carcinoma; Xp11 translocation renal cell carcinoma


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.

  • Site

    There is no...

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References and Further Reading

  1. Argani, P. (2015). MiT family translocation renal cell carcinoma. Seminars in Diagnostic Pathology, 32, 103–113.PubMedCrossRefGoogle Scholar
  2. 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.PubMedCrossRefGoogle Scholar
  3. 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.PubMedPubMedCentralCrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Diagnostic and Public Health, Section of PathologyUniversity of VeronaVeronaItaly
  2. 2.Department of PathologyPederzoli HospitalPeschiera del GardaItaly