What is the prevalence of translocation-associated renal cell carcinomas (RCCs)?

Updated: May 17, 2017
  • Author: Angela J Wu, MD; Chief Editor: Liang Cheng, MD  more...
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Answer

Answer

There have been approximately 160 reported cases of Xp11.2 translocation-associated renal cell carcinoma (RCC); this subtype occurs predominantly in children and young adults. The mean age in published cases is approximately 25 years; the median age is 20 years. [1, 2] This subtype now represents a significant proportion of RCC cases in pediatric patients, although widely divergent incidences have been reported in studies (20–83%). [3, 4, 5, 6, 7, 8]

In addition, Xp11.2 translocation-associated carcinoma is now being increasingly reported in patients over age 40 years. [9] There is a predilection for female patients (in published series, the female-to-male ratio is approximately 2:1) and for African American patients. [1, 2, 10]

Transcription factor EB (TFEB) RCC is much rarer, with only 12 reported cases in the literature; therefore, less is known about its epidemiology. The mean age in reported cases is 25 years; the median age is 19 years. [1, 2] There is no evidence for either gender or racial predilection.


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