CHEK2 mutations have been noted in bone, brain, breast, colon, lung, thyroid, and prostate cancer. Although now reported in both clear cell and non-clear cell renal cancer, we have not found CHEK2 2 mutations reported in the papillary type II subtype (PRCC). Here, we report a 63-year-old female with a PRCC type II with a concomitant CHEK2 C1100del mutation, who is currently in complete remission three years post tumor resection.
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