There is still no standard treatment for non-clear cell
renal cell carcinomas.
Sunitinib is the most examined
drug because of its effectiveness in retrospective studies and clinical trials, and is the preferred first-line
drug in the National Comprehensive
Cancer Network guideline.
Temsirolimus is an option as a first-line
drug, especially for poor-risk non-
clear cell renal cell carcinoma patients.
Everolimus,
pazopanib,
axitinib and nivolmab might also be viable options. Clinical trials are still required to gather evidence regarding non-
clear cell renal cell carcinoma treatment. Because each non-
clear cell renal cell carcinoma has a different genetic background and molecular features, specific treatment for each non-
clear cell renal cell carcinoma should be established. From the results of a Japanese multicenter study,
tyrosine kinase inhibitors might be better used for metastatic
papillary renal cell carcinoma in both first- and second-line settings. Both
tyrosine kinase inhibitors and mammalian target of rapamicin inhibitors are effective for metastatic
chromophobe renal cell carcinoma, but the preferred first-line
drug has not been determined.
Platinum-based
chemotherapies are currently recommended for metastatic
collecting duct carcinoma, and
anti-angiogenic drugs are effective in some cases.
Tyrosine kinase inhibitors, especially
sunitinib, appear to be effective for X11.2 translocation
renal cell carcinoma among the
microphthalmia-associated transcription family of translocation
renal cell carcinomas. Evidence is still lacking regarding the treatment for other rare non-clear cell
renal cell carcinomas. Appropriate sequential
therapies using antivascular
endothelial growth factor therapies, mammalian target of rapamicin inhibitors and immuno-oncology drugs should be established for each non-
clear cell renal cell carcinoma.