According to the S3 guideline renal cell carcinoma (S3-Leitlinie Nierenzellkarzinom) and international guidelines [49] nivolumab can be used as the first-line medicament in combination with other medicaments (ipilimumab, cabozantinib) or as the second-line therapy after the failure of the VEGF/R- or mTOR-based treatment. Here, MTOR is linked to hereditary clear cell renal cell carcinoma.