In the past few years, the
immune checkpoint inhibitor (ICI)
nivolumab has become standard of care in the treatment of metastatic
renal cell carcinoma (mRCC) progressing after
antiangiogenic agents. To date, neither expression of programmed death ligand-1 (PD-L1) nor any other
biomarker can be used to predict responses to ICIs, although intermediate-poor International Metastatic Database of
Renal Carcinoma (IMDC) risk patients and those with sarcomatoid
tumors appear to achieve superior benefit from
immunotherapy. Paradoxically, ICIs may sometimes increase the speed of
tumor growth. This rare phenomenon, called hyperprogression, has first been described in patients with
melanoma and
lung cancer treated with ICIs and is associated with poor survival. Here, we present the case of a patient affected by an intermediate IMDC risk mRCC with diffuse sarcomatoid features who achieved long disease control with first-line
sunitinib and then started a second-line treatment with
nivolumab. Unexpectedly, he experienced a dramatic acceleration of
tumor growth and died soon after the third infusion of
nivolumab. Then, we review the frequency of hyperprogression in mRCC and discuss the biological peculiarity of sarcomatoid RCC in terms of different responses to ICIs and
antiangiogenic agents.