...we present a case of a 49 year old man with an aggressive renal cell carcinoma bearing a novel pathogenic KAT6A::NRG1 fusion….A repeat CT Abdomen pelvis taken after 4 months of treatment with Ipilimumab-Nivolumab showed enlargement of the para-aortic lymph node to 3.6cm, increasing to 4.3cm on MRI at 6 months. This 72% increase in size from 2.5cm to 4.3 cm indicates disease progression despite on combination IPI-NIVO therapy.