Von Hippel-Lindau syndrome with bilateral renal and an interaortocaval mass

Patients with bilateral renal masses and an interaortocaval mass (IACM) present a complex scenario where, in addition to renal masses, management of the IACM adds to challenges in an already challenging case. A young man in his 30s presented with left flank pain, associated with loss of appetite, headache and sweating. The evaluation revealed multiple renal masses in the right kidney, a large mass in the left kidney and an IACM. Staged surgery revealed bilateral renal masses to be clear cell renal cell carcinoma (RCC) and the IACM as paraganglioma (PG). In bilateral RCC, should there be a retroperitoneal mass, PG should be strongly considered and ruled out in addition to the evaluation of Von Hippel-Lindau syndrome.