Abstract
Von Hippel Lindau (VHL) disease is known to be associated to be bilateral renal cell carcinoma (RCC) and death before the age of 40 if untreated. VHL associated with renal cell carcinoma and Inferior Vena-caval (IVC) thrombus is uncommon with only few cases reported in the literature so far. We report a 37 year old male who presented with gross hematuria and was subsequently found to have bilateral renal cell carcinoma with level 2 IVC thrombus necessitating IVC thrombectomy and bilateral synchronous nephrectomy needing long term hemo-dialysis. The patient had good post-operative outcome and was positive for VHL gene. Patient was well on hemo-dialysis and no evidence of metastatic recurrence after one year of follow-up and is awaiting kidney transplant. Patients with bilateral synchronous RCC with IVC thrombus with good performance status and absence of metastatic disease should be treated aggressively.
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