A case of von Hippel-Lindau disease with renal cell carcinoma treated by partial nephrectomy with pre- and post-surgical axitinib therapy.

von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary disease with benign and malignant tumors occurring in various organs including the kidneys. In patients with renal cell carcinoma (RCC) lesions in both kidneys, it is difficult to determine the treatment strategy. We report a case of VHL disease with RCC treated via partial nephrectomy after 6 months of axitinib therapy. Then, the patient continued to receive low-dose axitinib therapy without any signs of tumor progression for 3 years after surgery. Axitinib combined with surgery might be a treatment option for patients with VHL disease harboring bilateral RCC.