Sunitinib Treatment for Metastatic Renal Cell Carcinoma in Patients with Von Hippel-Lindau Disease
A 42-year-old female was found to have masses in both kidneys four years ago and was transferred to Asan Medical Center. Her family history included VHL disease in her father and her older sister. She also had hemangioblastoma in the cerebellum, which was removed 16 years ago. Abdominal computed tomography (CT) scan performed at this hospital showed masses in both kidneys which were suspected to be RCC; thus, the patient underwent partial nephrectomy of the left kidney. Two months later, the patient underwent radical nephrectomy of the right kidney. Pathology of the samples revealed clear cell RCC in both kidneys. The patient developed local recurrent RCC in the left kidney two years after nephrectomy and was subsequently treated by radiofrequency ablation. The patient then developed multiple metastases in the lungs and was transferred to the oncology department.\nChest CT subsequently confirmed multiple metastases in both lung fields and the patient was diagnosed with lung metastases of RCC and started treatment of sunitinib (50 mg for two weeks, followed by a one-week rest period). The metastatic tumors were found to have regressed following sunitinib administration and reached partial response according to the Response Evaluation Criteria in Solid Tumors (RECIST) criteria (). After administration of sunitinib for 16 months, the patient suffered hand and foot neuropathy (grade 2), and wanted a dose reduction; we decided on a dose reduction of sunitinib (25 mg for two weeks, followed by a one-week rest period). After that, this patient became tolerable to treatment. This partial response has been maintained at a steady state for three years and three months with sunitinib administration. No changes in pancreatic mass lesions or hemangioblastoma of the cerebellum during administration of sunitinib were observed during that period of time (). After that, a chest CT scan showed disease progression in the lung; therefore, the patient's treatment was changed to everolimus (Afinitor, Novartis, Basel, Switzerland). Chest CT at two months of treatment with everolimus showed progression of the disease, therefore, the medication was changed again to sorafenib (Nexavar, Bayer, Leverkusen, Germany). The patient also achieved partial response to sorafenib and is currently receiving treatment with sorafenib for five months.