Sunitinib Treatment for Metastatic Renal Cell Carcinoma in Patients with Von Hippel-Lindau Disease
A 44-year-old male patient presented to the hospital with left flank pain four years ago. Abdominal CT scan showed bilateral RCC and the patient subsequently received left radical nephrectomy and right partial nephrectomy. Pathology of the samples showed clear cell RCC in both kidneys. His older sister had been diagnosed with VHL disease and the patient also had several pancreatic cysts and VHL gene mutation (3p26-p25) resulting in a diagnosis of VHL disease. Three years prior to admission, the patient was found to have lung metastases in the right middle and lower lobes and underwent wedge resection of the right middle and lower lobes. Chest CT scan performed during post-operative follow up showed enlarged right hilar lymph nodes along with a metastatic mass invading into the superior vena cava. The patient was started on sunitinib treatment (50 mg, fourweek treatment with a two-week withdrawal period). After three months of treatment with sunitinib, the patient achieved partial response according to RECIST criteria (). Following treatment with sunitinib for one year and 11 months, the patient's abdominal and chest CT scan showed disease progression and the treatment was changed to everolimus. The patient is currently receiving treatment with everolimus for two months ().