Renal cell carcinoma

Renal cell carcinoma (RCC), also known as kidney cancer or hypernephroma, is a malignant tumor that originates in the lining of the proximal convoluted tubule of the kidney. It is the most common type of kidney cancer in adults, accounting for approximately 85-90% of all malignant kidney neoplasms. RCC primarily affects the urinary system but can metastasize to the lungs, bones, liver, brain, and other organs. The disease may present with hematuria (blood in the urine), flank pain, a palpable abdominal mass, unexplained weight loss, fatigue, fever, and night sweats. However, many cases are discovered incidentally during imaging studies performed for other reasons, as early-stage RCC is often asymptomatic. Several histological subtypes exist, including clear cell RCC (the most common, approximately 70-80% of cases), papillary RCC, and chromophobe RCC. While most cases of RCC are sporadic, hereditary forms account for approximately 3-5% of cases and are associated with genetic syndromes such as von Hippel-Lindau (VHL) disease, hereditary papillary renal carcinoma (associated with MET gene mutations), Birt-Hogg-Dubé syndrome (FLCN gene), and hereditary leiomyomatosis and renal cell cancer (FH gene). Sporadic clear cell RCC frequently involves somatic inactivation of the VHL gene on chromosome 3p25. Treatment depends on the stage at diagnosis. Localized disease is typically managed with surgical resection, including partial or radical nephrectomy. For advanced or metastatic RCC, the treatment landscape has evolved significantly with the introduction of targeted therapies (such as tyrosine kinase inhibitors including sunitinib, pazopanib, and cabozantinib, as well as mTOR inhibitors like everolimus) and immune checkpoint inhibitors (such as nivolumab and ipilimumab combinations, or pembrolizumab with axitinib). RCC is notably resistant to conventional chemotherapy and radiation therapy. Prognosis varies widely depending on stage, with early-stage disease having a favorable five-year survival rate, while metastatic disease carries a significantly poorer prognosis.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Renal cell carcinoma