The classic triad of hematuria, flank pain, and flank mass is seen in only 10-20% of pts; hematuria (40%), flank pain (40%), palpable mass (33%), and weight loss (33%) are the most common individual symptoms. Paraneoplastic syndromes of erythrocytosis (3%), hypercalcemia (5%), and nonmetastatic hepatic dysfunction (Stauffer's syndrome) (15%) may also occur. Workup should include IV pyelography, renal ultrasonography, CT of abdomen and pelvis, chest x-ray (CXR), urinalysis, and urine cytology. Stage I is disease restricted to the kidney, stage II is disease contained within Gerota's fascia, stage III is locally invasive disease involving nodes and/or inferior vena cava, stage IV is invasion of adjacent organs or metastatic sites. Prognosis is related to stage: 66% 5-year survival for I, 64% for II, 42% for III, and 11% for IV.
Treatment: Renal Cancer Radical nephrectomy is standard for stage I, II, and most stage III pts. Surgery may also be indicated in the setting of metastatic disease for intractable local symptoms (bleeding, pain). Response rates of 40-48% have been noted with five different single agents, sunitinib (50 mg/d 4 weeks out of 6), sorafenib (400 mg bid), and temsirolimus (25 mg IV weekly) and the related drug, everolimus (10 mg PO daily), the PD1 inhibitor, nivolumab and cabozantinib (60 mg PO daily), an inhibitor of MET, AXL and the vascular endothelial growth factor receptor. Sunitinib and sorafenib are thought to be antiangiogenic through inhibition of kinases in tumor cells. Temsirolimus is an inhibitor of mTOR. Nivolumab allows activation of T cells that kill the tumor cells. About 10-15% of pts with advanced-stage disease may benefit from interleukin 2 and/or interferon α (IFN-α). Addition of bevacizumab to IFN-α improves the response rate. Some remissions are durable. Chemotherapy is of little or no benefit. |
Section 6. Hematology and Oncology