Commonly metastasizes to lung: lung is only site of metastasis in 27% of cases
Urothelial Cell Carcinoma (Transitional Cell Carcinoma) of the Renal Pelvis (see Urothelial Cell Carcinoma, [[Urothelial Cell Carcinoma]])
Epidemiology: account for 8% of primary renal neoplasms
Physiology: originate from
Metastasis: xxx
Sarcoma
Epidemiology: rare
Wilm’s Tumor (Nephroblastoma)
Epidemiology: accounts for 5-6% of all primary renal neoplasms
Occurs in children
Clinical Manifestations of Renal Cell Carcinoma
General Features
Asymptomatic: many cases
25% of cases have either advanced local/regional disease or distant metastases on presentation
Classic symptom triad of flank pain, hematuria, and palpable renal mass is present in only 9% of cases: when present, it usually indicates locally advanced disease
Mechanism: kidney tumor obstructs the gonadal vein where it enters the renal vein
Clinical: varicocele fails to empty in the recumbent position
Paraneoplastic Syndromes
General Features: may occur due to the ectopic production of erythropoietin, parathyroid hormone-related protein (PTHrp), gonadotropins, human chorionic somatomammotropin, an ACTH-like substance, renin, glucagon, and insulin
AA (Secondary) Amyloidosis (see Amyloidosis, [[Amyloidosis]]): occurs in 3-5% of cases
Mechanism: reflects a chronic inflammatory response (as the amyloid fibrils are composed of fragments of the acute phase reactant serum amyloid A protein)
Anemia (see Anemia, [[Anemia]]): reported in 29-88% of patients with advanced disease
May precede the diagnosis of renal cell carcinoma you several months
Degree of anemia may be disproportionately severe
Anemia can be microcytic or normocytic
Iron studies are consistent with anemia of chronic disease
Cachexia
Erythrocytosis (see Polycythemia, [[Polycythemia]]): occurs in 1-5% of cases
Mechanism
Constitutive production of erythropoietin
Mutated von Hippel-Lindau protein -> impaired degradation of hypoxia-inducible transcription factors under normoxic conditions
Fever (see Fever, [[Fever]]): occurs in up to 20% of cases
Usually intermittent
May be accompanied by night sweats, anorexia, weight loss, and fatigue
Hypercalcemia (see Hypercalcemia, [[Hypercalcemia]]): occurs in 15% of cases
Mechanisms: lytic bone metastases, parathyroid hormone-related protein (PTHrp) production, increased IL-6 (which enhances effect of PTHrp), and prostaglandin-induced enhancement of bone resorption
Polymyalgia Rheumatica-Like Syndrome (see Polymyalgia Rheumatica, [[Polymyalgia Rheumatica]])
Epidemiology: cases have been reported
Treatment
Does not respond to prednisone treatment (in contrast to idiopathic polymyalgia rheumatica)
Nephrectomy appears to be effective
Stauffer’s Syndrome: hepatic dysfunction in renal cell carcinoma in the absence of liver metastases
Mechanism: may be due to tumor production of GM-CSF and IL-6
Elevated Alkaline Phosphatase: occurs in 21% of cases
Frequently associated with fever, weight loss, fatigue, and a poor prognosis
Nephrectomy may result in the improvement in the hepatic dysfunction
Recurrent liver function test elevations in such patients may signal the onset of local recurrence or distant metastases
Thrombocytosis (see Thrombocytosis, [[Thrombocytosis]]): rare in renal cell carcinoma
Mechanism: may be due to tumor synthesis of IL-6
Presence is associated with a poor prognosis
Metastases
Inferior Vena Cava (IVC) Involvement
May result in IVC obstruction with lower extremity edema, ascites, Budd-Chiari syndrome, or pulmonary embolism