History and exam

Key diagnostic factors

common

asymptomatic (incidental finding)

More than 50% of RCCs are diagnosed incidentally.[1][2]

hematuria

Microscopic/gross hematuria may occur alone or as part of classic triad of flank pain, hematuria, and palpable abdominal mass. This classic presentation occurs in <10% of patients and suggests a larger size of tumor, higher pathologic stage, and worse prognosis.[1][17]

uncommon

flank pain

May occur alone or as part of classic triad of flank pain, hematuria, and palpable abdominal mass. This classic presentation occurs in <10% of patients and suggests a larger size of tumor, higher pathologic stage, and worse prognosis.[1][17]

palpable abdominal mass

May occur alone or as part of classic triad of flank pain, hematuria, and palpable abdominal mass. This classic presentation occurs in <10% of patients and suggests a larger size of tumor, higher pathologic stage, and worse prognosis.[1][17]

Other diagnostic factors

uncommon

nonspecific systemic symptoms

Included in a spectrum of paraneoplastic presentations of RCC, and occur in up to 20% of patients.[18] May include fever, weight loss, sweats, pallor, cachexia, myoneuropathy.

signs of hepatic dysfunction

Included in a spectrum of paraneoplastic presentations of RCC.[18] Includes ascites, hepatomegaly, and spider angiomata.

myoneuropathy

Included in a spectrum of paraneoplastic presentations of RCC.[18]

lower limb edema

Often suggestive of inferior vena cava involvement, but not a common presentation.

scrotal varicocele

RCC is a cause of varicocele in a very small proportion of men.[67]

dermatologic manifestation (hereditary syndromes)

Patients may present with dermatologic signs of hereditary syndrome. Patients with Birt-Hogg-Dube syndrome may have dermatologic papules; and hereditary leiomyomatous patients may have skin fibromas.

vision loss (von Hippel-Lindau)

Patients with the hereditary syndrome von Hippel-Lindau may present with vision loss and retinal angiomatosis detected on fundoscopy.

Risk factors

strong

smoking

Has been consistently shown to be the most well-established modifiable risk factor for RCC in both men and women.[27][30]​​ It is implicated in 20% to 30% of renal cancers in men, and 10% to 20% in women.[26]

In one study, smokers with more than 22.5 pack-years of exposure had a greater than 50% increased risk of RCC, compared with those who never smoked.[42] The overall relative risk of RCC is 1.31 for all (current and former) smokers.[43]

male sex

The incidence in males is twice that of females in most populations studied.[22]

age over 55 years

Median age at diagnosis is 65 years.[19]

More than 75% of patients are >55 years at diagnosis.[19]

residence in developed countries

RCC has a higher incidence and prevalence in Europe and North America.[27]​​

non-Hispanic American Indian/Alaska Native ethnicity

In the US, the incidence and mortality rates for kidney cancer are highest among non-Hispanic American Indian/Alaska Native persons (39.5 new cases per 100,000 persons [2017-2021, age-adjusted] and 10.1 deaths per 100,000 persons [2018-2022 age-adjusted], respectively).[19][21]

obesity

Associated with increased risk for RCC.[27][30]​​​​ In one study, individuals with a BMI ≥35 had a 71% increased risk of RCC compared with normal weight individuals (BMI <25).[42]

There is some evidence to suggest that patients with RCC with obesity may have improved survival outcomes compared with patients with RCC without obesity.[44]​ The association of body composition with clinical outcomes is being evaluated in a multicenter prospective cohort study.[45]

hypertension

The risk of RCC increases with systolic and diastolic hypertension, and decreases with lower blood pressures.[46] Elevated systolic and diastolic blood pressures have been associated with a two- to threefold increased RCC risk, independent of sex, BMI, smoking, and use of antihypertensives.[30]

positive family history of RCC

A positive family history of renal cancer carries a 2.8- to 4.3-fold increased risk for RCC.[47]

Familial non-syndromic RCC is suspected when two or more relatives have RCC and there are no features to suggest an underlying "syndromic cause."[33]​​​​ Early-onset tumors and/or multiple/bilateral tumors increase suspicion of familial RCC, and genetic testing is usually performed.[33] Cases without an identifiable genetic cause are likely to be genetically heterogeneous.[33]

history of hereditary syndromes

Familial RCC is usually inherited in an autosomal dominant manner and accounts for 3% to 5% of RCC.[48][49]​​

Von Hippel-Lindau syndrome is the most common syndrome associated with clear cell RCC.[33] Type 1 papillary kidney cancers have mutations in the mesenchymal-epithelial transition factor (MET) gene and type 2 as part of hereditary leiomyomatosis and kidney cell cancer having fumarate hydratase (FH) mutations.[33] Chromophobe and oncocytic kidney cancers are predominantly associated with Birt-Hogg-Dubé syndrome, which has a folliculin (FLCN) mutation.[33] Tuberous sclerosis may be associated with early-onset RCC, but RCC is rare in this condition and renal lesions are most commonly angiomyolipomas.[33] 

history of acquired renal cystic disease

Acquired polycystic kidneys, as seen most commonly in those with chronic kidney disease requiring dialysis, may increase the risk of RCC by 30 to 50 times.[50][51]

weak

asbestos/cadmium exposure

The role of occupational exposures is questionable.[30] Evidence suggests asbestos and cadmium exposures may portend a higher risk.[52][53]

obstetric history/estrogen exposure

Reproductive factors (e.g., parity, age at first birth) have been associated with risk for renal cell carcinoma; however, study findings are not universally consistent.[54][55][56][57]​​​​​​

There is evidence to suggest that hormone replacement therapy is inversely associated with risk for renal cell carcinoma in women.[58]​ One cohort study found that women who reported hysterectomy appeared to be at increased risk for renal cell carcinoma.[54]

pelvic radiation

A small increase in RCC has been found in patients who have received ionizing radiation therapy.[26]

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