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Clinical Presentation
The classic triad of symptoms of renal cancer are flank pain, palpable abdominal mass, and gross hematuria. Other symptoms include anemia, fever, hepatic dysfunction, loss of appetite, malaise, night sweats, weight loss and varicocele.
History
A detailed history should be elicited. A thorough review of medical records should include the number and frequency of episodes of symptoms and previous imaging studies, interventions, evaluations and treatments. The family history with first- or second-degree relatives should be asked. It may also be appropriate to ask for the sexual and psychosexual history of the patient.
Physical Examination
Since most of the patients present with metastatic disease, physical examination of the lungs, bone, liver, cutaneous sites and the central nervous system should be thoroughly evaluated. Check the patients for hypertension, supraclavicular adenopathy, and flank or abdominal mass with bruit. The presence of varicocele and paraneoplastic syndromes (eg hypercalcemia, erythrocytosis and Stauffer syndrome) should raise suspicion. If patients present with gross hematuria with vermiform clots, consider upper urinary tract bleeding.
Screening
For patients with confirmed hereditary RCC without radiographic or
pathologic diagnosis, screening should start 10 years prior to the earliest age
at which a family member was diagnosed.
The frequency of imaging may be increased once the lesions have been
detected based on growth rate and lesion size. Abdominal MRI (preferred) or CT
scan with and without IV contrast may be used with and without contrast depending
on tumor type as follows:
- BAP1-tumor predisposition syndrome (BAP1-TPDS): Every 2 years starting at 30 years of age
- BHDS: Every 3 years starting at 20 years of age
- HLRCC: Annually starting at 8-10 years of age
- HPRC: Every 1-2 years starting at 30 years of age
- PGL/PCC: Every 2 years starting at 12 years of age
- TSC: Every 1-3 years starting at 12 years of age
- VHL: Every 2 years starting at 15 years of age, to assess the kidneys, pancreas and adrenals