Clinical features

Signs and symptoms Haematuria, pain, and flank mass are the classic triad of presenting symptoms, but nearly 40% of patients lack all of these and present with systemic symptoms, including weight loss, abdominal pain, anorexia, and fever {870}. Elevation of the erythrocyte sedimentation rate occurs in approximately 50% of cases {634}. Normocytic anaemia unrelated to haematuria occurs in about 33% {438,902}. Hepatosplenomegaly, coagulopathy, elevation of serum alkaline phosphatase, transaminase, and alpha-2-globulin concentrations may occur in the absence of liver metastases and may resolve when the renal tumour is resected {1441}. Systemic amyloidosis of the AA type occurs in about 3% of patients {2705}.

Renal cell carcinoma may induce para-neoplastic endocrine syndromes {1441,2525}, including humoral hyper-calcemia of malignancy (pseudohyper-parathyroidism), erythrocytosis, hypertension, and gynecomastia. Hyper-calcemia without bone metastases occurs in approximately 10% of patients and in nearly 20% of patients with disseminated carcinoma {736}. In about 66% of patients, erythropoietin concentration is elevated {2526}, but less than 4% have erythrocytosis {902,2526}. Approximately 33% are hypertensive, often with elevated renin concentrations in the renal vein of the tumour-bearing kidney {902,2491}. Gynecomastia may result from gonadotropin {904} or prolactin production {2486}. Renal cell carcinoma also is known for presenting as metastatic carcinoma of unknown primary, sometimes in unusual sites.

Imaging

The current imaging technology has altered the management of renal masses as it enables detection and characterization of very small masses. Radiological criteria established by Bosniak assist management of renal masses {283}. Ultrasonography is useful for detecting renal lesions and if it is not diagnostic of a simple cyst, CT before and after IV contrast is required. Plain CT may confirm a benign diagnosis by identifying fat in angiomyolipoma {284}. Lesions without enhancement require nothing further, but those with enhancement require follow-ups at 6 months, 1 year, and then yearly {258}. Increased use of nephron-sparing and laparoscopic surgery underscores the importance of preoperative imaging work-up. Routine staging work-up for renal cell carcinoma includes dynamic CT and chest radiography.

Familial renal cell carcinoma

F. Algaba B. Zbar

G. Kovacs P. Kleihues

A. Geurts van Kessel M. Kiuru V. Launonen R. Herva L.A. Aaltonen H.P.H. Neumann C.P. Pavlovich

The kidney is affected in a variety of inherited cancer syndromes. For most of them, the oncogene / tumour suppressor gene involved and the respective germline mutations have been identified, making it possible to confirm the clinical diagnosis syndrome, and to identify asymptomatic gene carriers by germline mutation testing {2510}. Each of the inherited syndromes predisposes to distinct types of renal carcinoma. Usually, affected patients develop bilateral, multiple renal tumours; regular screening of mutation carriers for renal and extrarenal manifestations is considered mandatory.

Von Hippel-Lindau disease (VHL)

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