Renal cell carcinoma (RCC) includes a number of distinct subtypes derived from the various parts of the nephron, each with a unique genetic basis and tumor biology.
RCC remains primarily a surgical disease—it is still considered the paradigm of the chemorefractory tumor; and although immune-based and targeted molecular approaches have shown promise, overall response rates remain low.
The individual approach to each case has been modified by the greater incidence of smaller lesions noted at initial diagnosis and the advent of laparoscopic surgery which provides different treatment options.
The molecular pathology of these lesions is better understood, yet advanced lesions remain difficult to treat by conventional cytoreductive or biological response modifier therapy.
.The incidence of RCC is .increasing; and despite a trend toward earlier detection, mortality rates remain high.
RCC, which accounts for 2% to 3% of all adult malignant neoplasms, is the most lethal of the common urologic cancers.
Traditionally, 30% to 40% of patients with RCC have died of their cancer, in contrast to the 20% mortality rates associated with prostate and bladder carcinomas.
Approximately 54,000 new diagnoses of RCC are made each year in the United States, and 13,000 patients die of disease.
Approximately 12 new cases are diagnosed per 100,000 population per year, with a male-to-female predominance of 3 : 2
This is primarily a disease of the elderly patient, with typical presentation in the sixth and seventh decades of life.
Incidence rates are 10% to 20% higher in African Americans for unknown reasons.
The majority of cases of RCC are believed to be sporadic; only 2% to 3% are familial.
The incidence of RCC has increased since the 1970s by an average of 3% per year for whites and 4% per year for African-Americans, largely related to the more prevalent use of ultrasonography and CT for the evaluation of a variety of abdominal complaints.
This trend has correlated with an increased proportion of incidentally discovered and localized tumors and with improved 5-year survival rates for patients with this stage of disease.
It has been reported that the incidence of advanced tumors per unit population has also increased; and although the proportion of advanced tumors has decreased, the mortality rate per unit population has still been negatively affected.
This suggests that a deleterious change in tumor biology may have occurred during the past several decades, perhaps related to tobacco use, dietary factors, or exposure to other carcinogens.
RCC has a mean age at presentation in children is 8 to 9 years, and the incidence is similar in boys and in girls. Although Wilms tumor is much more common in younger children, RCC is as common as Wilms tumor during the second decade of life.
RCC in children and young adults is more likely to be symptomatic and to exhibit papillary histology, and a predilection for locally advanced, high-grade disease, and unfavorable histologic subtypes has also been reported.
Most studies suggest that stage for stage, children and young adults with RCC may respond better to surgical therapy, and a number of long-term survivors have been reported after radical nephrectomy and lymphadenectomy for lymph node–positive diseas.
An aggressive surgical approach with formal lymphadenectomy has thus been recommended at the time of radical nephrectomy when RCC is suspected in children or young adults
25% of tumors present with advanced disease
Etiology (Risk factors)
The most generally accepted environmental risk factor for RCC is tobacco exposure, although the relative associated risks have been modest, ranging from 1.4 to 2.5 compared with controls.
Obesity is another major risk factor for RCC with an increased relative risk of 1.07 for each unit of rising body mass index.
The increased prevalence of obesity likely contributes to the increased incidence of RCC in Western countries, and it has been estimated that more than 40% of cases of RCC in the United States may be causally linked to obesity.
Hypertension appears to be the third major etiologic factor for RCC.
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