Renal cell carcinoma (RCC, also known as hypernephroma) is a kidney cancer that originates in the lining of the proximal convoluted tubule, the very small tubes in the kidney that filter the blood and remove waste products.
RCC is the most common type of kidney cancer in adults, responsible for approximately 80% of cases.
It is also known to be the most lethal of all the genitourinary tumors.
Initial treatment is most commonly a radical or partial nephrectomy and remains the mainstay of curative treatment.
Where the tumour is confined to the renal parenchyma, the 5-year survival rate is 60-70%, but this is lowered considerably where metastases have spread.
It is resistant to radiation therapy and chemotherapy, although some cases respond to immunotherapy.
Targeted cancer therapies such as sunitinib, temsirolimus, bevacizumab, interferon-alpha, and possibly sorafenib have improved the outlook for RCC (progression-free survival), although they have not yet demonstrated improved survival.
Recent genetic studies have altered the approaches used in classifying
renal cell carcinoma. The following system can be used to classify these
- Clear cell carcinoma (VHL and others on chromosome 3)
- Papillary carcinoma (MET, PRCC)
- Chromophobe renal carcinoma
- Collecting duct carcinoma
Renal epithelial neoplasms have characteristic cytogenetic
aberrations that can aid in classification. See also Atlas of Genetics
and Cytogenetics in Oncology and Haematology.
- Clear cell carcinoma: loss of 3p
- Papillary carcinoma: trisomy 7, 16, 17
- Chromophobe carcinoma: hypodiploid with loss of chromosomes
1, 2, 6, 10, 13, 17, 21
Array-based karyotyping can be used to identify
characteristic chromosomal aberrations in renal tumors with challenging
morphology. Array-based karyotyping performs well on paraffin embedded
tumors and is amenable to routine clinical use. See also Virtual
Karyotype for CLIA certified laboratories offering array-based
karyotyping of solid tumors.
Other associated genes include TRC8, OGG1, HNF1A, HNF1B,
TFE3, RCCP3, and RCC17.
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Last Updated: Feb 1, 2011