Types of Kidney Cancer and Related Syndromes

Clear cell renal cell cancer

The most common type of kidney cancer is called clear cell renal cell carcinoma (RCC). This accounts for up to 80% of kidney cancer. It is more common in men than women and usually occurs after age 55. Clear cell RCC is thought to arise from the cells that line the kidney tubules where urine is made. Through a microscope, the cells look clear and pale. This type of kidney cancer is usually due to a mutation in the von Hippel Lindau (VHL) gene. There are families with inherited mutations of VHL who develop kidney cancers in addition to other medical problems (see VHL Syndrome below). The majority of people, however, who develop clear cell RCC have a non-inherited or “sporadic” version. As a result of the medical research that has found this gene abnormality, there have been great advances in the treatment of this type of kidney cancer.


Papillary renal cell carcinoma

The second most common type is called papillary renal cell carcinoma (RCC). Papillary rcc accounts for about 15% of kidney cancers. The name is from microscopic finger-like projections (called papillae) in some, if not most, of the tumours. 

  • Type 1
    is generally less aggressive. There is a small subset of people who can inherit this type of papillary renal cell carcinoma. Papillary type I RCC, in both the sporadic and hereditary forms, is associated with a mutation of the MET oncogene. An oncogene is a gene in cells that promotes growth and this cancer is associated with abnormalities of the MET gene.
  • Type 2
    tends to be more aggressive, and in rare cases is part of a hereditary syndrome as well. Papillary type 2 tumors occur both sporadically and in patients who have the familial syndrome of hereditary leiomyomatosis and renal cell carcinoma (HLRCC). The genetic mutation associated with HLRCC is in the gene known as fumarate hydratase (FH).



Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC) or Reed’s Syndrome: www.hlrccinfo.org - HLRCC Family Alliance 

Hereditary Papillary Renal Cell Carcinoma: http://www.cancer.net/cancer-types/hereditary-papillary-renal-cell-carcinoma

Chromophobe renal cell carcinoma

This accounts for about 5% of RCC. Overall this is usually less aggressive than clear cell RCC and is usually confined to the kidney. However there are times it can be more serious and can spread.



Information on Chromophobe rcc is scarce. Recommended resources include PubMed for recent articles. Suggestions for helpful resources would be most welcome (info@kidneycancercanada.ca)

Collecting Duct renal cell carcinoma (Bellini duct carcinoma)

This subtype is very rare. The major feature is that the cancer cells can form irregular tubes and starts in a different part of the kidney tubule. This tumour type is very aggressive, and has traditionally been treated with chemotherapy although the best treatment is not yet known.



Information on Collecting Duct rcc is scarce. Recommended resources include PubMed for recent articles. Suggestions for helpful resources would be most welcome (info@kidneycancercanada.ca)


This accounts for about 5% of renal tumours. An oncocytoma is usually a solitary tumour that is benign. It is often diagnosed on biopsy or pathologically after treatment of presumed RCC . However, a form of oncocytic cancer, chromophobe RCC, can occur.




Genetic translocation cancer

This is a distinct form of kidney cancer that can occur in young adults (especially females) and probably children. It may make up approximately one-third of pediatric RCC. This can be diagnosed by special staining (immunohistochemical staining) of tissue from the kidney tumour. There are genetic abnormalities called translocations in the TFE3 gene (Xp11.2 translocation).

Approximately 10% to 15% of translocation RCCs occur in children who had prior chemotherapy for a prior cancer. Therefore translocation RCCs should be added to the list of chemotherapy-associated secondary cancers in children.

Renal medullary cancer

This rare yet aggressive cancer can be found in young adults that have sickle cell trait or rarely sickle cell disease, an inherited disease related to their haemoglobin in their red blood cells. In those with sickle cell trait the person carries one normal copy of the hemoglobin A gene (HbA) and one copy of the hemoglobin A gene harboring the genetic mutation found in sickle cell disease (HbS). These individuals do not have sickle cell disease but can develop kidney disease. These people may show visible (gross) hematuria (blood in the urine), abdominal or back/flank pain, abdominal mass, enlarged lymph nodes and significant weight loss.

Transitional cell carcinoma of the kidney, renal pelvis

Transitional cell carcinoma (TCC, more recently referred to as urothelial cell carcinoma or UCC) is a type of cancer that typically occurs in the lining of the urinary system - the kidney, bladder, and ureters). It is the most common type of bladder cancer and cancer of the ureter, urethra. It is the second most common type of kidney cancer (following renal cell carcinoma), but accounts for only five to 10 percent of all primary renal malignant tumors. Treatment is similar to that of bladder cancer and includes surgery and sometimes chemotherapy.



Bladder Cancer Canada: http://bladdercancercanada.org/en/