Types of kidney cancer

• What is renal cancer?
• Disease site
• Macroscopic features
• Microscopic features
• How is kidney cancer detected?
• Prognosis

What is renal cancer?

There are many types of renal cancers (also known as kidney cancers, renal cell carcinomas or RCCs). Each type of tumour is derived from a different cell type within the kidney and has its own distinct characteristics.
The three major types of RCCs are:

• Clear cell carcinoma–  75–85% of RCCs;
• Papillary cell carcinoma– 10–15% of RCCs; and
• Chromophobe carcinoma– 3–5% of RCCs.

Disease site

Clear cell RCCs generally develop as a solitary tumour; however, multiple tumours within one or both kidneys can rarely occur. This form of RCC can also occur in association with an inherited genetic disease known as von Hippel-Lindau syndrome (an inherited disease which causes both benign and malignant tumours).
Chromosomal changes are frequently seen in people with clear cell RCCs and these changes increase the likelihood of tumours occurring as they affect an important tumour-suppressing genetic sequence that normally helps the body to identify and kill cancer cells.
Papillary RCCs most frequently develop as a single tumour in one kidney. They can occur in association with other illnesses, known as hereditary renal cancer syndromes. Hereditary syndromes include:

• Hereditary papillary renal cell cancer syndrome;
• Hereditary leiomyomatosis; and
• Birt-Hogg-Dubé syndrome.

 
Chromophobe RCCs occasionally present as multiple tumours that can affect one or both kidneys. They can occur spontaneously or in association with Birt-Hogg-Dubé syndrome, a hereditary genetic disease.

For more information about the components of the renal system and how they function, see Urinary System (Renal System).

Macroscopic features

Clear cell RCCs are usually a solitary tumour affecting one kidney. They are often bright yellow on account of the abundant fat within the tumour itself. These tumours can present with a wide range of sizes; from millimetres to several kilograms. They are increasingly being detected incidentally on abdominal imaging (such as computed tomography (CT) or magnetic resonance imagine (MRI)) when individuals are being investigated for other diseases.  Most clear cell RCCs have well-defined borders and generally don’t invade the surrounding tissue. It is rare for a person to have multiple clear RCCs. Clear cell RCC in both kidneys is generally only seen in individuals with other hereditary diseases, such as von-Hippel-Lindau disease or tuberous sclerosis.
Papillary RCCs are usually solitary tumours in one kidney but can be multiple or occur in both kidneys. Typically, papillary RCCs have a thick outer capsule and the surrounding tissue may or may not be inflamed. There are two types of papillary RCCs (Type 1 or Type 2 papillary RCC); however, only microscopic differences distinguish these types.
Chromophobe cell RCCs also have well-defined borders but are generally tan brown in colour. On first detection they have a mean diameter of 8 cm (larger than other RCC types) and occasionally present as multiple tumours or in both kidneys.

Microscopic features

Clear cell RCCs are so called as the cancerous cells themselves are clear with a well-defined border. Papillary cell RCCs are so called due to their microscopic growth pattern which consists of small, rounded protuberances or “papillae”. Alternately, chromophobe cell RCCs  consist of microscopic tubular structures.

How is kidney cancer detected?

The increasingly early detection of RCCs is partly due to widespread use of abdominal imaging for investigating and diagnosing other diseases. Consequently, all RCC types are being detected more frequently as small, early-stage tumours that do not cause symptoms. However, in a quarter to a third of people with RCC, metastatic disease (spread to other sites in the body) is already in place at the time of diagnosis. In particular, clear cell RCCs often spread to other parts of the body via nearby major blood vessels. Metastatic clear cell RCC most commonly spreads to the lungs, abdominal lymph nodes, bone, brain, and liver.
The average age at diagnosis of clear cell RCC is 60 to 64 years and is more commonly found in men and in black populations. Only 7% of cases occur in people aged less than 40 and it is rarely seen in children.
Individuals with papillary RCC typically present in the 3^rd to 8^th decades of life and are more commonly male. When diagnosed, papillary RCCs tend to be smaller and at an earlier stage than clear cell RCCs. The lung is the commonest site to which papillary RCC spreads and these tumours appear to spread less than clear cell RCCs.
Chromophobe RCC is diagnosed mainly in the 6^th decade of life and men and women are equally likely to develop it. They are often diagnosed at an early stage and spread to the liver or the lung are the most common metastasis destination.

Prognosis

The overall prognosis for any RCC depends on the degree of advancement (cancer stage) at the time of diagnosis. 40–50% of people with RCC will eventually develop metastatic disease, which is associated with a poorer prognosis. Of all types, clear cell RCC has the worst prognosis with 5-year survival rates between 50 and 69%.
Papillary RCCs metastasise (spread to other sites in the body) less frequently than clear cell RCCs; however, they have similar sites of distant metastases (lung, bone and brain). When this type of RCC spreads into nearby blood or lymphatic vessels survival is reduced in comparison to clear cell RCC that has spread into nearby vessels.
Chromophobe cell RCCs generally have a more prolonged, less aggressive course and a more favourable disease outcome compared with clear cell RCC and have a slightly better or similar survival rate compared to papillary RCC.
 

For more information about the treatment options that are available for RCC and how effective they are, see Treatments for Renal Cell Carcinoma (RCC).

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