Subtypes of Kidney Cancer


Renal Cell Carcinoma (RCC) is more than 90% of all kidney cancers. However, not all Renal Cell Carcinomas are the same. There is an increasing understanding among clinicians and researchers that Renal Cell Carcinoma (RCC) has different subtypes and that each has different behavior. These subtypes of kidney cancer are different in how aggressive they are in patient and how they respond to treatment.

About 10 or 15 years ago, it was extremely common for a kidney cancer patient to read from the pathology report simply “Renal Cell Carcinoma”. However, this simple diagnosis is now thought to be not sufficient. Determining the specific cell type or subtype of kidney cancer has become extremely important for assessing the chance of recovery just like knowing the stage or grade. This chance of recovery can also be defined as prognosis.

For more information about the stages of Kidney Cancer, you can read:
Kidney Cancer Basics: Stages


When you are diagnosed with kidney cancer, your doctor will tell you about the histology, grade and stage of your kidney cancer according to the pathology report.

Also, in this article, information about subtypes of Renal Cell Carcinoma (RCC) will be presented.

Renal Cell Carcinoma (RCC) is more than 90% of all kidney cancers. However, not all Renal Cell Carcinomas are the same. There is an increasing understanding among clinicians and researchers that Renal Cell Carcinoma (RCC) has different subtypes and that each has different behavior. These subtypes of kidney cancer are different in how aggressive they are in patient and how they respond to treatment.

1. Clear Cell Renal Cell Carcinoma

This is the most common form of kidney cancer and represents approximately 66% to 75% of all cases. Clear cell carcinoma is associated with mutation in the von Hippel-Lindau gene in hereditary renal cancers. In fact, approximately 70% of all non-hereditary clear cell carcinoma also have the mutation on von Hippel-Lindau gene.

Today, many of researches which aims to find new effective treatments for patients with locally advanced or metastatic disease, are based on this form because it is one of the most common subtypes of kidney cancer. If the tumor did not metastasize at the time of diagnosis, the prognosis can be very good after tumor removal with surgical treatment.

For more information about the metastasis of Kidney Cancer, you can read:
Kidney Cancer Basics: Metastasis


The prognosis of a patient which has clear cell carcinoma depends on both the size and rate of growth which can be called stage, as well as the grade of the tumor, which expresses the characteristics of the cell structure

2. Papillary Renal Cell Carcinoma

Papillary cell carcinoma is the second most common tumor and accounts for approximately 15% of all renal tumor cases.

This form is also divided into two itself; while the incidence of Type I is 5%, the incidence of Type II is 10%.

It is known that papillary renal cell carcinoma is more common in African Americans and also the incidence of bilateral disease which means involving both kidneys, is increased.

There are also hereditary forms of both Type 1 and Type 2.

If papillary carcinoma does not spread at the time of diagnosis, the prognosis is excellent.

Targeted therapies are modestly effective in treating papillary carcinoma. Especially Sutent® (sunitinib) and Afinitor® (everolimus) are the first options in treatment.

In this form, it is highly recommended to be a part of the clinical trial.

3. Chromophobe Renal Cell Carcinoma

This is a rare subtype of Renal Cell Carcinoma and accounts for approximately 5% of all renal cell carcinoma cases. This form is thought to originate from the same cell type as those that form renal oncocytomas. Tumor cases that have the characteristics of both chromophobe renal cell carcinoma and renal oncocytoma have also been described.

It is also known to be a chromophobe renal cell carcinoma of the hereditary form known as Birt Hogg Dube syndrome and known to be associated with renal oncocytoma.

A hereditary form of chromophobe renal cell carcinoma which associated with renal oncocytoma and known as Birt-Hogg-Dubé syndrome is also known. This hereditary form is also associated with specific genetic mutations.

Chromophobe renal cell carcinoma metastasizes very rarely to the late stages of the tumor. It is usually can be treated with surgical removal even in locally advanced diseases and the prognosis is very good.

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4. Renal Oncocytoma

It is a benign tumor of the kidney and represents about 5% of all renal tumors. Although this kidney tumor has the potential to grow to very large sizes and invade local structures, it never metastasizes.

These tumors are thought to be associated with chromophobe renal cell carcinoma and it is very difficult to make a differential diagnosis of these two tumors.

Treatment of this tumor is partial or complete removal of the kidney.

5. Unclassified Renal Cell Carcinoma

Less than 1% of renal cell carcinomas have not yet been classified. When examined under a microscope, these tumors do not conform to any of the other common forms, in the manner of genetics and structure of cells.

This category usually includes aggressive tumors with poor prognosis that do not respond to traditional kidney cancer treatment.

6. Translocation Carcinomas

This form, called translocation carcinoma, which is a very different subtype of renal cell carcinoma, is associated with changes in genetic structures.

According to the studies, the cause of this cancer is the fusion of the TFE3 gene and many other genes on chromosome Xp11.2.

Compared to other types of kidney cancer, translocation carcinoma occurs at a younger age.

According to one series, the average age of patients is 24 and more common in women than in men.

This subtypes of kidney cancer are also seen more frequently in children receiving antecedent chemotherapy due to some conditions such as autoimmune diseases, malignancies, and bone marrow transplantation.

7. Collecting Duct Carcinoma

It is a very rare and highly aggressive subtype of renal cancer that represents less than 1% of cases. This form is usually spread to other organs at the time of diagnosis.

As with transitional cell carcinoma, therapies are usually directed at using chemotherapy-based regimens.

This cancer which usually seen at a younger age does not respond to traditional renal cell carcinoma treatments such as immunotherapy.

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8. Medullary Renal Cell Carcinoma

This subtype is also an extremely rare and aggressive form of renal cell carcinoma. This cancer, which is considered a variant of collecting duct carcinoma, is commonly associated with sickle cell trait.

It is more common in African Americans because of its association with sickle cell trait.

It accounts for less than 1% of all kidney tumors diagnosed. Chemotherapy is the main treatment option.

9. Sarcomatoid Renal Cell Carcinoma

This form of kidney cancer is a highly undifferentiated tumor. It can occur with any other common form of renal cell carcinoma.

When examined under a microscope, the cells of this type of cancer carry the appearance of sarcoma cells. The degree of sarcomatoid differentiation is associated with the aggressiveness of the tumor.

Previously, the prognosis associated with sarcomatoid renal cell carcinoma was thought to be extremely low, but there is now more hope with the availability of new drugs.

This form is found particularly common in cases of widely metastasized kidney cancer.

These subtypes of kidney cancer is sometimes treated with chemotherapy.

10. Transitional Cell Carcinoma of the Kidney

Transitional cell carcinoma of the kidney is an extremely rare and potentially very aggressive tumor. However, this tumor should not be considered a true kidney cancer and should be grouped with bladder cancer.

If the cancer is not metastasized at the time of diagnosis, treatment can be achieved by surgical removal of the kidney and its ureter.

However, recurrences are very common especially in transitional cancer of bladder, this situation can be also seen in the kidney.

If the tumor is too large or metastasized, the prognosis is extremely poor. In this case, treatment options are the same as for metastatic urinary bladder cancer and chemotherapy is the only treatment option.

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