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Rare Kidney Cancer Subtypes

Kidney cancer is a term that encompasses many different types of cancer that may arise in the kidney. The kind of kidney cancer that you may have depends on which cells of the kidney became abnormal or cancerous. The best treatment is based upon the type of kidney cancer you have and its size. In very rare cases, kidney cancer may be related to a hereditary disorder such as VHL Syndrome.

Clear cell renal cell carcinoma (ccRCC)

The most common type of kidney cancer is clear cell renal cell carcinoma (ccRCC). This form of kidney cancer represents about 75% of the kidney cancer diagnosed. Rare subtype renal cell carcinoma is a term for a group of kidney cancers that are not clear cell renal cell carcinomas. Rare subtype renal cell carcinoma is a group of diseases that encompasses many different subtypes of tumors, each with distinct genetic and biochemical characteristics. Typically, cancer is named after where the cancer grows in the kidney or what kind of cells the tumors look like. 

Rare subtype kidney cancers are uniquely different from each other. Each subtype of kidney cancer grows and reacts to treatment in various ways. Since many rare subtypes of kidney cancer affect children or young adults, reproductive difficulties may arise later in life due to treatment. It is recommended this should be discussed with your physician.

How do you know if you have a Rare Subtype kidney cancer?

To accurately determine the type of kidney cancer you may have, a diagnostic look at the tumor must occur. This can be done by biopsy or by a pathology report of a tumor upon removal. Most types of kidney cancers are identified this way. Other much rarer sub-types will need additional follow-up tests to accurately diagnose.

What are some factors that may lead to a Rare Subtype kidney cancer diagnosis?

There are still many unknown factors to the growth of kidney cancer, and specifically rare subtypes. However, if you are diagnosed with kidney cancer and you fall into the following groups, you should speak to your doctor about the possibility of a rare subtype kidney cancer.

AGE

  • Children do get kidney cancer. A clear diagnosis of the type of childhood kidney cancer is important to determining treatment options.
  • Young adults may be more susceptible to a rare subtype of kidney cancer. The average age of kidney cancer diagnosis is 63 years of age.

SICKLE CELL TRAIT

  • Young black men diagnosed with kidney cancer are often diagnosed with a rare subtype known as renal medullary carcinoma.

HEREDITARY

  • If you have had a family member diagnosed with kidney cancer previously genetic testing on your kidney cancer should occur.

Types of Rare Subtype Kidney Cancers

Birt-Hogg Dube (BHD) Syndrome

A rare genetic disorder with the most complicated symptoms manifesting as kidney cancer. BHD is caused by a mutation in the FLCN gene. Due to higher risk of being diagnosed with kidney cancer, it is recommended the patients with BHD maintain a regular screening schedule.

For further information contact the Birt-Hogg Dube Foundation

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chRCC or Chromophobe Renal Cell Carcinoma

A rare subtype of kidney cancer that forms in the cells of the kidney’s tubule. Symptoms of chromophobe renal cell carcinoma often include no noticeable symptoms (asymptomatic) or vague signs like weight loss, fatigue, and fever; however, when symptoms do appear, they may include blood in the urine (hematuria), flank pain (pain in the side), and a palpable mass, usually indicating an advanced stage of the cancer.

For further information contact the Chromophobe and Oncocytic Tumor Alliance

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HLRCC or Hereditary Leiomyomatosis and Renal Cell Cancer

This condition is a genetic form of kidney cancer and can affect multiple organs, including the skin, uterus, and kidneys. HLRCC is caused by mutations in the FH gene, which provides instructions for making an enzyme called fumarate hydratase. HLRCC is a complicated disease, and it is recommended to seek treatment from physicians familiar with HLRCC. Genetic Testing is also appropriate.

For further information contact HLRCC Foundation

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pRCC or Papillary Renal Cell Carcinoma

A type of kidney cancer that forms in the lining of the tiny tubes that return filtered substances that the body needs back to the blood and remove extra fluid and waste as urine. It’s the second most common type of kidney cancer, making up about 15% of all renal cell carcinoma cases. 

Doctors used to split papillary kidney cancer into “type 1” (usually slower growing) and “type 2” (often more aggressive). Recent research showed those two labels do not capture the full picture, so pathologists have stopped using them and now group these tumors together simply as papillary kidney cancer, also called papillary renal cell carcinoma, or pRCC. Patients with a genetic disorder called hereditary papillary renal cancer have an increased risk for the tumors once called “type 1”. Another genetic disorder called hereditary leiomyomatosis and renal cell cancer is typically linked to the more aggressive variety.

A video by KidneyCAN

RMC or Renal Medullary Cancer

A rare and aggressive type of kidney cancer that develops in the innermost part of the kidney. The most common first symptom of RMC is blood in the urine. Other symptoms may include pain in the kidney area, fever, night sweats, fatigue, and weight loss. RMC is more common in males and people with sickle cell trait. Due to the complicated treatment course, it is recommended to seek a physician specializing in RMC.

For further information contact the Chris “CJ” Johnson Foundation

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A video by KidneyCAN

tRCC or Translocation renal cell carcinoma

A rare and aggressive type of kidney cancer that occurs when a piece of one chromosome breaks off and attaches to another. This genetic mutation will then cause the cells not to work correctly. About 30% of childhood kidney cancer is tRCC. It is highly recommended that genetic testing is completed to ensure correct diagnosis. It is also advised to have treatment with a physician and institution familiar with tRCC.

For further information contact Joey’s Wings

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A video by KidneyCAN

VHL Disease

A genetic disease that can cause tumors (benign or malignant) to develop in up to 10 different organs throughout a patient’s life including the kidney. VHL is a complicated disease, and it is recommended to seek treatment from physicians familiar with VHL. It is important to note that VHL disease may not originate in the kidney but may begin in other areas of the body. The most common areas where tumors may develop include the brain, eyes, adrenal gland, and reproductive organs. The most common type of tumors are hemangioblastomas. Genetic Testing is also appropriate.

For further information contact the VHL Alliance

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Wilms Tumor or Nephroblastoma

The most common form of childhood kidney cancer. Wilms tumors develop when small cells have a mutation in the DNA which causes the tumor to grow. Children with this rare subtype may have swelling in the flank, pain in the stomach, or blood in the urine. Wilms Tumor kidney cancer is highly treatable.

For further information contact the Wilms Foundation

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tRCC Project

The Translocation Renal Cell Carcinoma Project (tRCC Project) is a collaborative research initiative out of the Broad Institute of MIT and Harvard that enables any person in the U.S. or Canada diagnosed with tRCC to share information about their experience by completing surveys, sharing biological sample(s), and copies of their medical records with researchers in order to accelerate the pace of research and discovery.

Learn more at tRCCProject.org.

VISIT WEBSITE

People with a recent diagnosis of kidney cancer are strongly encouraged to make an appointment with a major research cancer center. Kidney cancer is not one of the most common cancers, so advice from specialists is ideal when possible. Learn more about building your care team and find the right doctors for you.

Choosing the Right Kidney Cancer Specialist

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