Are you aware of Renal Medullary Carcinoma (RMC), a rare but serious cancer that develops in the kidneys?

Have you ever heard of a very rare and serious type of cancer that develops in the kidneys, especially in young people? Probably not. This is Renal Medullary Carcinoma, or RMC for short. Although it is not very common, it can be very important to be aware of it, especially for some people. Although this is a bit of a serious topic, don't be afraid. Let's talk about it in a simple, understandable way today.

What exactly is RMC?

Simply put, Renal Medullary Carcinoma (RMC) is a type of kidney cancer. But it is very rare, very aggressive, and spreads quickly. It is most common in young people, especially those with a blood condition called `sickle cell disease` or `sickle cell trait`.

When we talk about kidney cancer, the most common type is `renal cell carcinoma`. RMC is also a subtype of `renal cell carcinoma`. But not even one in 100 kidney cancer patients develops this RMC condition. It is that rare.

What causes this cancer?

Scientists and doctors still haven't found the 100% exact cause of this, but there is one main thing that has been identified.

Our kidneys have a part called the `renal medulla`. A person with `sickle cell` condition sometimes has red blood cells that change into a sickle shape. When this happens, the cells can stick together and block the blood vessels in the `renal medulla` part of the kidney.

This blockage of blood supply damages the cells in that area. In particular, a gene called `INI1` (also called `SMARCB1`) in those cells is damaged. This `INI1` gene acts as a `guardian` that stops cells from becoming cancerous. When RMC cancer cells are examined, this `INI1` gene is not found in them. So doctors check for the presence of this `INI1` gene to confirm whether a tumor is RMC or not.

Am I also at risk of developing RMC?

It's hard to say exactly who will develop this. Having someone in your family with RMC doesn't increase your risk of developing it. That means it's not something that runs in families.

The main risk factor is a blood disorder called `sickle hemoglobinopathy`. That is, having `sickle cell disease` or `sickle cell trait`. But very rarely , people without these conditions can also develop RMC. It is called “renal cell carcinoma, unclassified with medullary phenotype” (RCCU-MP).

If you look at the statistics:

• More common in men than women.
• It most often affects the right kidney.
• The disease is usually diagnosed at a young age, between 11 and 39 years old.

What are the symptoms of RMC?

There are several common symptoms of this disease. If you have one or more of these, it is best to seek medical advice as soon as possible.

How does a doctor diagnose this?

When you tell us about your symptoms, the first thing the doctor will do is examine you. Then you can follow these steps.

1. Ultrasound scan (`Ultrasound`): This can give a rough idea of ​​whether there is something like a tumor in the kidneys.

2. CT or MRI scan: If a tumor is suspected, a CT scan or MRI may be ordered to get a clear picture of the tumor. Based on these images, the doctor can determine how likely it is to be cancerous.

3. Biopsy: If cancer is strongly suspected, this test is done to confirm it. This involves inserting a fine needle into the area of ​​the kidney tumor, taking a very small piece of tissue and examining it under a microscope.

4. Genetic testing: The cells taken from the biopsy are checked for the presence of the cancer-fighting `INI1` gene we talked about earlier.

• If the `INI1` gene is missing , it is identified as RMC.
• If the `INI1` gene is missing and the patient does not have `sickle cell` condition, it is diagnosed as `RCCU-MP`.
• If the `INI1` gene is present , it is not RMC, but a different type of tumor.

What are the treatments for this?

Because RMC is a rapidly spreading, aggressive cancer, treatment must be equally swift and aggressive.

• Chemotherapy: This is the most common first treatment. It involves giving the body drugs that kill cancer cells.
• Surgery: Removal of the cancerous tumor and possibly the affected kidney.
• Radiation Therapy: Destroying cancer cells using powerful radiation beams.

Because this disease is rare, research is still ongoing. Scientists are always looking for new and more effective treatments for RMC.

Is there a way to prevent RMC?

Unfortunately, there is currently no known way to prevent RMC from developing, nor is there a test to detect it before symptoms appear.

The best thing you can do is be aware . If you know you have sickle cell disease or sickle cell trait, be on the lookout for the symptoms mentioned above. If you have any concerns, see your doctor right away.

Often, by the time this disease is diagnosed, the cancer has already spread to the lymph nodes or other organs such as the lungs, liver, and bones. Therefore, early detection is the best way to achieve the best results.

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