What you need to know about Mucosal Melanoma!

We've all heard of skin cancer, right? But did you know that sometimes these cancers can develop inside our bodies, in places that are not visible from the outside? One such rare but dangerous type of cancer is called Mucosal Melanoma. If this sounds a bit serious, don't worry. Let's talk about this in a simple way that you can understand.

What is this so-called Mucosal Melanoma?

Simply put, Mucosal Melanoma is a rare type of cancer called Melanoma . It starts in the mucous membranes of our body. Now you may be wondering what a mucous membrane is. It is a thin, moist membrane that covers the organs inside our body, like the inside of the nose, inside the mouth, the anus, and the vagina. Think of it like the thin, moist skin inside our mouth. That's where these mucous membranes are found.

So, you can get this condition called mucosal melanoma anywhere there are mucous membranes. But it's most common in:

• In the head and neck areas: especially the nose, lips, and inside the mouth.
• The anorectal region: This means the area around your anus and the passage through which your stool comes out.
• Vulvovaginal region: The external parts of the female vagina and surrounding areas .

This condition usually affects people over the age of 70. Unfortunately, the cancer can often have spread (metastasized) to other parts of the body by the time it is diagnosed. Doctors can treat this type of mucosal melanoma with surgery. However, in most cases, it is treated by a multidisciplinary team of doctors who work together to use a variety of treatments.

Is this a common condition?

No, not really. It's very rare . Think about it, the American Cancer Society estimates that there will be about 99,000 new cases of melanoma in 2022. But this type of melanoma, called mucosal melanoma, only accounts for about 1% of all melanoma cases. That's about 990 people. So you can see how rare this is.

What are the symptoms of Mucosal Melanoma?

This is where it gets a little confusing. Because the symptoms of mucosal melanoma can be easily missed . That's because this cancer develops in hidden places inside our bodies that we can't always check. Think inside our nose or the skin around our anus. We don't always notice changes in those places.

What's more, these symptoms can sometimes be similar to those of other, less serious diseases. So many people don't consider these changes to be signs of a bigger problem. Symptoms also vary depending on where the cancer starts.

Head and neck related

Between 31% and 51% of all mucosal melanoma cases occur in the head and neck. There are three main types, with their associated symptoms:

Oral mucosal melanoma

This cancer affects the inside of your mouth, on your lips. Symptoms may include:

• A lump on the tongue that is not healing and is getting bigger and bigger.
• A sore in the mouth that won't heal.
• Mouth pain.
• Bleeding from the mouth.
• The dentures are not fitting properly. (If you are a denture wearer)

Sinonasal mucosal melanoma

This is a cancer that develops in the nasal passages and sinuses. Symptoms may include:

• Continuous bleeding from one nostril (nosebleed - `epistaxis`) .
• A feeling of something being stuck inside the nose (nasal obstruction) .
• Frequent urination.
• Facial pain.

Laryngeal/pharyngeal mucosal melanoma

This is the least common type of mucosal melanoma. It affects your throat. Symptoms may include:

• Hoarseness of voice.
• Difficulty swallowing food (dysphagia) .
• Sore throat or infection (pharyngitis) .
• A lump or tumor appears on the outside of the neck.

In and around the anus

Anorectal mucosal melanoma occurs in your rectum and/or anus. It affects between 17% and 24% of all patients. Symptoms may include:

• Pain in the rectum or anus.
• A lump or tumor in the rectum or anus.
• Bleeding from the anus or rectum.
• Constipation.
• Itching around the anus (pruritus ani) .

In the vagina and external vaginal area

Vulvovaginal mucosal melanoma occurs in the walls of the vagina and vulva in women. It affects about 18% of all patients. Symptoms may include:

• Vaginal bleeding during menstruation or after menopause (that is, after menstruation has completely stopped).
• Vaginal pain.
• There may be an abnormal vaginal discharge, even if it is bloody .
• A lump or growth in the vagina or external vaginal area.
• Itching in the vagina or external vaginal area that does not improve despite treatment.

Very important: These symptoms can often be caused by other common medical conditions. However, if you experience any of these symptoms, especially if they persist, please consult a doctor . Early detection is always a great help in successful treatment.

Why does this mucosal melanoma develop? What are the causes?

The short answer is that medical researchers still don't know the exact cause of mucosal melanoma. Like all melanomas, this type of cancer starts in cells called melanocytes . Melanocytes are the cells that produce the pigment melanin, which determines the color of our eyes, hair, and skin. This melanin protects our skin and eyes from damage from the sun.

But surprisingly, researchers are still studying why these melanocyte cells, which protect against sunlight, are located in mucous membranes that rarely get sunlight.

However, researchers have found that two genetic mutations may contribute to the development of some of these mucosal melanomas. These are called somatic mutations , meaning they are not something we are born with, but rather changes that occur in our cells during our lives. Mutations in the KIT gene and the BRAF gene are found in a small percentage of mucosal melanoma patients:

• KIT gene: The KIT gene controls how fast some cells, including melanocytes, grow. Like the BRAF gene, the KIT gene makes a protein that helps cells grow. When the KIT gene is mutated, the KIT protein misdirects other proteins, causing cells to divide and multiply faster than normal. Studies show that KIT mutations are found in 7% to 17% of all cases of mucosal melanoma. They are also found in about 30% of cases of mucosal melanoma of the vagina and vulva.

• BRAF gene: The BRAF gene controls proteins that control the growth of melanocytes. These proteins get instructions from the BRAF gene about how fast cells should divide and multiply. When the BRAF gene is mutated, those instructions are changed, causing cells to multiply uncontrollably and eventually become cancerous. However, BRAF mutations are only seen in a small percentage of cases, between 3% and 15% of all cases of mucosal melanoma.

Sun exposure is a cause of skin melanoma. Is the same true for mucosal melanoma?

No, it doesn't. Mucosal melanoma develops in areas that are not exposed to the sun, such as inside the nose, inside the mouth, or in the anus. So sunlight is not a direct cause.

How do doctors diagnose this disease?

Doctors use various tests to diagnose this disease, including:

• Various imaging tests may be included, such as CT scans (Computed Tomography scans) and MRI scans (Magnetic Resonance Imaging scans) . These can look at the tumors inside the body, their size, and whether they have spread.
• Biopsies are performed. This means that a small sample of cells or tissue is taken from a suspicious area and examined under a microscope by pathologists. This is what determines exactly whether it is cancer and what type it is.
• Genetic tests can also be done to check for genetic mutations.

What is the cancer staging of Mucosal Melanoma?

Doctors use cancer staging systems to plan treatment and determine the prognosis, or expected outcome. In general, early-stage mucosal melanoma is a condition in which the cancer has not spread beyond where it started.

However, this is a bit complicated . Because each type of mucosal melanoma (e.g., mouth, nose, rectum) has its own staging system, and the details are very different. Cancer staging can be a bit difficult to understand. If you are unsure about what your doctor is saying, or if you have any concerns or questions, be sure to ask your doctor for more information .

How is this treated?

Because mucosal melanoma is a complex disease, treatment is often managed by a multidisciplinary team of doctors from different specialties . This team may include surgical oncology specialists, radiation oncology specialists, and medical oncology specialists. Treatment options include:

• Surgical removal of cancerous tumors and surrounding tissue. The type of surgery depends largely on the location of the tumor. Some tumors are very challenging to remove surgically.
• Radiation therapy is given as adjuvant therapy after surgery to destroy any remaining cancer cells.

Medical researchers are currently conducting clinical trials of immunotherapy for cancer and targeted therapy for cancer . Clinical trials are studies that evaluate whether new treatments are safe and effective. If you have mucosal melanoma, it's worth asking your doctor if you're eligible for one of these clinical trials.

What can you expect when living with this condition?

Mucosal melanoma is a potentially life-threatening disease . Doctors use surgery and other treatments to remove the cancerous growths and kill the cancer cells. However, mucosal melanomas are more likely to come back (recur) . If you have mucosal melanoma, you may need additional surgery or other treatments.

What is the prognosis of the disease?

When it comes to the chances of recovery or survival, it varies from person to person. However, overall, studies show that the five-year survival rate for mucosal melanoma is about 25% . This may seem low, but it is an average.

These survival rates also vary depending on the type of mucosal melanoma:

• Head and neck: In general, studies show that less than 30% of people are alive five years after diagnosis. (Survival rates range from 14% to 48%).
• Vaginal and vulva: About 36% of people with this condition are alive five years after diagnosis.
• Rectal and anal cancer: About 20% of people with this condition are alive five years after diagnosis.

Don't be alarmed by these statistics. These are just estimates based on the experiences of a group of people with a particular condition. What's true for them may not be true for you. If you have questions about these survival rates, ask your doctor to explain what you can expect based on your condition.

How should I take care of my health?

Mucosal melanoma is a life-threatening disease that can recur even after treatment. If you have recurrent disease, you may want to consider palliative care to help manage symptoms and side effects of treatment. Palliative care is care that aims to improve a patient's quality of life and reduce pain, rather than to cure the disease.

When should I see a doctor?

Although there are several types of mucosal melanoma, all types can usually come back after treatment. Depending on your condition, your doctor will schedule regular checkups to monitor your overall health and check for recurrence. It is very important to attend these appointments without missing a beat.

What questions should I ask my doctor?

Here are some questions you can ask your doctor:

• What type of mucosal melanoma do I have?
• What is the stage of my disease?
• What treatment options do I have?
• How will the treatment affect me? (Things like side effects)
• What is the prognosis of my disease?

Take-Home Message

Okay, so from what we've discussed, I hope you've got some idea about Mucosal Melanoma. Remember:

• This is a rare type of cancer that develops in the mucous membranes inside our bodies.
• Symptoms can be easily missed and can resemble other common illnesses. Therefore, seek medical advice if you have unusual, persistent symptoms in the mouth, nose, rectum, or vagina.
• Often, it may have spread even before it is detected.
• Treatment requires the assistance of a specialist medical team .
• Long-term medical monitoring is important, as the disease can recur .

We hope this information is useful to you. Being aware of this type of disease will help you take action quickly if necessary. Always take care of your health!

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