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What Causes Merkel Cell Carcinoma

There Are Three Main Types Of Skin Cancers:

What Causes Merkel Cell Carcinoma?

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Symptoms: Know The Early Warning Signs Of Merkel Cell Carcinoma

The shape and color of MCC nodules are less distinctive than other skin cancers. In fact, many mistake them for other skin issues, such as bug bites, sores, cysts, sties, or pimples, according to the American Academy of Dermatology. Often, it is only the speed with which these lesions grow that attracts the attention of patients and their doctors.

With early detection and treatment, MCC can be well contained and often cured. Treatment becomes more difficult as the tumor grows and spreads. Thats why its critical to recognize this cancer in its earliest stage.

Common characteristics of MCC:

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Patients May Want To Think About Taking Part In A Clinical Trial

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today’s standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

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Merkel Cell Carcinoma: Causes Symptoms And Treatment

Merkel cell carcinoma is a rare, aggressive type of skin cancer.

Merkel cell carcinoma gets its name because these skin cancer cells resemble Merkel cells, which are located in the top layer of skin. Merkel cells are most concentrated in the fingertips, lips, and face, but MCC cells are most likely to develop on the head, neck, and other areas that have received the most sun exposure.

Below, learn about the causes of MCC, its symptoms, and how to prevent it. We also describe the diagnosis and treatment options.

Carcinoma is a type of cancer that develops in the skin or tissues covering or lining internal organs. Cancer occurs when cells begin to randomly divide and grow out of control. This cellular growth eventually forms a mass called a cancerous tumor.

MCC is one type of skin cancer, and its cells resemble Merkel cells. These are located close to the skins nerve endings and help provide a sense of light touch. They also relay information from the skin to the brain about details such as pressure and texture.

Merkel cells share traits with nerve and hormone-producing cells. As a result, they are classed as neuroendocrine cells, and MCC, in turn, is sometimes called neuroendocrine carcinoma of the skin. Another name for MCC is trabecular carcinoma.

MCC is very rare. In 2018, around 2,000 people in the United States received the diagnosis. However, its prevalence seems to be growing.

Treatment Of Stage I And Stage Ii Merkel Cell Carcinoma

Merkel Cell Carcinoma

For information about the treatments listed below, see the Treatment Option Overview section.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

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How Is Merkel Cell Carcinoma Treated

Your treatment plan depends on how early your doctor diagnoses the cancer, your overall health, and whether cancer cells have spread to other tissues in your body. Treatment for MCC usually involves one or a combination of therapies, including:

  • Immunotherapy, which uses specific medications to help your immune system fight cancer. Immune agents in use for MCC include avelumab , nivolumab, and pembrolizumab .
  • Surgery, which is a procedure that cuts out cancerous tissues. This type of therapy often involves a type of surgery called Mohâs micrographic surgery, a layer-by-layer skin removal method. Mohâs allows doctors to assess tissue under a microscope during the treatment and to save skin appearance and function. This is especially beneficial to people who have cancer on their faces.
  • Radiation therapy, which uses highly charged particles to kill cancer cells. This method is often combined with Mohâs surgery to improve results.
  • Chemotherapy, which uses drugs to kill cancer. This method of treatment was previously used more often, but was not found to give lasting results.

Causes And Risk Factors: Who Gets Merkel Cell Carcinoma

Its not fully understood what causes MCC, but researchers recently discovered that a common virus, called the Merkel cell polyomavirus , plays a role in many cases. 37219-5/fulltext” rel=”nofollow”> 5)

MCP lives on the skin of most people without any signs or symptoms and without ever developing into MCC. Just how this virus triggers a dangerous skin cancer in some people and not others has yet to be determined, but researchers have identified a number of factors that markedly increase your risk.

These include:

  • Excessive exposure to UV light Chronic exposure to light from the sun or tanning beds damages the DNA of genes that control skin-cell growth. This puts you at risk of all skin cancers and MCC is no exception. The vast majority of MCCs appear on skin surfaces that are frequently exposed to the sun.
  • Older age Though MCC can occur at any age, your risk significantly increases as you get older. More than half of MCC patients are over the age of 65 years at the time of diagnosis. The average age of diagnosis is 74.
  • A weakened immune system If your immune system is suppressed , you are about 15 times more likely to develop MCC than people with healthy immune function.
  • Light skin color More than 9 out of 10 cases of MCC in the United States are diagnosed in whites.

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What Does Mcc Look Like

MCC tumors often, but not always, appear on sun-exposed areas of the body. The tumors are not nearly as distinctive as other skin cancers and can appear as a pearly pimple-like lump, sometimes skin-colored, red, purple or bluish-red, though they are rarely tender to the touch. The rapid speed at which they grow is what often causes patients and doctors to take notice.

A recurrence of Merkel cell carcinoma on the forehead.

Merkel cell carcinoma on the lower leg.

Please note: Since not all Merkel cell carcinomas have the same appearance, these photos serve as a general reference for what MCC can look like. If you see something new, changing or unusual on your skin, schedule an appointment with your doctor. Photos courtesy of Paul Nghiem, MD, PhD

What Virus Causes Merkel Cell Carcinoma

Merkel Cell Carcinoma 101 (primary cutaneous neuroendocrine carcinoma)

Merkel Cell Polyomavirus: The Merkel cell polyomavirus is an important cause of the Merkel cell carcinoma. During infection, the Merkel cell polyomavirus grows inside the skin and synthesizes a protein that reduces the release of tumor suppression gene and leads to the development of Merkel cell carcinoma in the patients. This virus is responsible for almost 80% of Merkel cell carcinoma cases.

Week immune System: The patients with a weak immune system have more chance of Merkel cell carcinoma because the Merkel cell virus easily grows inside the body of such patients and cause Merkel cell carcinoma.

Exposure To Sunlight: Ultraviolet radiations and artificial light may also increase the risk of developing Merkel cell carcinoma. Ultraviolet rays damage the DNA of the cell which is responsible for controlling the cell growth and their functions in the body. This is believed to be the first step in the path of cancer.

Immunosuppression: The Merkel cell carcinoma is also caused by the defective immune function, HIV infection and immune suppression during organ transplantation.

Age: Aging also increases the risk of Merkel cell carcinoma. The condition most commonly occurs in people with age above 50 years as the immune system becomes weak and the power to counter the damaging effects of ultraviolet radiations also decreases. White males are more vulnerable to Merkel cell carcinoma3.

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Treatment Of Stage Iv Merkel Cell Carcinoma

For information about the treatments listed below, see the Treatment Option Overview section.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Immune Function & Merkel Cell Carcinoma

Patients with weakened immune systems are at significantly higher risk of developing MCC. Conditions associated with weakened immunity include HIV/AIDS, kidney or heart transplantation, and autoimmune diseases requiring medications that suppress the immune system, chronic lymphocytic leukemiachronic lymphocytic leukemiaA slow-growing type of leukemia associated with immune suppression. Patients with CLL have a markedly increased risk and severity of Merkel cell carcinoma. and certain types of lymphoma. The risk of developing MCC is 8 times greater in HIV patients, 10 times greater in organ transplant patients, and about 40 times greater in CLL.234 Long-term suppression of the immune system appears to be a risk factor for MCC in some patients. While patients with profound immune suppression are at a higher risk of developing MCC, over 90% of all people who develop MCC have no known immune deficiency.3

The immune system is also very important after diagnosis of MCC. Patients whose tumors show a robust immune response with certain immune cells present in their tumor tend to do better.5 MCC patients without a primary tumor also do better, likely because their immune system was able to eliminate the primary tumor and thus are more likely to be able to fight small amounts of MCC elsewhere in the body as well.6 In contrast, patients on medications that reduce immune function are at higher risk of having their MCC recur.

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Treatment Of Recurrent Merkel Cell Carcinoma

For information about the treatments listed below, see the Treatment Option Overview section.

Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.

Limitations Of Studies Describing Rt For Mcc

Merkel Cell Carcinoma: From Diagnosis to Treatment

The level of evidence supporting the benefits of RT is important to consider when evaluating its use for MCC. The robustness of the current evidence is hampered by inherent limitations of the studies conducted thus far. Case reports, small retrospective case series or meta-analyses of the same are prone to bias and often lack sufficient numbers to draw definitive conclusions. Moreover, in such reports, the criteria for designating which patients received RT are not often defined. In nonrandomized case series, patient selection can introduce bias that is impossible to control for. For example, during clinical management, physicians will sometimes avoid RT in patients with poor performance status because these patients may not tolerate the treatments. By contrast, some centers may reserve RT for patients with prognostic signs that portend worse outcomes, such as large primary tumors, lymphovascular invasion, positive or close surgical margins or infiltrative growth patterns. Regardless of what motivates the selection, such nonrandom assignments create patient groups that are not comparable in their expected outcomes.

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What Are The Risk Factors For Merkel Cell Carcinoma

The known risk factors for Merkel cell carcinoma include:

  • Exposure to UV rays. Like many other types of skin cancer, the risk of Merkel cell carcinoma is higher in people who have been exposed to a lot of UV rays from the sun or from other sources like tanning beds. People who are treated for psoriasis with UV rays may also have a higher risk.
  • Weakened immune system. People with weakened immune systems are at higher risk for this cancer. This can include people who have had an organ transplant or are receiving chronic immunosuppressive treatment for autoimmune diseases or cancer.
  • Light-colored skin. People with lighter skin are at higher risk.
  • Older age. People older than 50 are more likely to get this cancer.

Researchers have found that most Merkel cell carcinoma tumors are infected with a virus known as Merkel cell polyomavirus . Most people are infected with this virus at some point but very few people develop this cancer.

Merkel Cell Carcinoma Usually Appears As A Single Painless Lump On Sun

This and other changes in the skin may be caused by Merkel cell carcinoma or by other conditions. Check with your doctor if you see changes in your skin.

Merkel cell carcinoma usually appears on sun-exposed skin as a single lump that is:

  • Fast-growing.
  • Firm and dome-shaped or raised.
  • Red or violet in color.

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Treatment Of Merkel Cell Carcinoma

  • Surgical removal of the tumor

  • Usually radiation therapy and removal of lymph nodes

  • Sometimes chemotherapy

If cancer has spread or returns, chemotherapy Chemotherapy Chemotherapy involves the use of drugs to destroy cancer cells. Although an ideal drug would destroy cancer cells without harming normal cells, most drugs are not that selective. Instead, drugs… read more may be recommended.

Having A Weakened Immune System

Merkel cell carcinoma | Symptoms | Causes | Treatment | Diagnosis

The immune system defends the body against germs such as viruses. It also seems to help the body fight cancer. People with weakened immune systems are more likely to develop some types of cancer, including MCC.

For example, people who get organ transplants usually are given drugs that weaken the immune system to help keep them from rejecting the new organ. This increases their risk of developing MCC. People with autoimmune diseases sometimes take medicines that suppress the immune system, which might increase their risk for other diseases.

People with HIV, the virus that causes AIDS, often have weakened immune systems and are also at increased risk for MCC.

People with some types of blood cancers, such as chronic lymphocytic leukemia or certain lymphomas, also tend to have weakened immune systems. This can be from the cancer itself, or from its treatment. People with these cancers are more likely to get MCC.

MCCs in people with weakened immune systems tend to grow faster and are more likely to be life-threatening.

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Does Merkel Cell Carcinoma Hurt

In Merkel cell carcinoma disease, a reddish and purple lesion occurs on the skin. It is a painless disease. Risk of Merkel cell carcinoma increased when the skin is exposed to sunlight and artificial light. The weak immune system of the patients is also one of the most common risk factors of Merkel cell carcinoma. In some cases, the lesion on the skin is broken open and bleeds. The bump on the skin is progressing rapidly and spread as a new lump on another part of the skin. However, the patient does not experience any pain.

Signs And Symptoms Of Merkel Cell Carcinoma

Merkel cell carcinoma usually starts on areas of skin exposed to the sun, especially the face, neck, arms, and legs, but it can occur anywhere on the body. It often first appears as a single pink, red, or purple shiny bump that usually doesn’t hurt. Sometimes the skin on the top of the tumor breaks open and bleeds.

These tumors grow quickly. They might spread as new lumps in the nearby skin. They might also reach nearby lymph nodes . Over time, the lymph nodes might grow large enough to be seen or felt as lumps under the skin .

Merkel cell carcinoma is rare, and it can look like many other, more common types of skin cancer or other skin problems when it first appears. Because of this, doctors usually don’t suspect MCC at first, and the diagnosis is often made only after the tumor is biopsied.

Its very important to have any new, growing, or changing lumps, bumps, or spots on your skin checked by a doctor as soon as possible so that the cause can be found and treated, if needed. The earlier any type of skin cancer is found, the easier it might be to treat.

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