What Are The Clinical Features Of Merkel Cell Carcinoma
Merkel cell carcinoma usually presents as a rapidly enlarging, solitary, irregular red nodule. It is often similar in appearance to other more common skin cancers such as basal cell carcinoma but grows much more quickly.
Merkel cell cancers spread through the lymphatic system and multiple metastases can develop around the main tumour . Merkel cell carcinoma may also spread to lymph nodes in the neck, axillae and groin. This is more likely in thicker tumours. Most recurrences occur within the first two years after diagnosis.
Merkel cell carcinoma
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Where Squamous Cell Carcinoma Occurs
SCC can be found anywhere on the body, but is most commonly seen in sun-exposed areas. Common SCC sites include the face, ears, lips, scalp, shoulders, neck, hands, and forearms. Its also possible to be diagnosed with SCC in areas without sun exposure, such as inside the mouth, under fingernails or toenails, on the genitals, or in the anus.
How Is Merkel Cell Cancer Diagnosed
The most common way to find Merkel cell cancer is when a lump is found and you see a healthcare provider about it. The healthcare provider will look at and feel the lump. They will ask about your health history, and do a physical exam of your skin.
You may be sent to a dermatologist. This is a doctor with special training to treat skin problems. The doctor may use a special light, magnifying lens, or camera to get a very close look at the lump. You will likely need a biopsy.
A biopsy is the only way to know if a lump or change is cancer. The doctor takes small pieces of tissue from the lump. These samples can be removed with a needle or scalpel, or during surgery. They are checked with a microscope to look for cancer cells.
It can be hard to diagnose MCC. It can look like many other types of cancer. Special lab tests can be used on the biopsy sample to find out whats caused the skin change.
After a diagnosis of MCC, youll likely need more tests. These help your healthcare providers learn more about the cancer. They can help determine the stage of cancer. The stage is how much and how far the cancer has spread in your body. Its one of the most important things to know when deciding how to treat MCC.
Once your cancer is staged, your healthcare provider will talk with you about what the stage means for your treatment. Ask your healthcare provider to explain the stage of your cancer to you in a way you can understand.
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Is Merkel Cell Carcinoma Fatal
Like with any other cancer the prognosis of Merkel cell carcinoma depends upon the stage at which the condition is diagnosed and the response of the patients towards treatment. Prognosis is poor when cancer has been metastasized to distant organs, while the condition is responsive to treatment at early stages. The condition has also the characteristic of frequent relapsing making is difficult to treat.
Merkel cell carcinoma is a rare disease and because of this reason, the accurate prognosis of this condition cannot be done. It has been estimated that if the cancer is localized and limited to its area of origin, the 5-year survival rate is 78% while Merkel cell carcinoma with regional spreading has 51% 5-year survival rate. The 5-year survival rate drops down to a mere 17% when cancer gets metastasized to distant organs3.
What Is Merkel Cellcarcinoma Of The Skin
Merkel cell carcinoma of the skin is a rare form of skin cancer. It may be very aggressive and often metastasises to other parts of the body. It has also been called Toker tumour, cutaneousneuroendocrine carcinoma, trabecular cell carcinoma, and primary small-cell carcinoma of the skin.
Merkel cell carcinoma
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Who Is Likely To Have Merkel Cell Carcinoma
Anyone can develop MCC. You are at higher risk of developing this type of cancer if you:
- Have lighter-colored skin
- Have a weakened immune system
- Use tanning beds or ultraviolet light therapy for psoriasis
- Have another type of cancer, especially another type of skin cancer
The incidence of MCC in the US is about 0.6 per 100,000 people per year. This is up about 4X since 1986. However, it must be noted that diagnosis has improved, the population is getting older, and immunosuppressant medications are rising. It is estimated that about 700 people with MCC die per year. The rate of death is about one in three. Melanoma, also a dangerous cancer, is said to kill about one in nine people per year.
Sun Exposure And Having A Weak Immune System Can Affect The Risk Of Merkel Cell Carcinoma
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for Merkel cell carcinoma include the following:
- Being exposed to a lot of natural sunlight.
- Having an immune system weakened by disease, such as chronic lymphocytic leukemia or HIVinfection.
- Taking drugs that make the immune system less active, such as after an organ transplant.
- Having a history of other types of cancer.
- Being older than 50 years, male, or White.
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Diagnosing Merkel Cell Carcinoma
Merkel cell carcinoma may be detected during a physical exam by your doctor or dermatologist. Your doctor will check for lumps, lesions, or irregularly shaped moles. They may also ask you for a detailed medical history, history of sun exposure, and if anyone in your family has had skin cancer.
If your doctor finds anything abnormal, they may perform a skin biopsy to check for cancer. During a skin biopsy, a tiny amount of the lump is removed and viewed under a microscope.
If the biopsy is positive for Merkel cell carcinoma, your doctor will run additional tests to determine the stage and extent of the cancer. These tests may include:
- a sentinel lymph node biopsy, to find out if the cancer has spread to your lymph nodes
Depending on the stage of the cancer and your overall health, your treatment options may include one or more of the following:
- surgery to remove the tumor and any affected lymph nodes
- radiation treatment, which directs high energy beams at the cancer cells
- chemotherapy drugs
Your doctors will discuss the benefits of each option, as well as the possible risks and side effects.
What Causes Merkel Cell Carcinoma
The causes of Merkel cell carcinoma are not fully understood. The Merkel cells are connected to the nerve endings in the skin that are responsible for the sense of touch. Recent research points to a common virus that likely plays a role in development of this rare skin cancer, the Merkel cell polyomavirus. This virus lives on the skin and doesnt have any symptoms and is very common. Its not clear why it sometimes may cause this rare skin cancer.
These are thought to be the risk factors for Merkel cell carcinoma:
- Excessive UV exposure
- Light skin color
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Cancer May Spread From Where It Began To Other Parts Of The Body
- Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor in another part of the body.
- Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor in another part of the body.
The metastatic tumor is the same type of cancer as the primary tumor. For example, if Merkel cell carcinoma spreads to the liver, the cancer cells in the liver are actually cancerous Merkel cells. The disease is metastatic Merkel cell carcinoma, not liver cancer.
What Does Merkel Cell Carcinoma Look Like What Are The Symptoms
Merkel cell carcinoma is different from other skin cancers in that it does not have a common identifiable trait across all cases.
Merkel cell carcinoma tumors:
- are most often found on sun-exposed areas of skin, such as the face, neck, and arms but they can start anywhere on the body.
- usually appear as firm, shiny skin lumps that don’t hurt. The lumps may be red, pink, purple, or blue.
- tend to grow very quickly.
Between 10 20% of cases present without skin involvement and are found as a swelling of a lymph node.
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Appearance Of Merkel Cell Carcinoma
MCC usually develops on sun-exposed skin as a painless, firm bump that can be red-purple or skin-colored. Patients frequently point out a new MCC to their doctor because a bump is growing rapidly and/or does not look like anything the patient has ever had before. Most MCCs are diagnosed when a skin biopsybiopsyThe removal of cells or tissue in order to determine the presence, characteristics, or extent of a disease by a pathologist usually using microscopic analysis. is performed to rule out another sun-induced skin cancercancerA term used to describe diseases in which abnormal cells continually divide without normal regulation. Cancerous cells may invade surrounding tissues and may spread to other regions of the body via blood and the lymphatic system. or to remove a presumed cyst. In the vast majority of cases, both the doctor and the patient are surprised by the diagnosis of MCC. For more examples of MCC tumors beyond those presented on this page, visit the Clinical Photos page.
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Clinical Manifestations Patient Evaluation Staging
MCC often appears as a painless, indurated, erythematous to violaceous nodule with a smooth, shiny surface on sun-damaged skin, and, less commonly, as a plaque with satellite metastases. Surface ulcerations are rare.236238,246,248,287 Heath and colleagues summarized clinical features of MCC in an acronym: AEIOU ,254 suggesting that the clinical appearance of MCC is rather nondescript. MCC occurs predominantly in the head and neck , followed by the extremities and trunk .287 Ten percent of these tumors are in the periocular areas .288 MCC also has been reported in extracutaneous sites, such as the vulva, endocervix, penis, esophagus, bladder, and calvaria.259,289292 The salivary glands, nasal cavity, lip, lymph nodes, vulva, vagina, and esophagus are the most common extracutaneous sites.243 The clinical differential diagnosis includes BCC, SCC, amelanotic melanoma, lymphoma, and metastatic disease. Histologic and immunohistochemical studies are the key to confirmation of the diagnosis.
Diana Bell, in, 2021
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What Is Merkel Cell Carcinoma
Cancer starts when cells begin to grow out of control. Cells in nearly any part of the body can become cancer, and can then spread to other parts of the body. To learn more see What Is Cancer?
Merkel cell carcinoma is a rare type of skin cancer. It starts when cells in the skin called Merkel cells start to grow out of control. MCC tends to grow quickly and can be hard to treat if it spreads beyond the skin.
How Is Merkel Cell Carcinoma Diagnosed
The diagnosis of Merkel cell carcinoma is made with a biopsy. This is a sample of tissue thats taken and tested in a lab. Tumor samples are removed with a needle or scalpel, or during surgery. They are checked with a microscope to see if cancer cells are present. Diagnosis of Merkel cell carcinoma can be difficult. It can look like many other types of cancer.
Early diagnosis and treatment of Merkel cell carcinoma is important to prevent the cancer from spreading. Be aware of any lumps, growths, moles, or other abnormal areas on your skin. Watch for new spots or areas that are changing. This can include skin marks that grow larger, bleed, crust, or itch. Your healthcare provider may recommend you do a skin self-exam once a month or more. See your healthcare provider if you have any new or changing marks on the skin.
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Squamous Cell Carcinomas Are More Likely To Spread
Like basal cell carcinomas, squamous cell carcinomas are curable and can usually be removed completely when caught in time. They are, however, more dangerous than BCC because of their higher likelihood to spread. SCC is more likely to grow into the deeper layers of skin and other tissues in the body than BCC. While basal cell carcinoma usually does not grow into other areas of the body, it can rarely grow into a large tumor on the skin.
Mcc Arising On The Right Temple
The tumor developed in an area of extensive sun damage. This lesion is a Merkel cell carcinoma and squamous carcinoma collision tumor, meaning the two tumors are directly adjacent to each other. Collision tumors such as this are caused by sunlight and are almost always negative for the Merkel cell polyomavirus.
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Who Gets Merkel Cell Carcinoma
Merkel cell carcinoma has an estimated incidence of 0.23 per 100,000 people in Caucasian populations, which is much less common than melanoma.
- Increasing numbers of Merkel cell carcinomas have been reported by some centres in recent years.
- Merkel cell carcinoma mainly affects older people, with most cases occurring after the age of 50.
- It is slightly more common in men.
- It occurs on parts of the body commonly exposed to sunlight, most often the head and neck.
- It is also more common and more serious in those that are immune suppressed, such as patients with solidorgan transplants, human immunodeficiency virus infection, haematologicalmalignancy or on drugs such as azathioprine.
Treatment Of Stage Iii 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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When To See A Doctor
If you notice a mole, freckle or bump that is changing in size, shape or color, growing rapidly or bleeding easily after minor trauma, such as washing your skin or shaving, make an appointment with your doctor.
Factors that may increase your risk of Merkel cell carcinoma include:
- Excessive exposure to natural or artificial sunlight. Being exposed to ultraviolet light, such as the light that comes from the sun or from tanning beds, increases your risk of malignant skin cancer. The majority of neuroendocrine carcinoma appear on skin surfaces that are frequently exposed to sun.
- A weakened immune system. People with weakened immune systems including those with HIV or those taking drugs that suppress the immune response are more likely to develop Merkel cell carcinoma.
- History of other skin cancers. neuroendocrine carcinoma is associated with the development of other skin cancers, such as basal cell or squamous cell carcinoma.
- Old age. Your risk of neuroendocrine carcinoma increases as you age. This cancer is most common in people older than age 50, though it can occur at any age.
- Light skin color. neuroendocrine carcinoma usually arises in people who have light-colored skin. Caucasians are much more likely to be affected by this skin cancer than other ethnicities.
What Is Merkel Cell Cancer
MCC is a rare, highly aggressive, and dangerous form of skin cancer. It starts when cells in the skin called Merkel cells start to grow out of control. Merkel cells are found in the top layer of the skin . These cells are close to our nerve endings and help us sense light touch. Merkel cells can grow quickly and can spread to other parts of the body when the cancer is still at an early stage.
MCC is most likely to be cured when it is found early. However, because MCC is an aggressive cancer, delays in detection can lead to a late diagnosis which makes treating it very difficult. In addition, it commonly comes back after treatment.
Though it is estimated that there are about 1600 cases of MCC each year in the United States, the incidence of the disease has more than tripled over the past 20 years. This is likely due to a combination of increased detection and more people having risk factors for MCC.
The exact cause of MCC is not known, though it appears that there is a strong link between MCC and the bodys immune system. For example, people who have had organ transplants, who are infected with HIV, or who have chronic lymphocytic leukemia, are at greater risk for developing MCC. This may be because patients with a weakened immune system are more likely to become infected by a virus called Merkel cell polyomavirus. In fact, about 80% of MCC lesions contain this virus.
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What Is The Treatment For Merkel Cell Carcinoma
There are a number of treatment options for Merkel Cell Carcinoma, which may be used in isolation or in combination depending on factors such as where the growth is, how advanced it is, and how fit the patient is for surgery.
The final treatment course will often depend on the carcinoma type, body area and of course patient preference. Your specialist will be able to discuss treatment options with you.