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How Rare Is Merkel Cell Carcinoma

Diagnosing Merkel Cell Carcinoma

Dermatologists warn of Merkel Cell Carcinoma during Skin Cancer Awareness Month

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 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.

Sunlight & Merkel Cell Carcinoma

It is believed that ultraviolet radiation from the sun plays a significant role in the development of MCC. MCC is most commonly found on sun-exposed areas of the body in older Caucasian individuals, who may also have other sun-induced skin cancers. There are more MCC cases in sunny climates as opposed to areas with less sun . While extensive sun exposure is a risk factor for MCC, MCC can also occur on sun-protected skin, such as a hair-covered scalp.

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What Are The Symptoms Of Merkel Cell Carcinoma

In most cases, MCC causes a small reddish or purplish lump or bump to form on areas of skin often exposed to ultraviolet light, such as the face, neck, arms, and hands. The lumps usually do not cause pain. In some cases, bumps appear inside the nostrils or esophagus. Lumps or bumps may crack open and bleed.

How Dangerous Is Mcc

Merkel Skin Cancer : Merkel Cell Carcinoma Incyte ...

While MCC is about three to five times more likely to be deadly than melanoma, with early detection, MCC can be treated successfully. If you think you might have MCC, see your doctor. Treatment becomes increasingly difficult once the disease has spread, but new options are now available. Thanks to advances in the field of immunotherapy, MCC survival rates are improving.

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Merkel Cell Carcinoma Symptoms

Typically, the first sign of MCC is the appearance of a red or purple lump or bump on the skin. They may be firm and dome-shaped, and usually doesn’t cause any pain.

Unfortunately, this symptom can be mistaken for a number of other skin blemishes, so it’s best to get the lump examined as soon as possible.

Some things an MCC tumor can mimic include:

  • Bites
  • Styes
  • Hair follicles

One sign that the lump or bump might be an MCC tumor is if it grows very quickly.

Key Points About Merkel Cell Cancer

  • Merkel cell cancer is a rare type of skin cancer. It forms in the Merkel cells. These cells are found in the outer layer of the skin.
  • Merkel cells are very close to nerve endings. They help the skin sense light touch.
  • Being exposed to a lot of UV light can raise your risk for this cancer.
  • Merkel cell cancer often looks like firm, shiny lumps on your skin that dont hurt. They may be red, pink, or blue.
  • This cancer grows and spreads quickly.
  • Treatment includes surgery, radiation, chemotherapy, and immunotherapy. One type of treatment or a combination of treatments may be used.

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What Are The Signs And Symptoms Of Mcc

MCC usually appears as a single, painless bump on the skin. These bumps are called lesions. The most common site for MCC to occur is the head and neck, followed by the arms and legs. The lesion is typically red/pink, although blue/purple is also common. MCC lesions vary in size but when the cancer is found, they are on average about 17 mm .

Stanford Expertise In Merkel Cell Carcinoma

Merkel Cell Carcinoma Explained by Mayo Clinic

The multispecialty Merkel Cell Carcinoma Program at Stanford provides a comprehensive treatment approach for patients with MCC and is investigating novel targeted-and immunotherapies to treat patients with advanced disease.

For this reason, the Stanford Merkel Cell Carcinoma Program offers a multispecialty approach to the treatment of MCC, utilizing the expertise of Stanford surgeons for wide local excision and sentinel lymph node biopsy staging.;

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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, such as people who have had an organ transplant, are at increased risk for this 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.

  • Being male.;Men are more likely to get Merkel cell carcinoma.

Researchers have found that Merkel cell carcinoma almost always shows infection with a virus known as Merkel cell polyomavirus . It is not known how the virus may contribute to the growth of this cancer. Most people are infected with this virus at some point. But very few people develop this cancer.

How Long Does It Take A Merkel Cell Carcinoma To Progress To An Advancedstate

Merkel cell carcinoma is considered anaggressive type of cancer,even more aggressive than melanoma. This means that itrapidly grows and spreads to nearby tissues or distant parts of the body.Estimates of the time it takes for MCC to progress from an early to advancedstage, or to cause death, is unknown because MCC is rare, the diagnosis can bedifficult to make, and treatments vary. One study of 195 patients didmention that approximately two-thirds of Merkel cell carcinomas expanded insize rapidly over the course of three months.

Many factors are thought to influence theprogression of MCC, includingthe size and location of the tumor, whether or not the tumor has spread tolymph nodes or distant sites in the body, whether the cancer regrows aftertreatment , andwhat types of treatments are used. Unfortunately, approximately onethird of individuals affected with MCC are diagnosed when the cancer isalready advanced, meaning the cancer has metastasized to the lymphnodes or farther.The advanced state at diagnosis is due to the fact that MCC can be difficultto diagnose because it appears similar tobenign skin findings, is oftenpainless, and grows so rapidly.

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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.

What Does Mcc Look Like

Merkel Cell Carcinoma Histology : Merkel Cell Carcinoma A ...

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

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Merkel Cell Carcinoma: Interdisciplinary Management Of A Rare Disease

Boban M. Erovic

1Department of OtolaryngologyHead and Neck Surgery, Medical University of Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria

Academic Editor:

Abstract

Background. The goal of this paper is to review contemporary multidisciplinary treatment with reference to Merkel cell carcinoma. Management of this rare but highly aggressive skin cancer is a complex undertaking that necessitates an understanding of its etiology, epidemiology, clinical presentation, and the coordinated work of several clinical specializations. Recent Findings. The contemporary literature employs a multidisciplinary approach to achieve the best patient’s treatment. . This paper presents an algorithm for contemporary management for the rare and aggressive Merkel cell carcinoma. Multidisciplinary approach in a tumor center provides high-quality care for patients with Merkel cell carcinoma.

1. Introduction

Merkel cell carcinoma is a rare neuroendocrine skin tumor, with a high risk of local and distant spread. The incidence of MCC is 0.32 per 100.000 , showing an increasing incidence with advanced age and in male Caucasians .

Progression in incidence might be linked to the constantly increasing exposure to ultraviolet B radiation considering the fact that MCCs are localized frequently in sun-exposed areas of the body. Other known risk factors are immunosuppression in transplant recipients , HIV , and in particular Merkel cell carcinoma polyomavirus infection .

2. Multidisciplinary Management

Can Merkel Cell Carcinoma Be Prevented

You cannot prevent MCC. You can reduce your likelihood of developing this condition â and any skin cancer â by protecting your skin from exposure to ultraviolet light. Protecting your skin from UV light may include:

  • Limiting your exposure to sunlight
  • Applying and reapplying appropriate sunscreen products while outside
  • Wearing protective clothing while outside
  • Avoiding tanning beds or sunlamps

You can also examine your skin regularly for any signs of change. If you notice any skin changes, see your doctor as soon as possible.

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Who Is At Risk For Merkel Cell Cancer

A risk factor is anything that may increase your chance of having a disease. The exact cause of someones cancer may not be known. But risk factors can make it more likely for a person to have cancer. Some risk factors may not be in your control. But others may be things you can change.

The risk factors for Merkel cell cancer are:

  • Being exposed to a lot of ultraviolet rays from the sun or other sources such as tanning beds or PUVA treatments
  • Not protecting your skin from the sun
  • Having light-colored skin
  • Having a weakened immune system, such as from HIV or an organ transplant
  • Arsenic exposure
  • Infrared light exposure, such as lasers or heat lamps

Researchers have found that Merkel cell cancer almost always shows infection with a virus known as Merkel cell polyomavirus . Doctors dont know much about MCV yet, or how it might be linked to cancer. Most people are infected with this virus at some point. But very few people develop this cancer.

Diagnosing And Treating Mcc

Merkel Cell Carcinoma Warning Signs

One of the issues faced by healthcare providers is that an MCC lesion may look like a cyst or swelling from an inflamed hair follicle. In a clinical study, 56% of Merkel cell carcinomas were initially thought to be benign by physicians. If you notice any new or changing lesions on your skinno matter how minortalk with your healthcare provider. It is important not to delay time to detection.

If your healthcare provider suspects that a lesion on your skin may be MCC, he or she will likely take a careful medical history and perform a physical exam. If your healthcare provider thinks you may have MCC, he or she will do a skin biopsy. That means removing the lesion and sending it to a lab where it will be viewed under a microscope to examine and confirm whether there are any cancerous cells.

Treatment of MCC is based on the stage of the disease and the overall health of the patient. The main treatments are surgery , radiation, and chemotherapy and other systemic therapies .

Research is underway to better understand this disease and to discover treatments. For more information about available clinical trials for Merkel cell carcinoma, go to;clinicaltrials.gov or;Pfizer: Find a Trial.

Learn more about sun safety

Exposure to the sun is one risk factor for MCC that we can control. We all need some sun exposure, and its important to engage in some physical activity outdoors, but too much sun can be harmful. Find out what you can do to stay healthy in the sun.

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Tumorlike Lesions Of Fibrous Or Elastic Tissue

The next set of localized abnormalities includes lesions that can emulate tumors. These benign lesions arise from either elastic tissue or dermal collagen. Often appearing to;develop from the fascia, with extension into dermal and subcutaneous fat, these benign fibrotic nodules must be distinguished from fibrosarcomas.

How Quickly Does Merkel Cell Carcinoma Spread

A physical exam may reveal a new skin lesion, an enlarged lymph node or an enlarged liver that may signal the spread of MCC. A lesion of metastatic MCC may appear as a 1-3 cm, flesh-colored to red-purple bump that feels firm, is deeper compared to the primary lesion, and grows rapidly over a period of 2-4 weeks.

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Premalignant Fibroepithelial Tumor Of Pinkus

The fibroepithelial tumor of Pinkus is best considered a type of basal cell carcinoma .;Originally identified by Herman Pinkus in the 1950s, the premalignant fibroepithelial tumor of Pinkus is a distinctive tumor that predominantly occurs on the lower trunk and thighs, often appearing as pedunculated lesions resembling fibromas.;These tumors typically develop between the fourth and sixth decade of life.

Histologically, the tumor is composed of epithelial cells resembling basal cells surrounded by a fibrous stroma. ;With further cellular characterization, Heenen and colleagues demonstrated that these basal-like epithelial cells have different cell cycle characteristics in comparison;with normal epithelial cells. ;It is suggested that this dysregulation of the cell cycle is a premalignant phenomenon and represents the potential progression into a transformed, cancerous cell. In rare cases, these faint-staining epithelial cells are replaced by more aggressive, smaller, dark-staining basaloid cells. These new basaloid cells eventually overtake the lesion, consequently, developing into an invasive basal cell epithelioma.

Who Is Likely To Have Merkel Cell Carcinoma

Dermoscopy Made Simple: Rare and Unusual tumours

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.

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Four Types Of Standard Treatment Are Used:

Surgery

One or more of the following surgical procedures may be used to treat Merkel cell carcinoma:

After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with cancer. It is used to treat Merkel cell carcinoma, and may also be used as palliative therapy to relieve symptoms and improve quality of life.

Chemotherapy

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