Masonic Cancer Center, University of Minnesota
Merkel cell carcinoma is a very rare disease in which malignant (cancer) cells form in the skin.
Merkel cells are hormone-making cells found in the top layer of the skin. These cells are very close to the nerve endings that receive the sensation of touch. Merkel cell carcinoma, also called neuroendocrine carcinoma, is a very rare type of skin cancer that develops when Merkel cells grow out of control. Merkel cell carcinoma starts most often in areas of skin exposed to the sun, such as the head, neck, arms, and legs.

Anatomy of the skin, showing the epidermis, dermis, and subcutaneous tissue. Merkel cells are in the layer of basal cells at the deepest part of the epidermis and are connected to nerves.
Merkel cell carcinoma tends to grow quickly and to metastasize (spread) at an early stage. It spreads first to nearby lymph nodes and then may spread to the liver, bone, lungs, brain, or other parts of the body.
Sun exposure and having a weak immune system can affect the risk of developing 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. People who think they may be at risk should discuss this with their doctor. Risk factors for Merkel cell carcinoma include the following:
Merkel cell carcinoma usually appears as a single painless lump on sun-exposed skin.
This and other changes in the skin may be caused by Merkel cell carcinoma. Other conditions may cause the same symptoms. A doctor should be consulted if changes in the skin are seen.
Merkel cell carcinoma usually appears on sun-exposed skin as a single lump that is:
Tests and procedures that examine the skin are used to detect (find) and diagnose Merkel cell carcinoma.
The following tests and procedures may be used:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
Prognosis also depends on how deeply the tumor has grown into the skin.
After Merkel cell carcinoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.
The process used to find out if cancer has spread to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following tests and procedures may be used in the staging process:
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
The following stages are used for Merkel cell carcinoma:

Pea, peanut, walnut, and lime show tumor sizes.
Stage IA
In stage IA, the cancer is smaller than 2 centimeters in diameter and has not spread to lymph nodes or other parts of the body.
Stage IB
In stage IB, the cancer is 2 centimeters or larger in diameter and has not spread to lymph nodes or other parts of the body.
Stage II
In stage II, the cancer may be any size and has spread to nearby lymph nodes, but has not spread to other parts of the body.
Stage III
In stage III, the cancer may be any size and has spread beyond nearby lymph nodes to other parts of the body.
Recurrent Merkel cell carcinoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the skin, lymph nodes, or other parts of the body. It is common for Merkel cell carcinoma to recur.
There are different types of treatment for patients with Merkel cell carcinoma.
Different types of treatments are available for patients with Merkel cell carcinoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Three types of standard treatment are used:
Surgery
One or more of the following surgical procedures may be used to treat Merkel cell carcinoma:

Mohs surgery. A surgical procedure to remove skin cancer in several steps. First, a thin layer of cancerous tissue is removed. Then, a second thin layer of tissue is removed and viewed under a microscope to check for cancer cells. More layers are removed one at a time until the tissue viewed under a microscope shows no remaining cancer. This type of surgery is used to remove as little normal tissue as possible and is often used to remove skin cancer on the face.

Sentinel lymph node biopsy of the skin. A radioactive substance and/or blue dye is injected near the tumor (first panel), the injected material is detected visually and/or with a probe (middle panel), and the sentinel nodes (the first lymph nodes to take up the material) are removed and checked for cancer cells (last panel).
Even if 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. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI Web site (see also: clinical trials at the Masonic Cancer Center) .
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.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Treatment of stage I Merkel cell carcinoma may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I neuroendocrine carcinoma of the skin (see also: clinical trials at the Masonic Cancer Center) . For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Treatment of stage II Merkel cell carcinoma may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II neuroendocrine carcinoma of the skin (see also: clinical trials at the Masonic Cancer Center) . For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Treatment of stage III Merkel cell carcinoma is usually chemotherapy.
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III neuroendocrine carcinoma of the skin (see also: clinical trials at the Masonic Cancer Center) . For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Treatment of recurrent Merkel cell carcinoma may include the following:
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent neuroendocrine carcinoma of the skin (see also: clinical trials at the Masonic Cancer Center) . For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
For more information from the National Cancer Institute about Merkel cell carcinoma, see the following:
For general cancer information and other resources from the National Cancer Institute, see the following:
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
2006-02-21Date last modified: 2009-08-28