Seattle Multidisciplinary MCC TeamUniversity of Washington MCC ResearchFred Hutchinson Cancer Research Center Seattle Cancer Care Alliance/Skin Cancer

We would like to thank the American Cancer Society and the Jerry Wachter Fund for a grant to make this educational effort possible.

Frequently Asked Questions about Merkel Cell Carcinoma

 

About the Disease

About Treatment

About Beating Merkel Cell Carcinoma (MCC)

More Details about Merkel cell carcinoma (MCC)

 


 

About the Disease

What is Merkel cell carcinoma (MCC)?
MCC, also referred to as neuroendocrine carcinoma, arises from uncontrolled growth of Merkel cells in the skin. It is a rare skin cancer with an incidence of about 1500 US cases per year, and this number is continuing to increase. MCC will prove fatal in roughly 1/3 of patients and should receive immediate treatment.

What does MCC look like?
MCC usually develops on sun-exposed skin as a firm, painless, flesh-colored to red-violet bump. The initial small bump tends to grow rapidly over weeks to months. Some images can be seen here.

Where does MCC occur on the body?
MCC primarily occurs on highly sun-exposed skin such as the head/neck and arms, but it can occur anywhere on the body, including sun-protected areas such as the buttock.

What is a Merkel cell?
Merkel cells are found in the epidermis (outer layer of the skin). Although the exact function of Merkel cells is unknown, they are thought to be touch receptors. They have both sensory and hormonal functions and are sometimes referred to as neuroendocrine cells.

What causes MCC?
The exact causes of MCC are not known. Factors strongly associated with the development of MCC include fair skin, a history of extensive sun exposure, chronic immune suppression (organ transplants or HIV), and over age 50.

Who gets MCC?
Individuals over the age of 65 are far more likely to develop MCC. Fair skin and a history of extensive sun exposure also increase the chances. MCC is associated with weakened immune function, such as in patients with HIV or organ transplants. However, most people who get MCC are not immune suppressed.

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About Treatment

How should my treatment plan be determined?
Your case should be reviewed by a multidisciplinary team including surgical oncologists, radiation oncologists, medical oncologists, and dermatologists with significant experience in managing MCC to develop a plan that could then be carried out by your local physicians. It is important that you and your doctors realize that optimal management of MCC is different than that for small cell lung carcinoma and for melanoma. The most significant difference is the important role of radiation in managing many cases of MCC. Another important difference is that adjuvant chemotherapy has not been shown to improve survival in MCC.

How should MCC be treated?
In most cases, complete surgical excision of the tumor should be carried out immediately following a sentinel lymph node biopsy procedure. After a few weeks of healing, in many cases, radiation therapy to the primary tumor site and sometimes the draining lymph node area may be appropriate. If no cancer remains after surgery and radiation, we do not typically recommend adding adjuvant chemotherapy for a variety of reasons as outlined here.

Do I need surgery?
A surgical excision is usually performed after a diagnostic biopsy as the first step of appropriate treatment for MCC. The goal of surgery is to remove any remaining cancer that can be seen either by the naked eye or under a microscope.

Do I need a sentinel lymph node biopsy?
A sentinel lymph node biopsy (SLNB) is a procedure in which a radioactive dye is injected near the primary tumor site, and the one to three nearby lymph nodes that “light up” (take up the dye) are removed by the surgeon and sent for pathologic examination (microscopic evaluation by a pathologist). This SLNB procedure is usually performed at the time of surgery and is much less accurate if a wide surgical excision has already been done. SLNB is important because MCC can spread by lymphatic vessels very early in the disease. At such a time, disease spread is not detectable by palpation (feeling the lymph nodes) or by scans but is readily detected by this test. For these reasons, SLNB is the best way to determine if MCC has spread elsewhere in the body, and it can help determine if the local lymph nodes require treatment.

Do I need radiation?
Radiation is one type of treatment used in some cancers. Radiation Oncologists plan and administer this type of treatment. Radiation therapy is often an important part of treatment for MCC. Usually radiation is given to the primary site (on the skin) of the tumor. If the sentinel lymph node biopsy was positive, radiation is often indicated to those lymph nodes.

Do I need scans?
CT scans (or "CAT" scans), X-rays, MRI, bone scan and PET scans are different types of imaging to look inside the body. They are sometimes used to find cancer inside the body that is not evident from the outside. There is a lot of controversy about how to use such scans. They are particularly important if MCC has been found to have spread to the lymph nodes to evaluate if significant disease has also developed elsewhere. In general, scans are far less effective in detecting early spread than a sentinel lymph node biopsy.

Do I need chemotherapy?
Chemotherapy is a method of using drugs to kill cancer cells. MCC is usually initially responsive to chemotherapy, leading to significant shrinkage. However, MCC often quickly gains resistance, and the tumor can continue to grow despite receiving chemotherapy drugs. In general, chemotherapy is reserved for late stages of MCC. For disease that is not metastatic, good control can often be achieved with surgery and radiation alone. Chemotherapy is the main treatment of some cancers, such as small cell lung cancer. In MCC, however, no evidence exists that it prolongs life. Rather, it may actually shorten life, possibly by suppressing immune function, which is particularly important in MCC as compared to other types of cancer.

Where can my physician get more information on MCC?
Here are some useful resources that can be printed for your physician:
  • Concise overview of the February 2007 MCC presentation by Dr. Nghiem at the American Academy of Dermatology in Washington, DC.
  • NCCN Clinical Practice Guidelines in Oncology: Merkel cell carcinoma
  • MCC chapter from the 7th edition of Fitzpatrick's Dermatology in General Medicine written by Drs. Nghiem and Jaimes.
  • Gupta SG, Wang LC, Penas PF, Gellenthin M, Lee SJ, Nghiem P. Sentinel lymph node biopsy for evaluation and treatment of patients with Merkel cell carcinoma: The Dana-Farber experience and meta-analysis of the literature. Archives of Dermatology 2006;142:685-90. Click here for a PDF.
  • Kelly Garneski, B.A. and Dr. Nghiem's adjuvant therapy paper, which was published in the Journal of the American Academy of Dermatology.

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About Beating Merkel cell carcinoma

What are my chances of beating Merkel cell carcinoma?
Your chance of beating MCC depends on how advanced the disease was when it was diagnosed, what treatment you receive and if your immune system is functioning properly. The "stage" of your cancer refers to whether the cancer is only in the skin, has spread to the lymph nodes or has spread elsewhere in the body. In general, about 1/3 of all MCC patients will succumb to this disease.

How can I increase my chances of survival?
You can increase your chance of survival by working with a team of doctors that is familiar with the unique management of MCC. In most cases, getting a sentinel lymph node biopsy is the best way to determine how advanced your cancer is. MCC is not the same as small cell lung cancer, and thus should usually not be treated with chemotherapy alone. Similarly, MCC is not the same as melanoma, and thus extra consideration should be taken before treating with surgery alone. Another important aspect of your treatment is to continue to eat a healthy diet and get regular exercise to help keep your immune system and overall health strong.

What immune problems make beating MCC more difficult?
People with severe defects in "T lymphocytes" (key cells of the immune system) have a 10-30 times greater chance of developing MCC and twice the chance of losing the battle with MCC compared to people with a normal immune system. The conditions that cause such severe T lymphocyte defects include HIV (AIDS), taking immune suppressive drugs after solid organ transplantation, and certain immune cancers such as chronic lymphocytic leukemia. In contrast, suffering from more "colds" than average will not importantly affect your ability to fight MCC.

How does staging work for MCC?
In general, cancers are staged according to where the cancer is in the body: local (skin only), nodal (involves the lymph nodes) or metastatic (spread elsewhere in the body). The best survival is for local stages, the poorest is in metastatic stages with nodal disease somewhere in between. Currently there are four different staging systems for MCC, and this creates confusion. A new consensus staging system is being developed for MCC and should be available in 2008. Until that time it is safest to avoid staging terminology or to clarify "stage I" by saying "a 1 cm MCC with a negative sentinel lymph node biopsy". Click here for a staging table.

How does MCC metastasize?
Cancer cells from an MCC tumor can break off and go into the lymphatic system or the blood. These cells can then go elsewhere in the body where they can either be killed by the immune system or take residence in a new location and start growing as a metastasis (tumor in another part of the body).

Where can it spread?
MCC can spread to other areas of the skin, the lymph nodes, or anywhere else in the body including the liver, bone, lung, and brain.

Does a patient support group exist for MCC?
An active support group exists for MCC. Please visit MCC Google Group for an interactive discussion with other MCC patients and their friends and family members.

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More Details about Merkel cell carcinoma (MCC)

Is MCC hereditary/are my children more likely to get MCC?

Probably not. There are no data suggesting that MCC runs in families. In fact, there are no reported cases of MCC occurring in multiple members of one family. However, through our cohort of over 1000 patients and others who have contacted us directly, we are now aware of four separate families in which two closely related family members had MCC. We do not think this supports the notion that MCC risk particularly runs in families.

What kind of follow-up do I need?
After your initial treatment, you will need to be followed closely by a physician to do regular skin and lymph node exams and take a thorough history. Visits should be approximately every 3 months for year 1 and then every 3-6 months for year 2, and then annually after that.

Can I participate in research?
Yes. There are observational (clinical database) studies and therapeutic research trials ongoing. For a list of options click here.

How can I support MCC education and research?
MCC is a relatively recently described cancer type. Very little research and funding has existed for it. If you would like to help, please click here.

What issues should I be sure to discuss with my physicians?

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FAQ Discussion For MCC Patients Merkel cell carcinoma discussion/support group at "GoogleGroups."