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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Treatment Options By Stage
While various considerations help determine the best approach to treatment, the stage of MCC is often the most important factor.Below, learn about the stages and the recommended treatments.
MCC without obvious spread
After having an SLNB to check whether the cancer has spread, most people undergo a wide local excision. This procedure removes the cancerous cells from the skin.
If MCC is on the face or other sensitive areas, a person may instead undergo Mohs micrographic surgery, which helps preserve more healthy skin.
Some people may also, or alternately, undergo radiation therapy, especially targeting the surrounding lymph nodes.
MCC with spread
This can be very difficult to cure. A person may need:
- surgery to remove affected cells
- radiation therapy
- immunotherapy drugs, such as pembrolizumab or avelumab
When MCC comes back, doctors often recommend a combination of:
- surgery to remove more of the affected tissue, possibly with surrounding lymph nodes
- radiation therapy
- immunotherapy drugs, such as Keytruda or Bavencio
Recurring MCC can be very difficult to treat, especially if it comes back in different areas. If this happens, doctors tend to focus on reducing symptoms, not curing the disease.
Some people also try complementary therapies for MCC, including:
- naturopathic medicine
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What Are The Risk Factors For Mcc
In addition to infection with the Merkel cell polyomavirus in people with weakened immune systems, risk factors that have been associated with MCC include:
- Exposure to a lot of natural sunlight
- Exposure to artificial sunlight
- Being over 50 years of age
- Having lighter skin
- Having a history of other types of cancer
Treatment Of Merkel Cell Carcinoma
Merkel cell carcinoma is highly treatable with surgical and nonsurgical therapies, particularly if caught early. Treatments are often highly individualized, depending on a patients general health, as well as the tumors location, size, depth, and degree of spread.
Patients with Merkel cell carcinoma are usually first treated with surgery. Patients with more advanced disease may receive adjuvant treatments such as radiation therapy and chemotherapy following, or instead of, surgery.
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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.
Merkel Cell Carcinoma Is A Very Rare Disease In Which Malignant Cells Form In The Skin
Merkel cells are 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 of the skin or trabecular cancer, is a very rare type of skin cancer that forms when Merkel cells grow out of control. Merkel cell carcinoma starts most often in areas of skin exposed to the sun, especially the head and neck, as well as the arms, legs, and trunk.
Merkel cell carcinoma tends to grow quickly and to metastasize at an early stage. It usually spreads first to nearby lymph nodes and then may spread to lymph nodes or skin in distant parts of the body, lungs, brain, bones, or other organs.
Merkel cell carcinoma is the second most common cause of skin cancer death after melanoma.
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Chemotherapy Treatments For Merkel Cell Carcinoma
Chemotherapy refers to drugs that kill quickly dividing cells, including cancer cells.
However, adjuvant chemotherapy is not recommended in all patients.
Research so far has not shown a strong benefit from adjuvant chemotherapy for patients with Merkel cell carcinoma. Chemotherapy is generally recommended primarily for patients who have not responded to radiation and cannot receive immunotherapy.
Side effects include hair loss, fatigue, nausea, and vomiting. The drugs can sometimes cause patients’ health to deteriorate more.
Survival Rates For Merkel Cell Carcinoma
Survival rates can give you an idea of what percentage of people with the same type and stage of cancer are still alive a certain amount of time after they were diagnosed. They cant tell you how long you will live, but they may help give you a better understanding of how likely it is that your treatment will be successful.
Keep in mind that survival rates are estimates and are often based on previous outcomes of large numbers of people who had a specific cancer, but they cant predict what will happen in any particular persons case. These statistics can be confusing and may lead you to have more questions. Talk with your doctor about how these numbers may apply to you, as he or she is familiar with your situation.
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Moffitt Cancer Centers Approach To Squamous Cell Carcinoma
At Moffitt Cancer Center, our multispecialty team of cancer experts takes a highly individualized approach to squamous cell carcinoma treatment. We offer the latest diagnostic and treatment options, and we work closely with each patient to offer customized guidance and help ensure the best possible outcome. For instance, there are many steps a patient can take to improve his or her own squamous cell carcinoma prognosis regardless of the general survival rate such as:
- Performing self-examinations from head to toe, including parts of the body that are not regularly exposed to UV rays, at least monthly, and promptly reporting any suspicious or unusual changes in skin texture or appearance to a physician
- Seeing a physician for a professional skin cancer examination yearly
- Avoiding exposure to the suns ultraviolet rays while outdoors, preventive measures include seeking shade, wearing sunglasses and a brimmed hat, covering up with clothing and using a broad spectrum sunscreen with both UVA and UVB protection
- Never using indoor tanning beds
If youd like to learn more about the squamous cell carcinoma survival rate, the experts at Moffitt can put this information into the proper context for you and help you take appropriate steps to achieve the best possible outcome. Call or complete a new patient registration form online. We see patients with and without referrals.
Radiation Treatments For Merkel Cell Carcinoma
Some patients may undergo radiation a few weeks after surgery to lower the risk of the cancer returning. The radiation destroys any cancerous cells left behind after surgery.
If you have stage IV Merkel cell carcinoma or cannot have surgery, radiation or immunotherapy may be the first treatment step.
Common side effects of radiation include skin irritation, changes in skin color or texture, hair loss, and fatigue.
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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 , 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.
What Questions Should I Ask My Doctor If I Have Merkel Cell Carcinoma
If your doctor diagnoses MCC, you may have many questions about your condition. These may include:
- How can I know whether the cancer has spread?
- Which treatments will be most beneficial for me?
- Is there a chance my cancer could come back after treatment?
- How can I protect my skin to lessen my chances of developing cancer again?
Make sure you contact your doctor if you notice any changes in your skin that worry you.
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Wife Advocate Motivator: When Someone You Love Is Diagnosed With Merkel Cell Carcinoma
Daisy Mae the puppy visits James in the hospital during his first bout with sepsis.
Jamie Gentrys husband, James Gentry, had been talking about losing weight for years when he shed 25 pounds over a few months in 2017. James suggested the transformation could be a result of his own efforts, but Jamie was unconvinced and deeply concerned.
Hes a big man, and ever since I met him hes been talking about losing weight, Jamie says, chuckling. Never changes what he does or what he eats, but he was going to lose the weight. I said, No, no, youve been trying for 12 years its not suddenly working. It really freaked me out. Jamies instincts were spot on she knows now, two years later, that a rapidly metastasizing Merkel cell carcinoma was the reason for James sudden weight loss.
But back in May of 2017, no one seemed to know much about the lump James, then 59, noticed on his leg. It took several visits to his primary care physician and his cardiovascular surgeon to get the bump more than a passing glance. James persisted, as he had noticed the spot seemed to be getting bigger. Eventually, his physician ordered a Doppler ultrasound to investigate. The radiologist admitted to having no idea what he was seeing. James PCP finally decided that the tumor, whatever it was, should be removed.
Are There Other Research Efforts Ongoing That You Would Like To Highlight
There is a great opportunity with MDM2 but related to that are MDM4 . MDM2 and MDM4 work collaboratively with each other to get rid of p53. Our lab has found that we can independently inhibit MDM2 and MDM4, and that combination will synergistically activate p53. There is a golden opportunity to target MDM2 and MDM4 together, so with some luck, we will have an approval for MDM2 inhibitors . This can also be expanded for drugs that inhibit both .
Another pathway weve uncovered is LSD1. There is an opportunity for LSD1 inhibitors in MCC and we have talked to a few companies . Because MCC is rare, it makes it challenging to put together a study. The MCC that we have studied has been very sensitive to LSD1 inhibitors.
Because the virus is driving a normal cell , weve found several other targets that could be opportunities for therapy. We are just getting started and there is certainly a really interesting possibility that one of the drug targets could synergize with checkpoint inhibitors. We could have a rational strategy to inhibit checkpoint blockade but then work with the virus to activate the immune response. We will look at some inhibitors in that regard too. Hopefully, we will all be reading about that soon.
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Immunotherapy Treatments For Merkel Cell Carcinoma
If the cancer has spread, your doctor may recommend immunotherapy. Clinical trials suggest immunotherapy drugs may be more effective against Merkel cell carcinoma than chemotherapy.
Two FDA-approved immunotherapy drugs for Merkel cell carcinoma are avelumab and pembrolizumab. Side effects of immunotherapy drugs can include fatigue, inflammation, itchiness, skin reactions, high or low blood pressure, and flu-like symptoms.
Regional Lymph Node Surgery
In some case series, local-regional recurrence rates are high when pathologic nodal staging is omitted. Surgical nodal staging in clinically negative patients has identified positive nodes in at least 25% to 35% of patients. In one retrospective series of 213 patients who underwent surgical treatment of the primary tumor and evaluation of the draining nodes, nodal positivity was found in 2 of 54 patients with small tumors and 51 of 159 patients with tumors larger than 1.0 cm.
The role of elective lymph node dissection in the absence of clinically positive lymph nodes has not been studied in formal clinical trials. In small case series, ELND has been recommended for larger primary tumors, tumors with more than ten mitoses per high-power field, lymphatic or vascular invasion, and the small-cell histologic subtypes.
Sentinel lymph node biopsy has been suggested as a preferred initial alternative to complete ELND for the proper staging of MCC. SLN biopsy has less morbidity than complete nodal dissection. Furthermore, for MCC sites with indeterminate lymphatic drainage, such as those on the back, SLN biopsy techniques can be used to identify the pertinent lymph node bed. If performed, SLN biopsy is done at the time of the wide resection when the local lymphatic channels are still intact.
Several reports have found the use of SLN biopsy techniques in MCC to be reliable and reproducible. However, the significance of SLN positivity remains unclear.
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Treatment Of Stage Iv Merkel Cell Carcinoma
For information about the treatments listed below, see the Treatment Option Overview section.
- A clinical trial of immunotherapy .
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.
Risks And Causes Of Mcc
As with other types of skin cancer, long term exposure to sunlight increases your risk of getting MCC. Other factors that can increase your risk include:
- ultraviolet light and psoralen ultraviolet light treatment for certain skin conditions such as psoriasis
- conditions or treatments that can weaken your immune system, including people who have had an organ transplant and people who have AIDS
Age is a risk factor for most types of cancer, including MCC. The average age of diagnosis of MCC is around 70 years old.
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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.