It is important to emphasize that optimal care depends on many issues that are highly variable between patients. It is thus best to obtain care from a multi-disciplinary team of physicians with significant experience with this disease and who take into consideration many factors such as: overall health of the patient, immune suppression, node status, tumor size and location, lymphovascular invasion in the primary lesion, age, and importantly, the patient's personal philosophy in making decisions affecting quality vs quantity of life.
Treatment is generally based on the stage of the disease. There are four major
treatments for MCC: 1) surgical excision of the primary lesion, 2) lymph node surgery, 3)
radiation therapy, and 4) chemotherapy. Each will be reviewed below in greater detail.
Depending on how well a patient tolerates the treatments, surgery, radiation therapy and
chemotherapy may be given at the same time or one after the other.
For most cases of MCC, excision of the primary lesion with a greater than or equal to 2 cm margin (wide surgical excision) may be a recommended part of care. As discussed above, it is important for almost all cases in which there is no obvious lymph node disease to also undergo sentinel lymph node biopsy at the time of wide surgical excision to determine whether or not microscopic disease is present. When microscopic disease is found, radiation of the affected nodes, or surgical removal of the remaining lymph nodes in a draining lymph node basin (lymph node dissection) may be indicated and results in a high 'cure' rate for the affected nodal region.
We typically recommend radiation therapy to the site of the primary lesion and to the draining lymph node basin in stage I, II and III disease. Chemotherapy should be reserved for patients with stage IV disease.
Metastatic disease should be treated with radiotherapy and/or chemotherapy. The purpose of treatment in stage IV disease is palliative. Palliative therapy is given to relieve symptoms, such as pain, and to help patients live more comfortably.
It is important to emphasize that optimal care depends on many issues that are highly variable between patients. It is thus important to obtain care from a multi-disciplinary team of physicians with significant experience with this disease and who take into consideration many factors such as: overall health of the patient, immune suppression, node status, tumor size and location, age, and the patient's personal philosophy in making decisions affecting quality vs. quantity of life.
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Wide surgical excision
The goal of wide excision is to control local recurrence and lymph node metastases. MCC should be removed with clear margins as judged by pathology examination. As noted below however, even with margins >2cm, surgery alone has a very high recurrence rate up to 42% depending on the study. This recurrence rate can typically be cut in half or better by the addition of radiation therapy.
To optimize the appearance and function of your scar, your surgeon may make an excision in the shape of a football (ellipse). The length of your scar will be roughly three times the diameter of the excision around the tumor (when possible, the excision is usually 2 cm beyond the tumor). Therefore, the scar may be up to 8 times as long as the width of the original MCC tumor.
Schematic of wide surgical excision with MCC shown in gray.
Depending on your general health and the location of the MCC, surgery may not
be possible. In that case, radiation therapy may be used alone. A recent study
suggests that radiation therapy alone may be as effective as both radiation
therapy and surgery. (Mortier, 2003).
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Mohs micrographic surgery
Cutaneous neoplasms that develop on the head & neck are more likely to recur and metastasize via lymphatics to regional lymph nodes. It may not be possible to excise some MCCs on the face with a margin of at least 2 cm. For these reasons, your doctor may refer you to Mohs micrographic surgery. Mohs micrographic surgery allows for conservation of skin to maintain function and appearance of sensitive areas of the body (face). Complete removal of the MCC is evaluated under the microscope during surgery. Our analysis of published studies on the treatment of MCC suggests that radiation therapy needs to be added to Mohs micrographic surgery to optimize control of the disease. Addition of radiation therapy to Mohs micrographic surgery appears to cut recurrence by roughly one-half (see table below, adapted from Longo & Nghiem, 2003).
Numbers in parentheses are % of patients with each type of recurrence
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Radiation therapy & side-effects
Radiation therapy, also referred to as radiotherapy or XRT, is the treatment of cancer with penetrating beams of energy waves or streams of particles that can destroy cancer cells. Radiation therapy is delivered to the cancer cells and a small margin of surrounding normal tissue, sometimes referred to as the radiation field. Radiation therapy damages the genetic material of cancer cells making them unable to grow. Radiation therapy also damages healthy cells in the field of radiation. Adjuvant radiation therapy is radiotherapy that is used to destroy any cancer cells that may remain after surgery and/or chemotherapy.
We typically recommend radiation therapy to the primary site as well as the draining lymph node basin. This recommendation is based on numerous studies showing marked improvement in control of disease at the primary site and draining lymph node basin when radiation therapy is added (Lewis, 2006).
Local recurrence is 3.7 times more likely if surgery only
Our analysis of the available literature
and our experience caring for dozens of patients with MCC at the DFCI since
2000 show that radiotherapy is associated with a statistically significant improvement
in local and nodal recurrence, but not in survival. Where wide excision is not
possible, a recent study suggests that radiotherapy alone may be as effective
as both radiotherapy and surgery. These data suggest a role for radiotherapy
to the primary site and draining lymph node basin in stage I, II & III disease.
The dose of radiotherapy is measured in units called Gray (Gy). The total dose of your radiation therapy should be greater than or equal to 50 Gy. Radiation therapy is usually administered in a doctor's office in divided doses for 10-15 minutes, 5 days a week (e.g., Monday through Friday) over a set number of weeks (e.g., 5 weeks).
Low-risk MCC patients who may not require adjuvant radiation
"Patients who have very low risk disease, as defined by having all of the favorable features below, are likely to benefit very little from adjuvant radiation therapy. We are currently not routinely recommending radiation therapy for such low-risk cases."
1) Primary tumor ≤ 1 cm in largest dimension
2) Negative sentinel lymph node biopsy
3) No chronic immune suppression (HIV disease, leukemia/lymphoma, transplant of heart or kidney or liver)
4) No lymphovascular invasion in the primary tumor (pathologist may need to be requested to go back to the original biopsy and specifically comment on this feature's presence or absense).
5) Confidently negative microscopic margins after excision
Possible side-effects of radiation therapy
Possible side-effects of radiation therapy in the area being treated include
loss of hair, skin irritation (like a sunburn), and changes in the color and
texture of the skin. Radiation to a draining lymph node basin may cause swelling
of the arm or leg on the same side that may be long-lasting in unusual cases.
A frequent side effect is fatigue, which usually resolves soon after the radiotherapy
is stopped. Accordingly, it is important to eat a well-balanced diet and get
plenty of rest. A radiation oncologist may adjust the dose or schedule of your
radiation therapy based on your side effects.
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Chemotherapy & side-effects
Chemotherapy is the treatment of cancer with drugs that can destroy cancer cells. Chemotherapy targets cells that divide quickly, including cancer cells that grow and multiply without control as well as healthy cells that divide rapidly. Adjuvant chemotherapy is chemotherapy that is used to destroy any cancer cells that may remain after surgery and/or radiation therapy have cleared the readily detected cancer cells.
Chemotherapy drugs may be given intravenously or orally on certain days of the week over a set number of weeks. For example, you may receive chemotherapy Monday, Wednesday and Friday on weeks 1, 4, 7 and 10.
Combination chemotherapy is when two or more drugs are given at the same time as they work better together than alone. There is no well-established combination chemotherapy for Merkel cell carcinoma. MCC has similarities to other neuroendocrine carcinomas, such as small cell lung cancer. For this reason, your medical oncologist may use drugs that have shown effectiveness against small cell lung cancer. Etoposide and carboplatin is one such regimen.
Information on treatment with chemotherapy (etoposide and carboplatin) comes from a study of 53 patients that demonstrated a 76% overall survival at 3 years in patients treated with both chemotherapy and radiotherapy (Poulsen, 2003). It is not possible to determine from this study whether adjuvant chemotherapy improves outcomes because 1) the survival was essentitally unchanged from that expected for a group of similarly staged (I, II, & III) MCC patients and 2) it was not randomized.
The following are reasons that adjuvant chemotherapy may not be routinely recommended. In the absence of definitive data, the decision to use chemotherapy should be customized to each situation and should be discussed with your medical team.
'Adjuvant' chemotherapy is used to destroy any cancer cells that may remain AFTER surgery and/or radiation therapy have cleared all detectable cancer cells.
- Mortality: there is a 4 to 7% acute death rate due to adjuvant chemotherapy in MCC partly due to the fact that these patients are often elderly (Voog 1999; Tai 2000).
- Morbidity: Neutropenia (low white blood count) occurs in 60% of patients with fever, and sepsis in 40% (Poulson 2001).
- Decreased quality of life: this is quite severe in this older population, including fatigue, hair loss, nausea and vomiting.
- Resistance to chemotherapy: Merkel cell carcinoma that recurs after chemotherapy is less responsive to later palliative chemotherapy.
- Immunity: chemotherapy suppresses immune function and this is known in general to be very important in preventing and controlling MCC.
- Apparent poorer outcomes: among patients with nodal disease, there was a 60% survival if chemotherapy was not given among 53 patients. In contrast, survival was only 40% among node positive MCC patients who did receive adjuvant chemotherapy (Allen 2005). While this is not a randomized trial and was not statistically significant, it certainly does not suggest a survival benefit for administering adjuvant chemotherapy.
Comparison of disease-specific survival in patients with lymph node disease who either received (23 patients) or did not receive (53 patients) chemotherapy. Adapted from Allen, 2005.
Pazopanib (Votrient) is an oral medication that has been reported to help control metastatic MCC in some cases. Please click here for more details and a Frequently Asked Questions handout.
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Complementary & Alternative Therapies
Patients often ask what complementary & alternative approaches to traditional therapies are available for MCC. No studies have been done to test these approaches, but some of our patients have used alternative therapies and are very happy with them. We also routinely encourage our patients to augment their physical activity through daily participation in their favorite forms of exercise. Please remember that this information alone can't take the place of health care or human services you may need. Because of possible adverse side effects and drug interactions, we strongly encourage you to consult your primary physician and oncologist before starting any new treatment regimen and to notify your pharmacist of any supplements you are taking.
[The Natural Medicines Database]
High quality informations about what natural medicines are likely to be safe and effective can be found at this website.
Consultation with a Naturopathic Physician
Dr. Leanna Standish at Bastyr University in Kenmore, WA (near Seattle). We are working with Dr. Standish to investigate whether or not certain complementary/alternative medications improve the immune response to MCC. It is possible to have an in person or remote consultation with Dr. Standish by phone through 425-602-3311.
Dr. Daniel Rubin of Scottsdale, AZ, has cared for several of our MCC patients and employs an integrated approach to diet and nutritional supplementation that he has customized for neuroendocrine carcinomas such as MCC. We have no financial links with Dr. Rubin. He is able to do phone consultations for those who cannot travel to Arizona. His office phone is 480-990-1111.
Click here for more details.
Optimizing Fruit & Vegetable Intake
It is universally agreed that eating fresh fruits and vegetables and having a healthy diet is good for general health including immune system function. It is, however, often difficult to get enough fruits and vegetables in a standard diet. Several of our patients are attempting to optimize their fruit and vegetable intake through "juicing." Click here for a sample regimen from one of these patients.
Several of our patients take supplements to improve their nutritional status and augment the immune system. These include Host Defense® MyCommunity Capsules (available from Fungi Perfecti). Please note that we are not recommending or endorsing these products but are passing along information that our patients have used and liked. Please consult with your physician prior to beginning any nutritional supplementation program as some supplements can interact with certain medications.
"Anticancer: A New Way of Life" by David Servan-Schreiber MD, PhD
An inspirational and insightful book about general health & lifestyle management that can aid in fighting cancer. Visit the Anticancer Ways website for more information or Amazon or Barnes & Noble to purchase the book.
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Merkel cell carcinoma is optimally cared for by a team of doctors with expertise in dermatology, surgery, medical oncology, and radiation oncology. The risk of MCC recurrence and death is highest within the first 3 years from diagnosis. Most patients should have regular appointments for skin and lymph nodes examinations every 3-6 months for the first 3 years. CT scans aregenerally performed every three months for a few years after a high-risk diagnosis. Regular surveillance scans are important, as the immune-stimulating agents that are often used in cases of metastatic Merkel cell carcinoma are thought to work more effectively if the cancer is caught relatively early while the lesion is smaller. If MCC has not recurred or metastasized in the first three years, it may not be necessary to visit a doctor as often. However, you should contact your doctor immediately if you have any unusual lesions or symptoms.
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