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Merkel Cell Carcinoma

Definition


Disease: Merkel Cell Carcinoma Merkel Cell Carcinoma
Category: Dermatological diseases
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Disease Definition:

There is a rare type of skin cancer that appears as a flesh-colored or bluish-red nodule, often on the face, head or neck, and it's called Merkel cell carcinoma or neuroendocrine carcinoma. The incidence of Merkel cell carcinoma is increasing worldwide, just like other types of skin cancers.

Older people develop this type of cancer more commonly. The risk of developing Merkel cell carcinoma may increase due to long-term of sun exposure or having a weak immune system.

It's critical to detect and treat Merkel cell carcinoma early because it usually grows fast and spreads quickly (metastasizes) to other parts of the body. Whether the cancer has spread beyond the skin by the time it is diagnosed is the thing that the patient’s treatment plan will depend on.

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

A fast-growing, painless nodule (tumor) on the skin is usually the first sign of Merkel cell carcinoma. The shiny nodule may appear in shades of purple, blue or red or it may be skin colored. Merkel cell carcinoma can develop anywhere on the body, even on areas not exposed to sunlight; however, nearly half of Merkel cell carcinoma appear on the face, neck or head.

The patient may experience swollen lymph nodes, pain or fatigue if the skin cancer has spread to other parts of the body.

A person should talk to a doctor when noticing a mole, freckle or bump that is changing in color, size or shape, growing rapidly or bleeding easily after minor trauma such as washing the skin or shaving. It's important to get checked out when seeing any changes, despite the fact that most skin lesions never become cancer.

Causes:

Merkel cell may be associated with the sense of touch. Merkel cell carcinoma is named after the Merkel cell, which is found at the base of the epidermis (the outermost layer of the skin).

What exactly causes Merkel cell carcinoma to develop is not certain. One of the theories says that Merkel cell carcinoma may arise from the Merkel cell itself. Yet another theory says that certain immature cells (skin stem cells) become cancerous and develop features that make them look like Merkel cells.

A number of genetic abnormalities are detected in Merkel cell carcinoma cells. However, how these genetic changes affect cancer development is not known.

The risk of developing this cancer may increase due to some factors, though there's no definite cause of Merkel cell carcinoma:

A weakened immune system:
Merkel cell carcinoma is more likely to develop when someone has a weakened immune system, such as when someone takes drugs to suppress the immune response or has HIV infection.

Race:
People who have light-colored skin are more likely to get Merkel cell carcinoma.

Artificial or natural sunlight:
Tanning beds and such artificial sunlight and even natural sunlight increase the risk of Merkel cell carcinoma, especially when the exposure to these things is frequent.

Age:
Merkel cell carcinoma is more likely to develop in older people like those older than the age of 70. So people who develop this type of cancer when they're younger have weakened immune systems.

History of other skin cancers:
Merkel cell carcinoma is associated with the development of other skin cancers such as squamous cell or basal cell carcinoma.

Complications

Complications:

The complications of Merkel cell carcinoma are not uncommon and this condition is an aggressive one; some of the complications that it may cause are:

Treatment side effects:
Chemotherapy and radiation therapy can kill healthy cells and cause complications although they may be effective in halting the growth of cancer.

Metastasis:
Up to half the people with Merkel cell carcinoma eventually develop cancer that spreads beyond the skin, even with treatment. First, Merkel cell carcinoma tends to travel to nearby lymph nodes and then may spread to the lungs, bone, liver or brain, where it can interfere with the functioning of these organs. The cancer that has metastasized is more difficult to treat and can be fatal. 

Treatments:

Whether the cancer has spread beyond the skin by the time it is diagnosed is the thing that specifies the type of treatment the patient will receive. The patient may have just one type of treatment or a combination of treatments:

Chemotherapy:
To stop cancer cells from dividing or to kill them, chemotherapy uses drugs. Chemotherapy drugs may be injected, swallowed or applied to the skin, depending on the type of drug.

When Merkel cell carcinoma has spread to other organs in the body or when it has recurred, chemotherapy is generally used. However, it is not clear whether chemotherapy improves the survival rate in people with Merkel cell carcinoma. In any case, the patient should talk with the doctor about the benefits and risks of using chemotherapy.

The side effects that chemotherapy often causes are significant; however most of these side effects are temporary. An increased risk of infection, hair loss, fatigue, nausea, vomiting and diarrhea are included in the common side effects. Permanent damage to the body organs may occur in some rare cases.

The patient will undergo physical exams including blood tests during the course of treatment to monitor how he/she is responding to chemotherapy or radiation therapy. The patient will continue having frequent checkups with the doctor to watch for recurrences afterwards.

Depending on a number of factors, the outlook after treatment for Merkel cell carcinoma varies. Whether the patient has other health conditions, the tumor size and location, the age of the patient, and the stage of the tumor at diagnosis are all included in these factors. In general, the best prognoses may be obtained by otherwise healthy people with small tumors and no evidence of cancer spreading beyond the skin.


Surgery:
During surgery, the tumor is removed along with a border of normal skin surrounding the tumor. Any remaining cancer cells may be caught by removing a border of normal skin. The lymph nodes in the area of the skin tumor are removed (lymph node dissection) if there's evidence that the cancer has spread to those lymph nodes.

A procedure called Mohs micrographic surgery is occasionally used. The doctor creates "slices" of the tumor tissue and examines each slice under a microscope after removing the visible tumor. Until cancer cells are no longer visible in the tissue slice, the process is repeated. Less normal tissue is taken out in this type of surgery, thus reducing scarring; however it ensures a tumor-free border of skin. In case the skin cancer is on the face, Mohs surgery is used.

Radiation Therapy:
Directing high-energy X-rays at cancer cells are involved in radiation therapy. This radiation damages the genetic material in growing cells. Because they divide more rapidly than normal cells do, cancer cells are particularly susceptible to the effects of radiation.

To kill any cancer cells that remain after the tumor is removed, radiation therapy is usually given in cases of Merkel cell carcinoma. There is no established consensus about who should receive radiation after surgery completely removes a tumor, because this type of cancer is so rare. The patient should talk to the doctor about the pros and cons of radiation after surgery in his/her particular situation.
Radiation may be used as the sole treatment in people who choose not to undergo surgery or to shrink Merkel cell carcinoma before it's removed. However, if the scalp needs to be radiated, general side effects from radiation therapy include fatigue, hair loss and red, irritated skin. The patient may notice that he/she has a dry mouth, difficulty swallowing and other symptoms if the radiation therapy that is received is to the head or neck.

Prognosis:

Not Available

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