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What is Merkel cell carcinoma?

Merkel cell carcinoma, also called neuroendocrine cancer of the skin, is a rare type of disease in which malignant (cancer) cells are found on or just beneath the skin and in hair follicles. Merkel cell carcinoma usually appears as firm, painless, shiny lumps of skin. These lumps or tumors … Merkel cell carcinoma (MCC) is an aggressive tumor, the incidence of which is seemingly increased in immunocompromised patients. Merkel cell carcinoma is an uncommon aggressive primary cutaneous neuroendocrine carcinoma. Histologically, the differential diagnosis includes the ‘small round cell’ tumor group, particularly metastatic small cell carcinoma and blastic hematological malignancies involving skin/soft tissues….

What are the causes of Merkel cell carcinoma?

The exact causes of Merkel cell carcinoma are not known. Factors strongly associated with the development of Merkel cell carcinoma are:

The best available information on characteristics of patients with Merkel cell carcinoma comes from a study of 1,034 patients summarized in the table below (Agelli, 2003). The average (median) age of the patients with Merkel cell carcinoma was 74 years. The most common sites of involvement were head followed by arms (upper limb). At the time of diagnosis, half of the patients had disease localized to the skin, while the other half had Merkel cell carcinoma that was no longer confined to the skin.

Treatment options for Merkel cell carcinoma

Treatment is generally based on the stage of the disease. There are four major treatments for Merkel cell carcinoma: 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.

Wide surgical excision

The goal of wide excision is to control local recurrence and lymph node metastases. Merkel cell carcinoma 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{abffe3e1318a9624791ae4064333f2f75374704dc13d09152fd440b16c9e1c0c} 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.

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 Merkel cell carcinoma 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 Merkel cell carcinoma is evaluated under the microscope during surgery. Our analysis of published studies on the treatment of Merkel cell carcinoma 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).

Radiation therapy

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).

Follow-up care

Merkel cell carcinoma is optimally cared for by a team of doctors from dermatology, surgery, medical oncology, and radiation oncology. Most recurrences of Merkel cell carcinoma and most deaths from this disease occur within the first 3 years. Patients should have regular appointments for skin and lymph nodes examinations every 3-6 months for the first 3 years. CT scans are sometimes performed every 6 months for a few years after a high-risk diagnosis. Unfortunately, by the time Merkel cell carcinoma is visible on a CT scan; curative treatment is no longer possible. Therefore, scans are not routinely recommended. If Merkel cell carcinoma 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.

Why India?

Treatment of Merkel cell carcinoma in India is provided at hospitals of Mumbai and Chennai with comprehensive surgical, medical and emergency services supported by state-of-the-art diagnostic and treatment facilities and innovative surgery techniques. Medical tourism in India is geared to expose clients to the best quality private and public healthcare in India, with a personalized flair, whilst facilitating treatment by highly qualified specialists at affordable and competitive prices.