How Aggressive Is Merkel Cell Carcinoma

The Immune System And Merkel Cell Carcinoma

It has been long suspected that immune dysregulation plays a role in the development of MCC. Clinically, it was observed that chronically immunosuppressed patients, such as organ transplant recipients or those with HIV or B-cell malignancies, were at increased risk for developing MCC . Early histological reports of primary MCC tumors demonstrated lymphocytic infiltration, evidence of MCCâs immunogenic biology . More recently, tumor-infiltrating lymphocytes were found to correlate with a better prognosis, a finding which has been confirmed by genomic analysis of primary MCC tumors . Of note, patients with an unknown primary lesion have a better prognosis than those with a known primary lesion, suggesting that an immune-based response at the primary site leads to improved immunological tumor control overall .

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

Cancer May Spread From Where It Began To Other Parts Of The Body

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if Merkel cell carcinoma spreads to the liver, the cancer cells in the liver are actually cancerous Merkel cells. The disease is metastatic Merkel cell carcinoma, not liver cancer.

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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.

Merkel Cell Carcinoma: An Update And Immunotherapy

• Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan

Merkel cell carcinoma is a rare but aggressive skin cancer with frequent metastasis and death. MCC has a mortality rate of 30%, making it more lethal than malignant melanoma, and incidence of MCC has increased almost fourfold over the past 20 years in the USA. MCC has long been considered to be an immunogenic cancer because it occurs more frequently in immunosuppressed patients from organ transplant and HIV infection than in those with immunocompetent. Chronic UV light exposure and clonal integration of Merkel cell polyomavirus are two major causative factors of MCC. Approximately 80% of MCC are associated with MCPyV, and T cells specific for MCPyV oncoproteins are present in the blood and tumors of patients. Several studies have shown that a subset of MCCs express PD-1 on tumor-infiltrating lymphocytes and express PD-L1 on tumor cells, which suggests an endogenous tumor-reactive immune response that might be unleashed by anti-PD-1 or anti-PD-L1 drugs.

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Biology And Pattern Of Spread

MCC can exhibit an aggressive course characterized by a propensity for local recurrence even after wide surgical excision and a high incidence of nodal involvement with hematogenous dissemination a frequent site of relapse and death. Most series document a cancer-specific death rate of around 30%.30 Patients may also present with metastatic nodal disease without an obvious index lesion. Such patients make up 10% to 20% of many series and have a better prognosis compared to patients with concomitant nodes and an index lesion.31 MCC is a highly immunogenic carcinoma, and patients who are immunosuppressed and patients diagnosed with non-Hodgkin’s lymphoma and chronic lymphocytic leukemia are at higher risk of developing MCC and having a poor outcome.32 The role that the MCV plays in the pathogenesis of MCC may account for its higher incidence inpatients who are immunosuppressed.

Yaohui G. Xu, … Gary S. Wood, in, 2020

A Case Report Of Leptomeningeal Carcinomatosis Secondary To Recurrent Merkel Cell Carcinoma After Avelumab

Pedro Mendoza; Kathy Lin Chuang, MD

Perm J 2021;25:20.235

E-pub: 03/25/2021

ABSTRACT

Introduction: Merkel cell carcinoma is a rare and aggressive neuroendocrine cancer with a high mortality rate of 33% to 46%. Merkel cell is a type of epidermis cell receptor responsible for contact sensitivity and is known to have neuroendocrine properties. Treatment of Merkel cell carcinoma with avelumab has been prominsing, but its rarity and poor prognosis necessitates close follow up.

Case Presentation: A 71-year-old woman presented with a left forearm mass that was initially suspected to be a sebaceous cyst. After surgical excision and biopsy, she was diagnosed with Merkel cell carcinoma. The patient underwent avelumab treatment for 2 years, with remission of cancer for 24 months. A positron emission tomographic scan at 24 months of treatment noted uptake in the left axilla and portocaval regions. Despite receiving different combinations of immunotherapy, chemotherapy, and radiation, the patients cancer metastasized to the leptomeninges. She was transitioned to hospice and passed away 3 months after diagnosis of leptomeningeal carcinoma.

This case highlights the efficacy of avelumab in keeping patients in remission, which can offer increased quality of life. However, it also highlights the aggressive nature of Merkel cell carcinoma and the importance of surveillance for early detection of recurrence.

INTRODUCTION

CASE PRESENTATION

Figure 1. Left distal humerus mass .

DISCUSSION

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What Is Merkel Cellcarcinoma Of The Skin

Merkel cell carcinoma of the skin is a rare form of skin cancer. It may be very aggressive and often metastasises to other parts of the body. It has also been called Toker tumour, cutaneousneuroendocrine carcinoma, trabecular cell carcinoma, and primary small-cell carcinoma of the skin.

Merkel cell carcinoma

Management Of Merkel Cell Carcinoma

For patients with operable disease, most agree that surgery is the treatment of choice. As with most tumors, treatment is based on the stage.

Guidelines released in 2015 by a collaborative group of multidisciplinary experts from the European Dermatology Forum , the European Association of Dermato-Oncology , and the European Organization of Research and Treatment of Cancer included the following with regard to the management of Merkel cell carcinoma :

• The primary tumor should be excised with 1-2 cm margins
• In patients with regional lymph node involvement, radical lymphadenectomy is recommended
• Adjuvant radiation therapy may be considered in patients with multiple affected lymph nodes of extracapsular extension
• In unresectable metastatic Merkel cell carcinoma, monochemotherapy or polychemotherapy achieve high remission rates; however, responses are usually short-lived
• Treatment within clinical trials is regarded as a standard of care in disseminated Merkel cell carcinoma

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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.

Patients Can Enter Clinical Trials Before During Or After Starting Their Cancer Treatment

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCIs clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

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Merkel Cell Carcinoma Is A Rare Aggressive Skin Cancer

Sometimes referred to as a neuroendocrine carcinoma of the skin, MCC arises from uncontrolled growth of cells in the skin that share some characteristics with normal Merkel cells in the skin. MCC primarily occurs on sun-exposed skin such as the head/neck and arms, but it can occur anywhere on the body, including sun-protected areas. MCC usually develops as a painless, firm bump that can be red-purple or skin-colored. Patients frequently point out a new MCC to their doctor because a bump is growing rapidly and/or does not look like anything the patient has ever had before.

Learn more about Merkel cells, MCC, its symptoms and appearances, and when a bump may be serious enough to see a specialist.

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How Is Merkel Cell Carcinoma Diagnosed

Merkel cell carcinoma should be considered;in any tumour with “AEIOU” clinical features, which are present in about 90% of patients with the disease.

• Asymptomatic or non-tender
• Older than 50
• UV-exposed fair skin;

The main test is a;biopsy of the tumour. This shows characteristic Merkel cell carcinoma pathology. Immunohistochemistry can be helpful as cytokeratin-20 is positive in up to 95% of tumours and thyroid transcription factor is usually negative.

A general examination, including evaluation of local lymph nodes, and staging investigations may be arranged to determine whether the tumour has spread to other sites.

Staging investigations may include:

• Lymph nodeultrasound scan
• Imaging using X-rays, computed tomography , magnetic resonance imaging and positron emission tomography scans.

A specific Merkel cell carcinoma staging system is published by the American Joint Committee on Cancer.

Longterm survival is likely if the lymph nodes do not contain tumour cells.

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Who Gets Merkel Cell Carcinoma

Merkel cell carcinoma has an estimated incidence of 0.23 per 100,000 people in Caucasian populations, which is much less common than melanoma.

• Increasing numbers of Merkel cell carcinomas have been reported by some centres in recent years.
• Merkel cell carcinoma mainly affects older people, with most cases occurring after the age of 50.
• It is slightly more common in men.
• It occurs on parts of the body commonly exposed to sunlight, most often the head and neck.
• It is also more common and more serious in those that are immune suppressed, such as patients with solidorgan transplants, human immunodeficiency virus infection, haematologicalmalignancy or on drugs such as azathioprine.

Merkel Cell Carcinoma Treatment

Treatment will usually include surgery to remove the merkel cell tumor along with a biopsy of a lymph node to see if the cancer has spread. Radiologic scans may also be recommended prior to surgery. The surgery can almost always be performed on an outpatient basis. Radiation therapy to the skin and/or lymph nodes may also be indicated for patients who have high-risk lesions. Patients whose merkel cell cancer has spread beyond their lymph nodes into their organs are likely to require other medical treatments.

For Merkel Cell Carcinoma that has spread, but is still confined within the leg or arm, we may recommend local immunotherapies or isolated regional perfusion or infusion.

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Merkel Cell Carcinoma: An Aggressive Skin Neoplasm

Division of Head and Neck Surgery , University of California at Los Angeles Medical Center; the Department of Radiation Oncology , Kaiser Permanente Medical Group, Los Angeles; and the Johnson Comprehensive Cancer Center , University of California at Los Angeles School of Medicine

Division of Head and Neck Surgery , University of California at Los Angeles Medical Center; the Department of Radiation Oncology , Kaiser Permanente Medical Group, Los Angeles; and the Johnson Comprehensive Cancer Center , University of California at Los Angeles School of Medicine

Division of Head and Neck Surgery , University of California at Los Angeles Medical Center; the Department of Radiation Oncology , Kaiser Permanente Medical Group, Los Angeles; and the Johnson Comprehensive Cancer Center , University of California at Los Angeles School of Medicine

Merkel Cell Carcinoma Usually Appears As A Single Painless Lump On Sun

This and other changes in the skin may be caused by Merkel cell carcinoma or by other conditions. Check with your doctor if you see changes in your skin.

Merkel cell carcinoma usually appears on sun-exposed skin as a single lump that is:

• Fast-growing.
• Firm and dome-shaped or raised.
• Red or violet in color.

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Life And Death Decisions

With neither avelumab;nor pembrolizumab approved for Merkel cell carcinoma in Canada, and running out of time to get the drug on compassionate grounds; which his;oncologist, , was pursuing;;Ewart decided to pay for pembrolizumab himself.

In the U.S., pembrolizumab costs upwards of about $6,700 US per dose, according to comparison shopping website;GoodRx.com. It is administered by injection approximately every three weeks; a full course of treatment generally lasts for about one year.

Ewart had his first injection;in late;April, at the private Bayshore Clinic north of Toronto. The;price tag:;$7,700.;

A few weeks later, Ewart and his family went to see his oncologist. According to son John, Butler walked in with a smile on his face. He saw that Fred’s skin had;improved and that he was in;better shape;;he had also already seen bloodwork results.

“He looked at my dad and gave him a big hug, and Dr. Butler started to cry, and of course we all started to cry, and Butler said, ‘It’s working, it’s working.'”

What Does Mcc Look Like

MCC tumors often, but not always, appear on sun-exposed areas of the body. The tumors are not nearly as distinctive as other skin cancers and can appear as a pearly pimple-like lump, sometimes skin-colored, red, purple or bluish-red, though they are rarely tender to the touch. The rapid speed at which they grow is what often causes patients and doctors to take notice.

A recurrence of Merkel cell carcinoma on the forehead.

Merkel cell carcinoma on the lower leg.

Please note: Since not all Merkel cell carcinomas have the same appearance, these photos serve as a general reference for what MCC can look like. If you see something new, changing or unusual on your skin, schedule an appointment with your doctor. Photos courtesy of Paul Nghiem, MD, PhD

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Diagnosing And Treating Mcc

One of the issues faced by healthcare providers is that an MCC lesion may look like a cyst or swelling from an inflamed hair follicle. In a clinical study, 56% of Merkel cell carcinomas were initially thought to be benign by physicians. If you notice any new or changing lesions on your skinno matter how minortalk with your healthcare provider. It is important not to delay time to detection.

If your healthcare provider suspects that a lesion on your skin may be MCC, he or she will likely take a careful medical history and perform a physical exam. If your healthcare provider thinks you may have MCC, he or she will do a skin biopsy. That means removing the lesion and sending it to a lab where it will be viewed under a microscope to examine and confirm whether there are any cancerous cells.

Treatment of MCC is based on the stage of the disease and the overall health of the patient. The main treatments are surgery , radiation, and chemotherapy and other systemic therapies .

Research is underway to better understand this disease and to discover treatments. For more information about available clinical trials for Merkel cell carcinoma, go to;clinicaltrials.gov or;Pfizer: Find a Trial.

Learn more about sun safety

Exposure to the sun is one risk factor for MCC that we can control. We all need some sun exposure, and its important to engage in some physical activity outdoors, but too much sun can be harmful. Find out what you can do to stay healthy in the sun.

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Clinical Manifestations Patient Evaluation Staging

MCC often appears as a painless, indurated, erythematous to violaceous nodule with a smooth, shiny surface on sun-damaged skin, and, less commonly, as a plaque with satellite metastases. Surface ulcerations are rare.236238,246,248,287 Heath and colleagues summarized clinical features of MCC in an acronym: AEIOU ,254 suggesting that the clinical appearance of MCC is rather nondescript. MCC occurs predominantly in the head and neck , followed by the extremities and trunk .287 Ten percent of these tumors are in the periocular areas .288 MCC also has been reported in extracutaneous sites, such as the vulva, endocervix, penis, esophagus, bladder, and calvaria.259,289292 The salivary glands, nasal cavity, lip, lymph nodes, vulva, vagina, and esophagus are the most common extracutaneous sites.243 The clinical differential diagnosis includes BCC, SCC, amelanotic melanoma, lymphoma, and metastatic disease. Histologic and immunohistochemical studies are the key to confirmation of the diagnosis.

Diana Bell, in, 2021

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What It Means For You

After testing is finished, your medical team will try to pinpoint how far the Merkel cell carcinoma has progressed. This process is known as staging.

Staging is a way to understand how much the cancer has grown and how far it has spread. The process also;helps doctors determine how to best treat MCC, and the risk of it coming back.

Cancer staging can be complex and confusing. If you have been diagnosed with MCC, ask your doctor to explain your stage clearly, in a way that you can understand.