Supporting the

Neuroendocrine Cancer Community

Merkel Cell Carcinoma (MCC)

May 11, 2021

Merkel cell carcinoma (MCC) is a rare, highly aggressive skin cancer, primarily affecting older patients, and thought to arise from the cutaneous Merkel cell, a neuroendocrine cell. 

The exact cause of Merkel cell carcinoma (MCC) is not known.

Factors strongly associated with the development of Merkel cell carcinoma (MCC) are:

  • Age over 50 years
  • Fair skin
  • History of extensive UV exposure
  • Chronic immunosuppression (eg kidney or heart transplantation, HIV)
  • Merkel cell polyomavirus – a virus discovered to live harmlessly on the skin in most healthy people. However in some people the virus may get trapped in the cell by mistake, which can lead to cancer development. The virus within the cell cannot spread – Merkel Cell Carcinoma (MCC) is not contagious.

The most common sites are the head and neck followed by the arms – but they can also be found on the shoulders, torso, legs and areas of the body not usually exposed to sunlight.

They start as painless, firm, blue-red/ violet or even skin-coloured nodules – less than a centimetre, but may grow rapidly – rarely they may also ulcerate.

Early detection and intervention is essential – Merkel cell cancers can spread  – to surrounding skin, to nearby lymph nodes in the neck, armpits or groins or to other internal organs.

There is a very good chance of curing people with a small primary Merkel cell cancer.

There is a reasonable chance of curing people with bigger primary Merkel cell cancers or Merkel cell cancers that have spread to lymph nodes.

Surgery is the most common treatment used for the primary Merkel cell cancer

Stage IV Merkel cell cancer may not be currently curable, but it can be controlled.

NICE’s recent approval of Avelumab as a first line option of therapy for metastatic MCC is a very positive step in improving overall survival and, as it appears to be well tolerated, quality of life.

Further information on Merkel Cell Carcinoma (MCC) can be found on our website and information about NICE’s recent approval of Avelumab can be found in our News Section or on the NICE website here.

D’Angelo et al (2020) JAVELIN trial Journal for ImmunoTherapy of Cancer (NCT 02155647)

Avelumab showed continued durable responses and meaningful long-term survival outcomes in patients with mMCC

Heath et al (2008) Clinical characteristics of Merkel cell carcinoma- the AEIOU features.

– Asymptomatic, Expanding rapidly, Immune suppression, Older than 50 & UV-exposed skin.

Lebbe C. et al. Merkel Cell Carcinoma. European consensus-based interdisciplinary guideline, Eur J Cancer (2015)

NICE recommends Avelumab as 1st line option for treating metastatic Merkel Cell Carcinoma in adults [TA691] April 2021 https://www.nice.org.uk/guidance/TA691

Primary Care Dermatology Society MCC resource page:  http://www.pcds.org.uk/clinical-guidance/merkel-cell-tumour

Vilani et al Dermatol Ther (Heidelb) (2019) an overview of the different possibilities for the treatment of Merkel Cell Carcinoma

Walker, Lebbé et al (2020) Efficacy and safety of avelumab treatment in patients with metastatic Merkel cell carcinoma: experience from a global expanded access program JITC