Merkel Cell Carcinoma (MCC)

• Merkel cell carcinoma (MCC) is the eponym for :
  • Primary cutaneous neuroendocrine carcinoma:
    • A dermal neoplasm with cytoplasmic, dense-core neuroendocrine granules and keratin filaments. 
    • MCC is a rare primary cutaneous neuroendocrine malignancy
• The incidence of MCC in the United States has recently been reported to have increased by an estimated annual percentage change of 8% from 1986 to 2001:
  • Approximately 1500 new cases were predicted by the American Cancer Society in the United States alone in 2008. 
• MCC is one of the few cancers found to be:
  • Associated with immune suppression
  • Polyomavirus has been shown to be integrated within the genome
• Merkel cell carcinoma (MCC) is an uncommon and aggressive cutaneous neoplasm that lacks distinguishing clinical features. 
• More than half of Merkel cell carcinomas (MCCs) occur in the head and neck of elderly people in areas of actinically damaged skin:
  • The most common site of occurrence is the periorbital region. 
• Merkel cell carcinoma (MCC) has a propensity to recur and to cause:
  • Local (25% to 30% of the cases) and distant metastases (30% to 35% of the cases). 
• Distant metastases indicate a condition that is nearly always fatal.
• Merkel cell carcinoma (MCC) is a deadly disease with a poor likelihood for survival:
  • Local recurrence occurs in:
    • 44% of patients:
      • Multiple local recurrences occur in 15% of patients. 
• These tumors appear as:
  • Rapidly growing, painless nodules in elderly caucasian individuals or in young adults with ectodermal dysplasia syndromes. 
  • Merkel cell carcinomas (MCCs) usually appear as:
    • Indurated plaques or violaceous (red or deep purple) solitary and dome-shaped nodules. 
    • The surface is typically shiny, with telangiectasias and possibly ulceration. 
    • Most tumors measure 0.7 cm to 1.2 cm in diameter.
    • Merkel cell carcinomas (MCCs) usually occur in sun-damaged skin. 
    • They are often found near other lesions of actinically damaged skin, including skin involved with:
      
      • Bowen disease, squamous cell carcinoma, basal cell carcinoma, solar keratoses, or lentigo maligna. 

• The mean age at presentation is 68 years:
  • No gender bias is observed. 
• Merkel cell carcinoma (MCC) has also been linked to:
  • Previous radiation exposure and B-cell lymphoma.
• The nonspecific characteristics of Merkel cell carcinoma (MCC) lead to a lengthy differential diagnosis that includes:
  • Basal cell carcinoma
  • Squamous cell carcinoma
  • Keratoacanthoma
  • Amelanotic melanoma
  • Epidermal cysts
  • Lymphoma
  • Metastatic carcinoma of the skin
    • As a result:
      • Merkel cell carcinoma (MCC) is rarely diagnosed until biopsy is performed
  • Approximately 53% of Merkel cell carcinomas (MCCs) occur in the head and neck:

    • 35% occur in the extremities. 
    • In the head and neck:
      • 46% of tumors occur in the periorbital region
      • 29%, on the cheek
      • 18%, on the eyelid
      • 17%, on the forehead. 
      • Other sites in the head and neck include:
        • The lips (9%)
        • Ears (7%)
        • Nose and neck (5.4%)
        • Scalp (4%)
    • Tumors have also been reported in areas not exposed to sun, such as the:
      • Nasal cavity
      • Buccal mucosa
      • Gingiva
      • Hard palate
      • Postauricular skin

• Approximately 3% of patients with Merkel cell carcinoma (MCC) have tumors at several sites. 
• MCC is an aggressive tumor:
  • With an overall five-year survival of 40%
• Reported five year survival rates of local, nodal and metastatic disease are:
  • 64%, 39% and 18%, respectively
• Reported rates of regional lymph node involvement at the time of presentation vary from 10% to 45%:
  • Around 50% of patients with lymph node metastases harbor concurrent distant metastases:
    • Most often in the:
      • Liver, lung, brain, bone or skin
• MCC has high rates of local recurrence:
  • 25% to 30% of patients.
• MCC has high rates of distant metastasis:
  • 30% to 35% of patients.

 

Rodrigo Arrangoiz MS, MD, FACS a head and neck surgeon / endocrine surgeon / surgical oncologist and is a member of Sociedad Quirúrgica S.C at the America British Cowdray Medical Center in Mexico City:

• He is an expert in the management Merkel Cell Carcinoma.

Training:

• General surgery:

• Michigan State University:

• 2004 al 2010

• Surgical Oncology / Head and Neck Surgery / Endocrine Surgery:

• Fox Chase Cancer Center (Filadelfia):

• 2010 al 2012

• Masters in Science (Clinical research for health professionals):

• Drexel University (Filadelfia):

• 2010 al 2012

• Surgical Oncology / Head and Neck Surgery / Endocrine Surgery:

• IFHNOS / Memorial Sloan Kettering Cancer Center:

• 2014 al 2016

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