Epidemiology and Genetics (Including High Risk Patients, Polyomavirus, Prognostic Factors)
Merkel cell carcinoma (MCC) is a rare, highly aggressive form of skin cancer that has a strong propensity toward local recurrence, regional nodal metastasis, distant metastasis, and high mortality rates. MCC usually affects elderly white persons and has b
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Epidemiology and Genetics (Including High Risk Patients, Polyomavirus, Prognostic Factors) Garrett C. Lowe, Jerry D. Brewer, and Jeremy S. Bordeaux
Abbreviations CLL HIV LT MCC MCPyV NHL NMSC PUVA RB SEER SLNB
Chronic lymphocytic leukemia Human immunodeficiency virus Large T Merkel cell carcinoma Merkel cell polyomavirus Non-Hodgkin lymphoma Nonmelanoma skin cancer Psoralen-UV-A Retinoblastoma Surveillance, Epidemiology, and End Results Sentinel lymph node biopsy
Overview and Incidence Merkel cell carcinoma (MCC) is an uncommon, rapidly growing, and aggressive cutaneous malignancy of elderly persons, affecting mainly whites [1]. White-skinned individuals make up roughly 95 % of those affected; African Americans make G.C. Lowe (*) • J.D. Brewer Department of Dermatology, Mayo Clinic, 200 1st Street SW, 55905 Rochester, MN, USA e-mail: [email protected]; [email protected] J.S. Bordeaux Department of Dermatology, University Hospitals Case Medical Center, Case Western Reserve University, 11100 Euclid Avenue, Lakeside 3500, Cleveland, OH 44106-5000, USA e-mail: [email protected]
up less than 1 % [2, 3]. Local recurrences, nodal involvement, metastases, and high mortality rates are more the rule than the exception in MCC. The most common region of the body affected by MCC is the head and neck, with roughly 50 % of MCCs occurring in this area [2, 4–6] and nearly one of every ten MCCs occurring on the eyelid and periocular areas [7, 8] (see Fig. 2.1). The incidence of MCC in the head and neck region increases substantially after the age of 65 years, especially in men [9]. The next most common sites are the upper and lower extremities, which are the more common sites of MCC involvement in individuals younger than 65 years [2, 5]. Upper-extremity MCC seems to carry a better prognosis and occurs in roughly 20 % of cases [4]. The lower extremities are affected in one of seven patients with MCC and seem to be associated with increased recurrence [10]. The trunk comprises 11 % of MCC development. In addition to these common sites of involvement, 5 % of MCCs occur in non-sun-exposed mucosal areas, the most common of which is the larynx [2]. Other less common locations for MCC include the breasts, buttocks, esophagus, penis, salivary glands, scrotum, and vulva [4, 11–14]. The majority (80 %) of MCCs measure less than 2 cm in diameter at diagnosis and present with localized disease clinically (50–70 %) [2, 5, 15]. The rest have regional node involvement or distant metastases, or both, at diagnosis [1, 16, 17]. Even with aggressive excision of localized MCC, the incidence of recurrence and metastasis is high, and these traits ultimately lead to death in
M. Alam et al. (eds.), Merkel Cell Carcinoma, DOI 10.1007/978-1-4614-6608-6_2, © Springer Science+Business Media New York 2013
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Fig. 2.1 Pictorial breakdown of Merkel cell carcinoma (MCC) distribution in a sample population from Finland. Filled circles represent MCC positive for Merkel cell polyomavirus; open circles represent MCC
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