Merkel Cell Carcinoma: Difference between revisions

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== Introduction  ==
== Introduction  ==


Merkel Cell Carcinoma (MCC) is a rare and aggressive skin cancer typically seen on the facial skin and extremities of elderly Caucasians and immunocompromised people. This neuroendocrine carcinoma accounts for less than 1% of all cutaneous malignancies and is associated with UV exposure and infection with the Merkel cell polyomavirus (MCPyV).<ref>Gauci ML, Aristei C, Becker JC, Blom A, Bataille V, Dreno B, Del Marmol V, Forsea AM, Fargnoli MC, Grob JJ, Gomes F. [https://www.sciencedirect.com/science/article/pii/S0959804922002532 Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline–Update 2022.] European Journal of Cancer. 2022 Aug 1;171:203-31.</ref><ref>Zwijnenburg EM, Lubeek SF, Werner JE, Amir AL, Weijs WL, Takes RP, Pegge SA, van Herpen CM, Adema GJ, Kaanders JH. [https://www.mdpi.com/2072-6694/13/7/1614 Merkel cell carcinoma: New trends]. Cancers. 2021 Mar 31;13(7):1614.</ref><ref>Walsh NM, Cerroni L. [https://onlinelibrary.wiley.com/doi/epdf/10.1111/cup.13910 Merkel cell carcinoma: A review.] Journal of Cutaneous Pathology. 2021 Mar;48(3):411-21.</ref>
Merkel Cell Carcinoma (MCC) is a rare and aggressive skin cancer typically seen on the facial skin and extremities of elderly Caucasians and immunocompromised people. This neuroendocrine carcinoma accounts for less than 1% of all cutaneous malignancies and is associated with UV exposure and infection with the Merkel cell polyomavirus (MCPyV).<ref name=":0">Gauci ML, Aristei C, Becker JC, Blom A, Bataille V, Dreno B, Del Marmol V, Forsea AM, Fargnoli MC, Grob JJ, Gomes F. [https://www.sciencedirect.com/science/article/pii/S0959804922002532 Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline–Update 2022.] European Journal of Cancer. 2022 Aug 1;171:203-31.</ref><ref>Zwijnenburg EM, Lubeek SF, Werner JE, Amir AL, Weijs WL, Takes RP, Pegge SA, van Herpen CM, Adema GJ, Kaanders JH. [https://www.mdpi.com/2072-6694/13/7/1614 Merkel cell carcinoma: New trends]. Cancers. 2021 Mar 31;13(7):1614.</ref><ref name=":1">Walsh NM, Cerroni L. [https://onlinelibrary.wiley.com/doi/epdf/10.1111/cup.13910 Merkel cell carcinoma: A review.] Journal of Cutaneous Pathology. 2021 Mar;48(3):411-21.</ref>


== Epidemiology ==
== Epidemiology ==
The worldwide incidence of MCC varies from 0.1 to 1.6 cases per 100,000 people annually with Australia recording the highest rate ranging from 0.82 to 2.5 <ref name=":0" /><ref name=":1" /><ref>Garbutcheon-Singh K, Curchin D, McCormack C, Smith S. Trends in the incidence of merkel cell carcinoma in Victoria between 1986 and 2016. InAUSTRALASIAN JOURNAL OF DERMATOLOGY 2019 May 1 (Vol. 60, pp. 40-40). 111 RIVER ST, HOBOKEN 07030-5774, NJ USA: WILEY.</ref>. It is then followed by New Zealand (0.88–0.96)<ref>Lee Y, Chao P, Coomarasamy C, Mathy JA. Epidemiology and survival of Merkel cell carcinoma in New Zealand: a population‐based study between 2000 and 2015 with international comparison. Australasian Journal of Dermatology. 2019 Nov;60(4):e284-91.</ref><ref>Robertson JP, Liang ES, Martin RC. Epidemiology of Merkel cell carcinoma in New Zealand: a population‐based study. British Journal of Dermatology. 2015 Sep 1;173(3):835-7.</ref> and United States (0.66–0.79)<ref name=":0" /><ref>Fitzgerald TL, Dennis S, Kachare SD, Vohra NA, Wong JH, Zervos EE. Dramatic increase in the incidence and mortality from Merkel cell carcinoma in the United States. The American surgeon. 2015 Aug;81(8):802-6.</ref><ref>Paulson KG, Park SY, Vandeven NA, Lachance K, Thomas H, Chapuis AG, Harms KL, Thompson JA, Bhatia S, Stang A, Nghiem P. Merkel cell carcinoma: Current US incidence and projected increases based on changing demographics. Journal of the American Academy of Dermatology. 2018 Mar 1;78(3):457-63.</ref>. European countries such as France, Spain and Scotland report relatively similar incidence rates of 0.25 to 0.35 while Scandinavian countries have less incidence rates: 0.19 in Sweden and 0.12 in Finland. <ref name=":0" />


== Aetiology ==
=== Risk factors of MCC ===
Predisposing factors for MCC are as follows:<ref name=":0" />
 
* Old age. The median age at diagnosis is documented to be 77 years with the highest incidence observed among people over 85 years old.
* UV exposure
* White skin type
* Male sex. The male-to-female ratio of MCC is over 2.5 incidence is over 2.5 in all reported studies.
* Immunosuppression. Approximately 6–12% of all patients with MCC are immunosuppressed.


== Mechanism of Injury / Pathological Process  ==
== Mechanism of Injury / Pathological Process  ==

Revision as of 15:51, 29 December 2023

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Introduction[edit | edit source]

Merkel Cell Carcinoma (MCC) is a rare and aggressive skin cancer typically seen on the facial skin and extremities of elderly Caucasians and immunocompromised people. This neuroendocrine carcinoma accounts for less than 1% of all cutaneous malignancies and is associated with UV exposure and infection with the Merkel cell polyomavirus (MCPyV).[1][2][3]

Epidemiology[edit | edit source]

The worldwide incidence of MCC varies from 0.1 to 1.6 cases per 100,000 people annually with Australia recording the highest rate ranging from 0.82 to 2.5 [1][3][4]. It is then followed by New Zealand (0.88–0.96)[5][6] and United States (0.66–0.79)[1][7][8]. European countries such as France, Spain and Scotland report relatively similar incidence rates of 0.25 to 0.35 while Scandinavian countries have less incidence rates: 0.19 in Sweden and 0.12 in Finland. [1]

Risk factors of MCC[edit | edit source]

Predisposing factors for MCC are as follows:[1]

  • Old age. The median age at diagnosis is documented to be 77 years with the highest incidence observed among people over 85 years old.
  • UV exposure
  • White skin type
  • Male sex. The male-to-female ratio of MCC is over 2.5 incidence is over 2.5 in all reported studies.
  • Immunosuppression. Approximately 6–12% of all patients with MCC are immunosuppressed.

Mechanism of Injury / Pathological Process[edit | edit source]

add text here relating to the mechanism of injury and/or pathology of the condition

Clinical Presentation[edit | edit source]

add text here relating to the clinical presentation of the condition

Diagnostic Procedures[edit | edit source]

add text here relating to diagnostic tests for the condition

Outcome Measures[edit | edit source]

add links to outcome measures here (see Outcome Measures Database)

Management / Interventions
[edit | edit source]

add text here relating to management approaches to the condition

Differential Diagnosis
[edit | edit source]

add text here relating to the differential diagnosis of this condition

Resources
[edit | edit source]

add appropriate resources here

References[edit | edit source]

  1. 1.0 1.1 1.2 1.3 1.4 Gauci ML, Aristei C, Becker JC, Blom A, Bataille V, Dreno B, Del Marmol V, Forsea AM, Fargnoli MC, Grob JJ, Gomes F. Diagnosis and treatment of Merkel cell carcinoma: European consensus-based interdisciplinary guideline–Update 2022. European Journal of Cancer. 2022 Aug 1;171:203-31.
  2. Zwijnenburg EM, Lubeek SF, Werner JE, Amir AL, Weijs WL, Takes RP, Pegge SA, van Herpen CM, Adema GJ, Kaanders JH. Merkel cell carcinoma: New trends. Cancers. 2021 Mar 31;13(7):1614.
  3. 3.0 3.1 Walsh NM, Cerroni L. Merkel cell carcinoma: A review. Journal of Cutaneous Pathology. 2021 Mar;48(3):411-21.
  4. Garbutcheon-Singh K, Curchin D, McCormack C, Smith S. Trends in the incidence of merkel cell carcinoma in Victoria between 1986 and 2016. InAUSTRALASIAN JOURNAL OF DERMATOLOGY 2019 May 1 (Vol. 60, pp. 40-40). 111 RIVER ST, HOBOKEN 07030-5774, NJ USA: WILEY.
  5. Lee Y, Chao P, Coomarasamy C, Mathy JA. Epidemiology and survival of Merkel cell carcinoma in New Zealand: a population‐based study between 2000 and 2015 with international comparison. Australasian Journal of Dermatology. 2019 Nov;60(4):e284-91.
  6. Robertson JP, Liang ES, Martin RC. Epidemiology of Merkel cell carcinoma in New Zealand: a population‐based study. British Journal of Dermatology. 2015 Sep 1;173(3):835-7.
  7. Fitzgerald TL, Dennis S, Kachare SD, Vohra NA, Wong JH, Zervos EE. Dramatic increase in the incidence and mortality from Merkel cell carcinoma in the United States. The American surgeon. 2015 Aug;81(8):802-6.
  8. Paulson KG, Park SY, Vandeven NA, Lachance K, Thomas H, Chapuis AG, Harms KL, Thompson JA, Bhatia S, Stang A, Nghiem P. Merkel cell carcinoma: Current US incidence and projected increases based on changing demographics. Journal of the American Academy of Dermatology. 2018 Mar 1;78(3):457-63.