A review of the epidemiology and treatment of Merkel cell carcinoma
Identifieur interne : 003D60 ( Pmc/Curation ); précédent : 003D59; suivant : 003D61A review of the epidemiology and treatment of Merkel cell carcinoma
Auteurs : João Pedreira Duprat [Brésil] ; Gilles Landman [Brésil] ; João Victor Salvajoli [Brésil] ; Eduard Rene Brechtbühl [Brésil]Source :
- Clinics [ 1807-5932 ] ; 2011.
Abstract
Merkel cell carcinoma is a very rare and aggressive neoplasm. Due to its rarity, therapeutic guidelines are not well established, especially for regionally advanced disease. Articles in English, French, Italian, Portuguese, and Spanish from the last 20 years were identified in MEDLINE and reviewed. The key word “Merkel” was used for the search, relevant articles were selected, and their references were examined. The most important articles related to epidemiology, genesis and treatment were reviewed. The incidence of Merkel cell carcinoma is increasing due to the advancing age of the population, higher rates of sun exposure and an increasing number of immunocompromised individuals. With regard to etiology, the recently described Merkel Cell polyomavirus is thought to play a role. Either local or regional surgical intervention remains the standard of care, but adjuvant radiotherapy or radiotherapy as a primary treatment have been discussed as reasonable therapeutic options. An update on this rare neoplasia is essential because of its increasing incidence and changing treatment options.
Url:
DOI: 10.1590/S1807-59322011001000023
PubMed: 22012057
PubMed Central: 3180159
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cell carcinoma</title><author><name sortKey="Duprat, Joao Pedreira" sort="Duprat, Joao Pedreira" uniqKey="Duprat J" first="João Pedreira" last="Duprat">João Pedreira Duprat</name><affiliation wicri:level="1"><nlm:aff id="af1-cln_66p1817">Hospital do Câncer AC Camargo, Department of Skin Câncer, São Paulo/SP, Brazil.</nlm:aff><country xml:lang="fr" wicri:curation="lc">Brésil</country><wicri:regionArea>Hospital do Câncer AC Camargo, Department of Skin Câncer, São Paulo/SP</wicri:regionArea></affiliation></author><author><name sortKey="Landman, Gilles" sort="Landman, Gilles" uniqKey="Landman G" first="Gilles" last="Landman">Gilles Landman</name><affiliation><nlm:aff>NONE</nlm:aff></affiliation><affiliation wicri:level="1"><nlm:aff id="af4-cln_66p1817">Federal University of São Paulo, Department of Pathology, São Paulo/SP, Brazil.</nlm:aff><country xml:lang="fr" wicri:curation="lc">Brésil</country><wicri:regionArea>Federal University of São Paulo, Department of Pathology, São 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wicri:curation="lc">Brésil</country><wicri:regionArea>Hospital do Câncer AC Camargo, Department of Skin Câncer, São Paulo/SP</wicri:regionArea></affiliation></author></analytic><series><title level="j">Clinics</title><idno type="ISSN">1807-5932</idno><idno type="eISSN">1980-5322</idno><imprint><date when="2011">2011</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Merkel cell carcinoma is a very rare and aggressive neoplasm. Due to its rarity, therapeutic guidelines are not well established, especially for regionally advanced disease. Articles in English, French, Italian, Portuguese, and Spanish from the last 20 years were identified in MEDLINE and reviewed. The key word “Merkel” was used for the search, relevant articles were selected, and their references were examined. The most important articles related to epidemiology, genesis and treatment were reviewed. The incidence of Merkel cell carcinoma is increasing due to the advancing age of the population, higher rates of sun exposure and an increasing number of immunocompromised individuals. With regard to etiology, the recently described Merkel Cell polyomavirus is thought to play a role. Either local or regional surgical intervention remains the standard of care, but adjuvant radiotherapy or radiotherapy as a primary treatment have been discussed as reasonable therapeutic options. 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<front><journal-meta><journal-id journal-id-type="nlm-ta">Clinics (Sao Paulo)</journal-id><journal-title-group><journal-title>Clinics</journal-title></journal-title-group><issn pub-type="ppub">1807-5932</issn><issn pub-type="epub">1980-5322</issn><publisher><publisher-name>Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">22012057</article-id><article-id pub-id-type="pmc">3180159</article-id><article-id pub-id-type="doi">10.1590/S1807-59322011001000023</article-id><article-id pub-id-type="publisher-id">cln_66p1817</article-id><article-categories><subj-group subj-group-type="heading"><subject>Review</subject></subj-group></article-categories><title-group><article-title>A review of the epidemiology and treatment of Merkel cell carcinoma</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Duprat</surname><given-names>João Pedreira</given-names></name><xref ref-type="aff" rid="af1-cln_66p1817">I</xref></contrib><contrib contrib-type="author"><name><surname>Landman</surname><given-names>Gilles</given-names></name><xref ref-type="aff" rid="af2-cln_66p1817">II,</xref><xref ref-type="aff" rid="af4-cln_66p1817">IV</xref></contrib><contrib contrib-type="author"><name><surname>Salvajoli</surname><given-names>João Victor</given-names></name><xref ref-type="aff" rid="af3-cln_66p1817">III</xref></contrib><contrib contrib-type="author"><name><surname>Brechtbühl</surname><given-names>Eduard Rene</given-names></name><xref ref-type="aff" rid="af1-cln_66p1817">I</xref></contrib><aff id="af1-cln_66p1817"><label>I</label>Hospital do Câncer AC Camargo, Department of Skin Câncer, São Paulo/SP, Brazil.</aff><aff id="af2-cln_66p1817"><label>II</label>Hospital AC Camargo - Post Graduation, São Paulo/SP, Brazil.</aff><aff id="af3-cln_66p1817"><label>III</label>Instituto do Câncer do Estado de São Paulo Octavio Frias de Oliveira (ICESP), Radio-Oncology, São Paulo, SP, Brazil.</aff><aff id="af4-cln_66p1817"><label>IV</label>Federal University of São Paulo, Department of Pathology, São Paulo/SP, Brazil.</aff></contrib-group><author-notes><corresp id="c1-cln_66p1817">E-mail: <email>jduprat@uol.com.br</email> Tel.: <phone>55 11 2189-5135</phone></corresp></author-notes><pub-date pub-type="ppub"><month>10</month><year>2011</year></pub-date><volume>66</volume><issue>10</issue><fpage>1817</fpage><lpage>1823</lpage><history><date date-type="received"><day>11</day><month>3</month><year>2011</year></date><date date-type="rev-recd"><day>18</day><month>4</month><year>2011</year></date><date date-type="accepted"><day>30</day><month>5</month><year>2011</year></date></history><permissions><copyright-statement>Copyright © 2011 Hospital das Clínicas da FMUSP</copyright-statement><copyright-year>2011</copyright-year><license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc/3.0/"><license-p>This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by-nc/3.0/">http://creativecommons.org/licenses/by-nc/3.0/</ext-link>) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p></license></permissions><abstract><p>Merkel cell carcinoma is a very rare and aggressive neoplasm. Due to its rarity, therapeutic guidelines are not well established, especially for regionally advanced disease. Articles in English, French, Italian, Portuguese, and Spanish from the last 20 years were identified in MEDLINE and reviewed. The key word “Merkel” was used for the search, relevant articles were selected, and their references were examined. The most important articles related to epidemiology, genesis and treatment were reviewed. The incidence of Merkel cell carcinoma is increasing due to the advancing age of the population, higher rates of sun exposure and an increasing number of immunocompromised individuals. With regard to etiology, the recently described Merkel Cell polyomavirus is thought to play a role. Either local or regional surgical intervention remains the standard of care, but adjuvant radiotherapy or radiotherapy as a primary treatment have been discussed as reasonable therapeutic options. An update on this rare neoplasia is essential because of its increasing incidence and changing treatment options.</p></abstract><kwd-group><kwd>Carcinoma, Merkel CELL</kwd><kwd>Literature review</kwd><kwd>Radiotherapy</kwd><kwd>Polyomavirus</kwd></kwd-group><counts><page-count count="7"></page-count></counts></article-meta></front><floats-group><table-wrap id="t1-cln_66p1817" position="float"><label>Table 1</label><caption><p>TNM Classification.</p></caption><table frame="hsides" rules="groups"><thead><tr><td align="left" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1">Tumor (T)</td></tr></thead><tbody><tr><td align="left" valign="top" rowspan="1" colspan="1">T1</td><td align="center" valign="top" rowspan="1" colspan="1">≤2 cm tumor size</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">T2</td><td align="center" valign="top" rowspan="1" colspan="1">>2 cm but no more than 5 cm</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">T3</td><td align="center" valign="top" rowspan="1" colspan="1">>5 cm</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">T4</td><td align="center" valign="top" rowspan="1" colspan="1">invasion of bone, muscle, fascia or cartilage</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1">Nodal (N)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">cN0</td><td align="center" valign="top" rowspan="1" colspan="1">node negative by clinical exam (clinical or imaging exam)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">pN0</td><td align="center" valign="top" rowspan="1" colspan="1">node negative by pathologic exam</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">N1</td><td align="center" valign="top" rowspan="1" colspan="1">metastasis in regional node</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">N1a</td><td align="center" valign="top" rowspan="1" colspan="1">micrometastasis (sentinel node or elective lymphadenectomy)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">N1b</td><td align="center" valign="top" rowspan="1" colspan="1">macrometastasis (clinically detectable, confirmed by surgery or fine-needle aspiration)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">N2</td><td align="center" valign="top" rowspan="1" colspan="1">in-transit metastasis</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1">Metastasis (M)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">M0</td><td align="center" valign="top" rowspan="1" colspan="1">no distant metastasis</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">M1</td><td align="center" valign="top" rowspan="1" colspan="1">metastasis beyond regional lymph nodes</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">M1a</td><td align="center" valign="top" rowspan="1" colspan="1">metastasis to skin, subcutaneous tissues or distant lymph nodes</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">M1b</td><td align="center" valign="top" rowspan="1" colspan="1">metastasis to lung</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">M1c</td><td align="center" valign="top" rowspan="1" colspan="1">metastasis to all other visceral sites</td></tr></tbody></table></table-wrap><table-wrap id="t2-cln_66p1817" position="float"><label>Table 2</label><caption><p>Staging System.</p></caption><table frame="hsides" rules="groups"><thead><tr><td align="left" valign="top" rowspan="1" colspan="1">Stage</td><td align="center" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1">Five-yearSurvival (%)<xref ref-type="table-fn" rid="tfn1-cln_66p1817">*</xref></td></tr></thead><tbody><tr><td align="left" valign="top" rowspan="1" colspan="1">0</td><td align="center" valign="top" rowspan="1" colspan="1">Tis</td><td align="center" valign="top" rowspan="1" colspan="1">N0</td><td align="center" valign="top" rowspan="1" colspan="1">M0</td><td align="left" valign="top" rowspan="1" colspan="1"></td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">IA</td><td align="center" valign="top" rowspan="1" colspan="1">T1</td><td align="center" valign="top" rowspan="1" colspan="1">pN0</td><td align="center" valign="top" rowspan="1" colspan="1">M0</td><td align="center" valign="top" rowspan="1" colspan="1">79</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">IB</td><td align="center" valign="top" rowspan="1" colspan="1">T1</td><td align="center" valign="top" rowspan="1" colspan="1">cN0</td><td align="center" valign="top" rowspan="1" colspan="1">M0</td><td align="center" valign="top" rowspan="1" colspan="1">60</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">IIA</td><td align="center" valign="top" rowspan="1" colspan="1">T2/T3</td><td align="center" valign="top" rowspan="1" colspan="1">pN0</td><td align="center" valign="top" rowspan="1" colspan="1">M0</td><td align="center" valign="top" rowspan="1" colspan="1">58</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">IIB</td><td align="center" valign="top" rowspan="1" colspan="1">T2/T3</td><td align="center" valign="top" rowspan="1" colspan="1">cN0</td><td align="center" valign="top" rowspan="1" colspan="1">M0</td><td align="center" valign="top" rowspan="1" colspan="1">49</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">IIC</td><td align="center" valign="top" rowspan="1" colspan="1">T4</td><td align="center" valign="top" rowspan="1" colspan="1">N0</td><td align="center" valign="top" rowspan="1" colspan="1">M0</td><td align="center" valign="top" rowspan="1" colspan="1">47</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">IIIA</td><td align="center" valign="top" rowspan="1" colspan="1">Any T</td><td align="center" valign="top" rowspan="1" colspan="1">N1a</td><td align="center" valign="top" rowspan="1" colspan="1">M0</td><td align="center" valign="top" rowspan="1" colspan="1">45</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">IIIB</td><td align="center" valign="top" rowspan="1" colspan="1">Any T</td><td align="center" valign="top" rowspan="1" colspan="1">N1b/N2</td><td align="center" valign="top" rowspan="1" colspan="1">M0</td><td align="center" valign="top" rowspan="1" colspan="1">30</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">IV</td><td align="center" valign="top" rowspan="1" colspan="1">Any T</td><td align="center" valign="top" rowspan="1" colspan="1">Any N</td><td align="center" valign="top" rowspan="1" colspan="1">M1</td><td align="center" valign="top" rowspan="1" colspan="1">18</td></tr></tbody></table><table-wrap-foot><fn id="tfn1-cln_66p1817"><p>* (Lemos).</p></fn></table-wrap-foot></table-wrap><table-wrap id="t3-cln_66p1817" position="float"><label>Table 3</label><caption><p>Radiotherapy: protocols and results.</p></caption><table frame="hsides" rules="groups"><thead><tr><td align="left" valign="top" rowspan="1" colspan="1">Author</td><td align="center" valign="top" rowspan="1" colspan="1">Number of patients</td><td align="center" valign="top" rowspan="1" colspan="1">Control primary without resection % (n<sup>o</sup>)</td><td align="center" valign="top" rowspan="1" colspan="1">Control with adjuvant rdt primary (n<sup>o</sup>)/vs. no radiotherapy</td><td align="center" valign="top" rowspan="1" colspan="1">Reference</td></tr></thead><tbody><tr><td align="left" valign="top" rowspan="1" colspan="1">Allen</td><td align="center" valign="top" rowspan="1" colspan="1">251</td><td align="left" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1">90%(32)/92%</td><td align="center" valign="top" rowspan="1" colspan="1">(29)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">Warner</td><td align="center" valign="top" rowspan="1" colspan="1">9</td><td align="left" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1">100%(9)/(0)</td><td align="center" valign="top" rowspan="1" colspan="1">(43)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">Boyle</td><td align="center" valign="top" rowspan="1" colspan="1">41</td><td align="center" valign="top" rowspan="1" colspan="1">36%(30)</td><td align="center" valign="top" rowspan="1" colspan="1">91%(11)</td><td align="center" valign="top" rowspan="1" colspan="1">(33)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">Gillenwater <xref ref-type="table-fn" rid="tfn3-cln_66p1817">##</xref></td><td align="center" valign="top" rowspan="1" colspan="1">66</td><td align="left" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1">44%(26)12%/(34)</td><td align="center" valign="top" rowspan="1" colspan="1">(47)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">Mortier</td><td align="center" valign="top" rowspan="1" colspan="1">26</td><td align="center" valign="top" rowspan="1" colspan="1">100%(9)</td><td align="center" valign="top" rowspan="1" colspan="1">93%(27)/0</td><td align="center" valign="top" rowspan="1" colspan="1">(50)</td></tr><tr><td align="left" valign="top" rowspan="1" colspan="1">Veness</td><td align="center" valign="top" rowspan="1" colspan="1">43</td><td align="center" valign="top" rowspan="1" colspan="1">75%(43)</td><td align="left" valign="top" rowspan="1" colspan="1"></td><td align="center" valign="top" rowspan="1" colspan="1">(51)</td></tr></tbody></table><table-wrap-foot><fn id="tfn3-cln_66p1817"><p>## Radiotherapy to primary and lymph node 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