<?xml version="1.0" encoding="UTF-8"?>
<article article-type="research-article" dtd-version="1.3" xml:lang="en">
  <front>
    <journal-meta>
      <journal-title-group>
        <journal-title>Czech and Slovak Ophthalmology</journal-title>
      </journal-title-group>
    </journal-meta>
    <article-meta>
      <article-id pub-id-type="publisher-id">47</article-id>
      <article-id pub-id-type="doi">10.31348/2018/1/6-1-2018</article-id>
      <article-categories>
        <subj-group>
          <subject>Original article</subject>
        </subj-group>
      </article-categories>
      <title-group>
        <article-title>Merkel cell carcinoma of the eyelid and orbit</article-title>
      </title-group>
      <contrib-group>
        <contrib contrib-type="author">
          <name>
            <surname>Furdová</surname>
            <given-names>Alena</given-names>
          </name>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Michalková</surname>
            <given-names>Mária</given-names>
          </name>
        </contrib>
        <contrib contrib-type="author">
          <name>
            <surname>Javorská</surname>
            <given-names>Lívia</given-names>
          </name>
        </contrib>
      </contrib-group>
      <pub-date date-type="pub" publication-format="electronic">
        <day>3</day>
        <month>7</month>
        <year>2018</year>
      </pub-date>
      <issue>1</issue>
      <elocation-id>6-1-2018</elocation-id>
      <abstract>
        <p>The incidence of Merkel cell carcinoma has tended to increase worldwide in recent years. Merkel cell carcinoma is a rare tumor of the skin that occurs mainly in the sun exposed sites. The malignant reversal of Merkel cells is currently associated with an infection caused by a Merkel cell polyomavirals. In some cases, the disease may have a relatively inconspicuous clinical picture in the initial phase, which is in contrast to its extensive microscopic propagation. For this reason, the risk of late diagnosis or insufficient primary surgery is increased. The diagnostic standard is histological and, in particular, immunohistochemical examination of tumor tissue samples. Merkel cell carcinoma is a marked tendency to local recurrence and early development of metastases in regional lymph nodes, followed by generalization. The basis of treatment is radical excision of the tumor by in most cases by adjuvant radiotherapy targeted at primary place of occurrence and the area of regional draining lymph nodes. The effectiveness of different chemotherapeutic protocols in Merkel cell carcinoma is mostly low and the median survival is low. From a prognostic point of view, Merkel cell carcinoma plays the most important role of staging the tumor at the time of capture. The suspected lesions in the area around the eye, eyelid and orbit need to indicate adequate therapeutic approach that the detection of the disease at the earliest stage. The authors describe the clinical experience in 2 patients with Merkel cell carcinoma of the eyelid and orbit.</p>
      </abstract>
      <kwd-group>
        <kwd>eyelid tumors</kwd>
        <kwd>tumors of the orbit</kwd>
        <kwd>Merkel cell carcinoma</kwd>
      </kwd-group>
    </article-meta>
  </front>
  <back>
    <ref-list>
      <title>References</title>
      <ref id="R7572">
        <mixed-citation>Allen, P.J., Bowne, W.B., Jaques, D.P., et al.: Merkel cell carcinoma: prognosis and treatment of patients from a single institution. J. Clin. Oncol. Off. J. Am. Soc. Clin. Oncol. 2005; 23 (10): 2300–9.</mixed-citation>
      </ref>
      <ref id="R7573">
        <mixed-citation>Becker, J., Mauch, C., Kortmann, R-D., et al.: Short German guidelines: Merkel cell carcinoma. J. Dtsch. Dermatol. Ges. J. Ger. Soc. Dermatol. JDDG. 2008; 6 (Suppl 1): S15-16.</mixed-citation>
      </ref>
      <ref id="R7574">
        <mixed-citation>Becker, J.C., Houben, R., Ugurel, S. et al.: MC polyomavirus is frequently present in Merkel cell carcinoma of European patients. J. Invest. Dermatol. 2009; 129 (1): 248–50.</mixed-citation>
      </ref>
      <ref id="R7575">
        <mixed-citation>Becker, J.C., Schrama, D., Houben, R.: Merkel cell carcinoma. Cell. Mol. Life Sci. CMLS. 2009; 66 (1): 1–8.</mixed-citation>
      </ref>
      <ref id="R7576">
        <mixed-citation>Boratyńska, M., Watorek, E., Smolska, D. et al.: Anticancer effect of sirolimus in renal allograft recipients with de novo malignancies. Transplant. Proc. 2007; 39 (9): 2736–9.</mixed-citation>
      </ref>
      <ref id="R7577">
        <mixed-citation>Bossuyt, W., Kazanjian, A., De Geest, N. et al.: Atonal homolog 1 is a tumor suppressor gene. PLoS Biol. 2009; 7 (2): e39.</mixed-citation>
      </ref>
      <ref id="R7578">
        <mixed-citation>Brenner, B., Sulkes, A., Rakowsky, E. et oligonucleotide-based therapeutics for cancer. Oncogene. 2003; 22 (56): 9087– 96.</mixed-citation>
      </ref>
      <ref id="R7579">
        <mixed-citation>Brunner, M., Thurnher, D., Pammer, J. et al.: Expression of VEGF-A/C, VE- GF-R2, PDGF-alpha/beta, c-kit, EGFR, Her-2/Neu, Mcl-1 and Bmi-1 in Merkel cell carcinoma. Mod. Pathol. Off. J. U. S. Can. Acad. Pathol. Inc. 2008; 21 (7): 876–84.</mixed-citation>
      </ref>
      <ref id="R7580">
        <mixed-citation>Buell, J.F., Trofe, J., Hanaway, M.J. et al.: Immunosuppression and Merkel cell cancer. Transplant. Proc. 2002; 34 (5): 1780–1.</mixed-citation>
      </ref>
      <ref id="R7581">
        <mixed-citation>Busse, P.M., Clark, J.R., Muse, V.V. et al.: Case records of the Massachusetts General Hospital. Case 19-2008. A 63-year-old HIV-positive man with cutaneous Merkel-cell carcinoma. N. Engl. J. Med. 2008; 358 (25): 2717–23.</mixed-citation>
      </ref>
      <ref id="R7582">
        <mixed-citation>Concannon, R., Larcos, G.S., Veness, M.: The impact of (18)F-FDG PET-CT scanning for staging and management of Merkel cell carcinoma: results from Westmead Hospital, Sydney, Australia. J. Am. Acad. Dermatol. 2010; 62 (1): 76–84.</mixed-citation>
      </ref>
      <ref id="R7583">
        <mixed-citation>Dean, N.M., Bennett, C.F.: Antisense</mixed-citation>
      </ref>
      <ref id="R7584">
        <mixed-citation>Dráberová, E., Lukás, Z., Ivanyi, D. et al.: Expression of class III beta-tubulin in normal and neoplastic human tissues. Histochem. Cell Biol. 1998; 109 (3): 231–9.</mixed-citation>
      </ref>
      <ref id="R7585">
        <mixed-citation>Engels, E.A., Frisch, M., Goedert, J.J. et al.: Merkel cell carcinoma and HIV infection. Lancet Lond. Engl. 2002; 359 (9305): 497–8.</mixed-citation>
      </ref>
      <ref id="R7586">
        <mixed-citation>Feng, H., Shuda, M., Chang, Y. et al.: Clonal integration of a polyomavirus in human Merkel cell carcinoma. Science. 2008; 319 (5866): 1096–100.</mixed-citation>
      </ref>
      <ref id="R7587">
        <mixed-citation>Fortina, A.B., Piaserico, S., Caforio, A.L.P. et al.: Immunosuppressive level and other risk factors for basal cell carcinoma and squamous cell carcinoma in heart transplant recipients. Arch. Dermatol. 2004; 140 (9): 1079–85.</mixed-citation>
      </ref>
      <ref id="R7588">
        <mixed-citation>Friedlaender, M.M., Rubinger, D., Rosenbaum, E. et al.: Temporary regression of Merkel cell carcinoma metastases after cessation of cyclosporine. Transplantation. 2002; 73 (11): 1849–50.</mixed-citation>
      </ref>
      <ref id="R7589">
        <mixed-citation>Garneski, K.M., Warcola, A.H., Feng, Q. et al.: Merkel cell polyomavirus is more frequently present in North American than Australian Merkel cell carcinoma tumors. J. Invest. Dermatol. 2009; 129 (1): 246–8.</mixed-citation>
      </ref>
      <ref id="R7590">
        <mixed-citation>Girschik, J., Fritschi, L., Threlfall, T. et al.: Deaths from non-melanoma skin cancer in Western Australia. Cancer Causes Control CCC. 2008; 19 (8): 879–85.</mixed-citation>
      </ref>
      <ref id="R7591">
        <mixed-citation>Goepfert, H., Remmler, D., Silva, E. et al.: Merkel cell carcinoma (endocrine carcinoma of the skin) of the head and neck. Arch. Otolaryngol. Chic. Ill 1960. 1984; 110 (11): 707–12.</mixed-citation>
      </ref>
      <ref id="R7592">
        <mixed-citation>Helmbold, P., Schröter, S., Holzhausen, H.J. et al.: [Merkel cell carcinoma: a diagnostic and therapeutic challenge]. Chir. Z. Alle Geb. Oper. Medizen. 2001; 72 (4): 396–401.</mixed-citation>
      </ref>
      <ref id="R7593">
        <mixed-citation>Hodgson, N.C.: Merkel cell carcinoma: changing incidence trends. J. Surg. Oncol. 2005; 89 (1): 1–4.</mixed-citation>
      </ref>
      <ref id="R7594">
        <mixed-citation>Jirásek, T., Mandys, V., Viklický, V.: Ex - pression of class III beta-tubulin in neuroendocrine tumours of gastrointestinal tract. Folia Histochem. Cytobiol. 2002; 40 (3): 305–10.</mixed-citation>
      </ref>
      <ref id="R7595">
        <mixed-citation>Jirásek, T., Matěj, R., Pock, L. et al.: Karcinom z Merkelových buněk – imunohistologická studie v souboru 11 pacientů. Čes.-Slov. Patol. 2009; 1 (45): 9–13.</mixed-citation>
      </ref>
      <ref id="R7596">
        <mixed-citation>Kassem, A., Schöpflin, A., Diaz, C. et al.: Frequent detection of Merkel cell polyomavirus in human Merkel cell carcinomas and identification of a unique deletion in the VP1 gene. Cancer Res. 2008; 68 (13): 5009–13.</mixed-citation>
      </ref>
      <ref id="R7597">
        <mixed-citation>Kim, J., McNiff, J.M.: Nuclear expression of survivin portends a poor prognosis in Merkel cell carcinoma. Mod. Pathol. Off. J. U. S. Can. Acad. Pathol. Inc. 2008; 21 (6): 764–9.</mixed-citation>
      </ref>
      <ref id="R7598">
        <mixed-citation>Koljonen, V., Kukko, H., Tukiainen, E. et al.: Incidence of Merkel cell carcinoma in renal transplant recipients. Nephrol. Dial. Transplant. Off. Publ. Eur. Dial. Transpl. Assoc. - Eur. Ren. Assoc. 2009; 24 (10): 3231–5.</mixed-citation>
      </ref>
      <ref id="R7599">
        <mixed-citation>Krasagakis, K., Krüger-Krasagakis, S., Tzanakakis, G.N. et al.: Interferon-alpha inhibits proliferation and induces apoptosis of merkel cell carcinoma in vitro. Cancer Invest. 2008; 26 (6): 562–8.</mixed-citation>
      </ref>
      <ref id="R7600">
        <mixed-citation>Krejčí, K., Tichý, T., Horák, P. et al.: Merkel cell carcinoma of the gluteal region with ipsilateral metastasis into the pancreatic graft of a patient after combined kidney-pancreas transplantation. Onkologie. 2010; 33 (10): 520–4.</mixed-citation>
      </ref>
      <ref id="R7601">
        <mixed-citation>Lawenda, B.D., Thiringer, J.K., Foss, R.D. et al.: Merkel cell carcinoma arising in the head and neck: optimizing therapy. Am. J. Clin. Oncol. 2001; 24 (1): 35–42.</mixed-citation>
      </ref>
      <ref id="R7602">
        <mixed-citation>Ludueña, R.F.: Are tubulin isotypes functionally significant. Mol. Biol. Cell. 1993; 4 (5): 445–57. atment. Br. J. Surg. 1991; 78 (2): 138–</mixed-citation>
      </ref>
      <ref id="R7603">
        <mixed-citation>Maubec, E., Duvillard, P., Velasco, V. et al.: Immunohistochemical analysis of EGFR and HER-2 in patients with metastatic squamous cell carcinoma of the skin. Anticancer Res. 2005; 25 (2B): 1205–10.</mixed-citation>
      </ref>
      <ref id="R7604">
        <mixed-citation>Medina-Franco, H., Urist, M.M., Fiveash, J. et al.: Multimodality tre - atment of Merkel cell carcinoma: case series and literature review of 1024 cases. Ann. Surg. Oncol. 2001; 8 (3): 204–8.</mixed-citation>
      </ref>
      <ref id="R7605">
        <mixed-citation>Moosa, M.R., Gralla, J.: Skin cancer in renal allograft recipients--experience in different ethnic groups residing in the same geographical region. Clin. Transplant. 2005; 19 (6): 735–41.</mixed-citation>
      </ref>
      <ref id="R7606">
        <mixed-citation>Pastrana, D.V., Tolstov, Y.L., Becker, J.C. et al.: Quantitation of human seroresponsiveness to Merkel cell polyomavirus. PLoS Pathog. 2009; 5 (9): e1000578.</mixed-citation>
      </ref>
      <ref id="R7607">
        <mixed-citation>Pectasides, D., Pectasides, M., Economopoulos, T.: Merkel cell cancer of the skin. Ann. Oncol. Off. J. Eur. Soc. Med. Oncol. 2006; 17 (10): 1489–95.</mixed-citation>
      </ref>
      <ref id="R7608">
        <mixed-citation>Pilotti, S., Rilke, F., Lombardi, L.: Neu - roendocrine (Merkel cell) carcinoma of the skin. Am. J. Surg. Pathol. 1982; 6 (3): 243–54.</mixed-citation>
      </ref>
      <ref id="R7609">
        <mixed-citation>Popp, S., Waltering, S., Herbst, C. et al.: UV-B-type mutations and chromosomal imbalances indicate common pathways for the development of Merkel and skin squamous cell carcinomas. Int. J. Cancer. 2002; 99 (3): 352–60.</mixed-citation>
      </ref>
      <ref id="R7610">
        <mixed-citation>Poulsen, M., Rischin, D., Walpole, E. et al.: High-risk Merkel cell carcinoma of the skin treated with synchronous carboplatin/etoposide and radiation: a Trans-Tasman Radiation Oncology Group Study--TROG 96:07. J. Clin. Oncol. Off. J. Am. Soc. Clin. Oncol. 2003; 21 (23): 4371–6.</mixed-citation>
      </ref>
      <ref id="R7611">
        <mixed-citation>Poulsen, M.G., Rischin, D., Porter, I. et al.: Does chemotherapy improve survival in high-risk stage I and II Merkel cell carcinoma of the skin? Int. J. Radiat. Oncol. Biol. Phys. 2006; 64 (1): 114–9.</mixed-citation>
      </ref>
      <ref id="R7612">
        <mixed-citation>Ratner, D., Nelson, B.R., Brown, M,D. et al.: Merkel cell carcinoma. J. Am. Acad. Dermatol. 1993; 29 (2 Pt 1): 143–56.</mixed-citation>
      </ref>
      <ref id="R7613">
        <mixed-citation>Rečková, M., Lawrence, H.E.: Nezvyčajný prípad dlhodobého prežívania pacientky s chemorefraktérnym Merkelovym karcinómom. Klin. Onkol. 2007; 20 (5): 354–6.</mixed-citation>
      </ref>
      <ref id="R7614">
        <mixed-citation>Schmid, C., Beham, A., Feichtinger, J. et al.: Recurrent and subsequently metastasizing Merkel cell carcinoma in a 7-year-old girl. Histopathology. 1992; 20 (5): 437–9.</mixed-citation>
      </ref>
      <ref id="R7615">
        <mixed-citation>Scott, C.A., Walker, C.C., Neal, D.A. et al.: Beta-tubulin epitope expression in normal and malignant epithelial cells. Arch. Otolaryngol. Head Neck Surg. 1990; 116 (5): 583–9.</mixed-citation>
      </ref>
      <ref id="R7616">
        <mixed-citation>Shaw, J.H., Rumball, E.: Merkel cell tumour: clinical behaviour and tre-</mixed-citation>
      </ref>
      <ref id="R7617">
        <mixed-citation>Sihto, H., Kukko, H., Koljonen, V. et al.: Clinical factors associated with Merkel cell polyomavirus infection in Merkel cell carcinoma. J. Natl. Cancer Inst. 2009; 101 (13): 938–45.</mixed-citation>
      </ref>
      <ref id="R7618">
        <mixed-citation>Smith, D.F., Messina, J.L., Perrott, R. et al.: Clinical approach to neuroendocrine carcinoma of the skin (Merkel cell carcinoma). Cancer Control J. Moffitt Cancer Cent. 2000; 7 (1): 72–83.</mixed-citation>
      </ref>
      <ref id="R7619">
        <mixed-citation>Swick, B.L., Ravdel, L., Fitzpatrick, J.E. et al.: Platelet-derived growth factor receptor alpha mutational status and immunohistochemical expression in Merkel cell carcinoma: implications for treatment with imatinib mesylate. J. Cutan. Pathol. 2008; 35 (2): 197–202.</mixed-citation>
      </ref>
      <ref id="R7620">
        <mixed-citation>Tai, P.T., Yu, E., Tonita, J. et al.: Merkel cell carcinoma of the skin. J. Cutan. Med. Surg. 2000; 4 (4): 186–95.</mixed-citation>
      </ref>
      <ref id="R7621">
        <mixed-citation>Tai, P.T., Yu, E., Winquist, E. et al.: Chemotherapy in neuroendocrine/Merkel cell carcinoma of the skin: case series and review of 204 cases. J. Clin. Oncol. Off. J. Am. Soc. Clin. Oncol. 2000; 18 (12): 2493–9.</mixed-citation>
      </ref>
      <ref id="R7622">
        <mixed-citation>Toker, C.: Trabecular carcinoma of the skin. Arch. Dermatol. 1972; 105 (1): 107–10.</mixed-citation>
      </ref>
      <ref id="R7623">
        <mixed-citation>Ullrich, S.E.: Sunlight and skin cancer: lessons from the immune system. Mol. Carcinog. 2007; 46 (8): 629–33.</mixed-citation>
      </ref>
      <ref id="R7624">
        <mixed-citation>Ullrich, S.E.: Mechanisms underlying UV -induced immune suppression. Mutat. Res. 2005; 571 (1–2): 185–205.</mixed-citation>
      </ref>
      <ref id="R7625">
        <mixed-citation>Van Keymeulen, A., Mascre, G., Youseff, K.K. et al.: Epidermal progenitors give rise to Merkel cells during embryonic development and adult homeostasis. J. Cell Biol. 2009; 187 (1): 91–100.</mixed-citation>
      </ref>
      <ref id="R7626">
        <mixed-citation>Veness, M.J., Harris, D.: Role of radio - therapy in the management of organ transplant recipients diagnosed with non-melanoma skin cancers. Australas. Radiol. 2007; 51 (1): 12–20.</mixed-citation>
      </ref>
      <ref id="R7627">
        <mixed-citation>Veness, M.J., Morgan, G.J., Gebski, V.: Adjuvant locoregional radiotherapy as best practice in patients with Merkel cell carcinoma of the head and neck. Head Neck. 2005; 27 (3): 208–16.</mixed-citation>
      </ref>
      <ref id="R7628">
        <mixed-citation>Walsh, N.M.: Primary neuroendocri - ne (Merkel cell) carcinoma of the skin: morphologic diversity and implications thereof. Hum. Pathol. 2001; 32 (7): 680–</mixed-citation>
      </ref>
      <ref id="R7629">
        <mixed-citation>Weller, K., Vetter-Kauzcok, C., Kähler, K. et al.: Guideline Implementation in Merkel Cell Carcinoma: An Example of a Rare Disease. Dtsch. Ärztebl. Int. 2006; 103 : A 2791–6.</mixed-citation>
      </ref>
      <ref id="R7630">
        <mixed-citation>Yiengpruksawan, A., Coit, D.G., Thaler, H.T. et al.: Merkel cell carcinoma. Prognosis and management. Arch. Surg. Chic. Ill 1960. 1991; 126 (12): 1514–9.</mixed-citation>
      </ref>
    </ref-list>
  </back>
</article>
