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Merkel-Zell-Karzinom des Augenlids – eine häufig verkannte Diagnose

Klinische Aspekte und Behandlungsmethoden

Merkel cell carcinoma of the eyelid. An often unrecognized tumor entity

Clinical aspects and treatment strategies

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Zusammenfassung

Das Merkel-Zell-Karzinom des Augenlids ist eine aggressive, hochmaligne Neoplasie der Haut. Es stellt mit ca. 0,5 % eine kleine Gruppe der Lidtumoren dar. Dennoch ist das Merkel-Zell-Karzinom von Bedeutung für den Augenarzt, da durch sein klinisches Erscheinungsbild häufig eine Verwechslung mit einem Chalazion oder Hordeolum oder dem wesentlich weniger gefährlichen Basalzellkarzinom auftritt und somit die Behandlung verzögert wird. In diesem Beitrag werden klinische Aspekte genannt, die dem Augenarzt helfen sollen, ein Merkel-Zell-Karzinom früher in Erwägung zu ziehen. Zusätzlich wird eine diagnostische und therapeutische Aufarbeitung bei Merkel-Zell-Karzinomen unter Berücksichtigung der speziellen Lidanatomie besprochen.

Abstract

Merkel cell carcinoma of the eyelid is an aggressive, highly malignant tumor of the skin. Totaling approximately 0.5 % of all tumors of the eyelid, it constitutes a relatively small group of lid tumors. Nevertheless Merkel cell carcinoma is of significance to the ophthalmologist. Because of its clinical presentation it can be easily confused as a chalazion, a hordeolum or the lesser aggressive basal cell carcinoma. This often leads to delayed treatment. In this article we describe clinical aspects, which aim to help the ophthalmologist suspect Merkel cell carcinoma earlier. Additionally we outline a diagnostic and therapeutic workup taking into consideration the special anatomy of the eyelid.

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Literatur

  1. Al-Ahmadie HA, Mutasim DF, Mutema GK (2004) A case of intraepidermal Merkel cell carcinoma within squamous cell carcinoma in-situ: Merkel cell carcinoma in-situ? Am J Dermatopathol 26:230–233

    Article  PubMed  Google Scholar 

  2. Amber K, Mcleod MP, Nouri K (2013) The Merkel cell polyomavirus and its involvement in Merkel cell carcinoma. Dermatol Surg 39:232–238

    Article  CAS  PubMed  Google Scholar 

  3. An KP, Ratner D (2001) Merkel cell carcinoma in the setting of HIV infection. J Am Acad Dermatol 45:309–312

    Article  CAS  PubMed  Google Scholar 

  4. Becker JC, Kauczok CS, Ugurel S et al (2008) Merkel cell carcinoma: molecular pathogenesis, clinical features and therapy. J Dtsch Dermatol Ges 6:709–719

    Article  PubMed  Google Scholar 

  5. Edge SB, Compton CC (2010) The American Joint Committee on Cancer: the 7th edition of the AJCC cancer staging manual and the future of TNM. Ann Surg Oncol 17(6):1471–1474. doi:10.1245/s10434-010-0985-4

    Article  PubMed  Google Scholar 

  6. Esen BA, Pinarbasi B, Buyukbabani N et al (2005) Merkel-cell carcinoma arising after liver transplantation: a case report. Transplant Proc 37:4413–4415

    Article  CAS  PubMed  Google Scholar 

  7. Espinoza GM, Prost AM (2016) Upper eyelid reconstruction. Facial Plast Surg Clin North Am 24:173–182

    Article  PubMed  Google Scholar 

  8. Ferringer T, Rogers HC, Metcalf JS (2005) Merkel cell carcinoma in situ. J Cutan Pathol 32:162–165

    Article  PubMed  Google Scholar 

  9. Fields RC, Busam KJ, Chou JF et al (2012) Recurrence after complete resection and selective use of adjuvant therapy for stage I through III Merkel cell carcinoma. Cancer 118:3311–3320

    Article  PubMed  Google Scholar 

  10. Gillenwater AM, Hessel AC, Morrison WH et al (2001) Merkel cell carcinoma of the head and neck: effect of surgical excision and radiation on recurrence and survival. Arch Otolaryngol Head Neck Surg 127:149–154

    Article  CAS  PubMed  Google Scholar 

  11. Hauschild A, Garbe C (2006) Brief guideline – cutaneous neuroendocrine carcinoma (Merkel cell carcinoma). J Dtsch Dermatol Ges 4:508–510

    Article  PubMed  Google Scholar 

  12. Heath M, Jaimes N, Lemos B et al (2008) Clinical characteristics of Merkel cell carcinoma at diagnosis in 195 patients: the AEIOU features. J Am Acad Dermatol 58:375–381

    Article  PubMed  PubMed Central  Google Scholar 

  13. Heindl LM, Holbach LM (2008) Eyelids. In: Naumann GOH, Holbach L, Kruse FE (Hrsg) Applied pathology for ophthalmic microsurgeons. Springer, Berlin, S 30–44

    Google Scholar 

  14. Herbert HM, Sun MT, Selva D et al (2014) Merkel cell carcinoma of the eyelid: management and prognosis. JAMA Ophthalmol 132:197–204

    Article  PubMed  Google Scholar 

  15. Hishmi AM, Koch KR, Matthaei M et al (2016) Modified Hughes procedure for reconstruction of large full-thickness lower eyelid defects following tumor resection. Eur J Med Res 21(1). doi:10.1186/s40001-016-0221-1

    PubMed  PubMed Central  Google Scholar 

  16. Holds JB (2016) Lower eyelid reconstruction. Facial Plast Surg Clin North Am 24:183–191

    Article  PubMed  Google Scholar 

  17. Houben R, Shuda M, Weinkam R et al (2010) Merkel cell polyomavirus-infected Merkel cell carcinoma cells require expression of viral T antigens. J Virol 84:7064–7072

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Kleffner F, Schurholz J, Burckhardt S et al (2014) Merkel cell carcinoma. Hautarzt 65:823–830 (quiz 831–822)

    Article  CAS  PubMed  Google Scholar 

  19. Kokoska ER, Kokoska MS, Collins BT et al (1997) Early aggressive treatment for Merkel cell carcinoma improves outcome. Am J Surg 174:688–693

    Article  CAS  PubMed  Google Scholar 

  20. Kopecky A, Koch KR, Bucher F et al (2016) Results of Cutler-Beard procedure for reconstruction of extensive full thickness upper eyelid defects following tumor resection. Ophthalmologe 113:309–313

    Article  CAS  PubMed  Google Scholar 

  21. Lemos BD, Storer BE, Iyer JG et al (2010) Pathologic nodal evaluation improves prognostic accuracy in Merkel cell carcinoma: analysis of 5823 cases as the basis of the first consensus staging system. J Am Acad Dermatol 63:751–761

    Article  PubMed  PubMed Central  Google Scholar 

  22. Lentzsch A, Kakkassery V, Heindl LM (2016) Basalzellkarzinom – aktuelle Leitlinien und neue Behandlungsoptionen. Z Prakt Augenheilkd 37:59–62

    Google Scholar 

  23. Merritt H, Sniegowski MC, Esmaeli B (2014) Merkel cell carcinoma of the eyelid and periocular region. Cancers (Basel) 6:1128–1137

    Article  Google Scholar 

  24. Metz KA, Jacob M, Schmidt U et al (1998) Merkel cell carcinoma of the eyelid: histological and immunohistochemical features with special respect to differential diagnosis. Graefes Arch Clin Exp Ophthalmol 236:561–566

    Article  CAS  PubMed  Google Scholar 

  25. Moll I, Bladt U, Jung EG (1992) Distribution of Merkel cells in acute UVB erythema. Arch Dermatol Res 284:271–274

    Article  CAS  PubMed  Google Scholar 

  26. Oram CW, Bartus CL, Purcell SM (2016) Merkel cell carcinoma: a review. Cutis 97:290–295

    PubMed  Google Scholar 

  27. Pan D, Narayan D, Ariyan S (2002) Merkel cell carcinoma: five case reports using sentinel lymph node biopsy and a review of 110 new cases. Plast Reconstr Surg 110:1259–1265

    Article  PubMed  Google Scholar 

  28. Peters GB 3rd, Meyer DR, Shields JA et al (2001) Management and prognosis of Merkel cell carcinoma of the eyelid. Ophthalmology 108:1575–1579

    Article  PubMed  Google Scholar 

  29. Schlaak M, Podewski T, Von Bartenwerffer W et al (2012) Induction of durable responses by oral etoposide monochemotherapy in patients with metastatic Merkel cell carcinoma. Eur J Dermatol 22:187–191

    CAS  PubMed  Google Scholar 

  30. Schwartz JL, Griffith KA, Lowe L et al (2011) Features predicting sentinel lymph node positivity in Merkel cell carcinoma. J Clin Oncol 29:1036–1041

    Article  PubMed  Google Scholar 

  31. Sinclair N, Mireskandari K, Forbes J et al (2003) Merkel cell carcinoma of the eyelid in association with chronic lymphocytic leukaemia. Br J Ophthalmol 87:240

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Smith VA, Camp ER, Lentsch EJ (2012) Merkel cell carcinoma: identification of prognostic factors unique to tumors located in the head and neck based on analysis of SEER data. Laryngoscope 122:1283–1290

    Article  PubMed  Google Scholar 

  33. Sniegowski MC, Warneke CL, Morrison WH et al (2014) Correlation of American Joint Committee on Cancer T category for eyelid carcinoma with outcomes in patients with periocular Merkel cell carcinoma. Ophthal Plast Reconstr Surg 30:480–485

    Article  PubMed  Google Scholar 

  34. Su W, Kheir SM, Berberian B et al (2008) Merkel cell carcinoma in situ arising in a trichilemmal cyst: a case report and literature review. Am J Dermatopathol 30:458–461

    Article  PubMed  Google Scholar 

  35. Suarez C, Rodrigo JP, Ferlito A et al (2004) Merkel cell carcinoma of the head and neck. Oral Oncol 40:773–779

    Article  PubMed  Google Scholar 

  36. Tang CK, Toker C (1978) Trabecular carcinoma of the skin: an ultrastructural study. Cancer 42:2311–2321

    Article  CAS  PubMed  Google Scholar 

  37. Toker C (1972) Trabecular carcinoma of the skin. Arch Dermatol 105:107–110

    Article  CAS  PubMed  Google Scholar 

  38. Voog E, Biron P, Martin JP et al (1999) Chemotherapy for patients with locally advanced or metastatic Merkel cell carcinoma. Cancer 85:2589–2595

    Article  CAS  PubMed  Google Scholar 

  39. Yin VT, Merritt HA, Sniegowski M et al (2015) Eyelid and ocular surface carcinoma: diagnosis and management. Clin Dermatol 33:159–169

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Zentrum für Augenheilkunde, Universitätsklinik Köln, Kerpener Str. 62, 50924, Köln, Deutschland

    R. Hoerster, K. R. Koch & L. M. Heindl

  2. Klinik für Dermatologie und Venerologie, Hauttumorzentrum im CIO Köln-Bonn, Universitätsklinik Köln, Kerpener Str. 62, 50924, Köln, Deutschland

    M. Schlaak & C. Mauch

  3. Zentrum für Pathologie, Universitätsklinik Köln, Kerpener Str. 62, 50924, Köln, Deutschland

    M. Ortmann

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  1. R. Hoerster
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  2. M. Schlaak
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  3. K. R. Koch
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  6. L. M. Heindl
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Correspondence to R. Hoerster.

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Interessenkonflikt

R. Hoerster, M. Schlaak, K.R. Koch, M. Ortmann, C. Mauch und L.M. Heindl geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Hoerster, R., Schlaak, M., Koch, K.R. et al. Merkel-Zell-Karzinom des Augenlids – eine häufig verkannte Diagnose. Ophthalmologe 114, 134–139 (2017). https://doi.org/10.1007/s00347-016-0355-0

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