Zusammenfassung
Das Merkel-Zell-Karzinom ist ein seltener, aggressiver maligner neuroendokriner Hauttumor, der früh lymphogen metastasieren kann und zu lokalen Rezidiven neigt. Die Prognose des Merkel-Zell-Karzinoms variiert in Abhängigkeit von Tumorgröße und Erkrankungsstadium. Ein Großteil der Rezidive tritt während der ersten 2 Jahre nach Diagnosestellung auf. Die 5-Jahres-Überlebensrate liegt bei Primärtumoren mit einem Durchmesser von < 2 cm bei 66–75 %, bei Primärtumoren > 2 cm bei 50–60 %. Im Fall von Lymphknotenmetastasen liegt sie bei 42–52 %, während sie bei einer bestehenden Fernmetastasierung nur noch 17–18 % beträgt. Ein umfassendes Staging inklusive der Wächterlymphknotenbiopsie ist notwendig, um die Risikoabschätzung für eine Fernmetastasierung sowie die bestmöglichen Therapieempfehlungen zu gewährleisten. Nach der Operation mit ausreichendem Sicherheitsabstand gilt eine adjuvante Radiatio des Tumorbetts und der Lymphknotenstation leitlinienkonform als notwendig, um Lokalrezidive bzw. metastatische Absiedlungen zu vermeiden.
Abstract
Merkel cell carcinoma is a rare aggressive malignant neuroendocrine skin tumor, which can metastasize to lymph nodes early and often shows local recurrence. The prognosis depends on tumor size and disease stage. The majority of recurrences appear during the first 2 years after the primary diagnosis. The 5-year survival rate for primary tumor < 2 cm is 66–75 % and for primary tumors > 2 cm is 50–60 %. With lymph node metastases the 5-year survival rate is 42–52 %, while with distant metastases it drops to 17–18 %. Extensive staging inclusive sentinel lymph node biopsy is essential to assess the risk for distant metastasis and to allow the best recommendations for therapy. After surgical treatment with adequate safety margin, subsequent adjuvant radiation therapy of the tumor region and lymphatic draining basin is recommended to reduce the risk of local recurrence and lymphatic spread.
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Interessenkonflikt. F. Kleffner, J. Schürholz, S. Burckhardt, C. Mauch und M. Schlaak geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben. Im Falle von nicht mündigen Patienten liegt die Einwilligung eines Erziehungsberechtigten oder des gesetzlich bestellten Betreuers vor.
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Ein Erratum zu diesem Beitrag ist unter http://dx.doi.org/10.1007/s00105-014-3564-y zu finden.
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Kleffner, F., Schürholz, J., Burckhardt, S. et al. Merkel-Zell-Karzinom. Hautarzt 65, 823–832 (2014). https://doi.org/10.1007/s00105-014-2854-8
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DOI: https://doi.org/10.1007/s00105-014-2854-8