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Aktuelle Dermatologie 2019; 45(01/02): 60-66
DOI: 10.1055/a-0806-9934
Eine Klinik im Blickpunkt
© Georg Thieme Verlag KG Stuttgart · New York

Das Merkelzellkarzinom: Ätiopathogenese und Management

Merkel Cell Carcinoma: Aetiopathogenesis and Management
J. Heitmann
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Würzburg
,
R. Houben
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Würzburg
,
D. Schrama
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Würzburg
,
M. Goebeler
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Würzburg
,
A. Gesierich
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Würzburg
,
B. Schilling
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Würzburg
› Author Affiliations
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Publication History

Publication Date:
12 February 2019 (online)

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Zusammenfassung

Das Merkelzellkarzinom (MCC) ist ein seltenes Malignom mit neuroendokriner Differenzierung, an dem v. a. ältere und immunsupprimierte Patienten erkranken. Die Inzidenz des Tumors nimmt weiter zu; die Prognose ist mit einer 5-Jahres-Überlebensrate von 0 – 18 % im Stadium III und IV schlecht. Ein Zusammenhang mit dem Merkelzell-Polyoma-Virus oder einer vermehrten UV-Exposition gelten als gesichert. Das MCC präsentiert sich als solitärer, derber, kuppelförmiger, schnell wachsender Tumor. Die Diagnose wird mittels histologischer und immunhistochemischer Untersuchungen gestellt. 2017 wurde von der UICC eine neue Stadieneinteilung basierend auf der TNM-Klassifikation erstellt. Die Prognose ist stark abhängig vom Tumorstadium bei Erstdiagnose, weshalb entsprechende Staginguntersuchungen durchgeführt werden sollten. Therapeutisch steht, sofern möglich, die komplette Exzision des Tumors mit ausreichendem Sicherheitsabstand und ggf. die Exstirpation von Lymphknotenmetastasen im Vordergrund. Anschließend sollte eine Radiatio des Tumorbetts und der Lymphabflussbahnen erfolgen. In metastasierten Stadien hat sich in den letzten Jahren die Immuntherapie als Therapie der Wahl herauskristallisiert; die Chemotherapie spielt seitdem eine zunehmend geringere Rolle. So zeigten prospektive Studien mit Pembrolizumab vielversprechende Ergebnisse mit einer Ansprechrate von 56 % und einem progressionsfreien Überleben nach 6 Monaten von 67 %. Seit Oktober 2017 ist mit Avelumab, einem anti-PD-L1-Antikörper, die erste Immuntherapie zur Behandlung des metastasierten MCC in Deutschland zugelassen. Da die Therapie des metastasierten MCC trotz dieser innovativen Behandlungsansätze auch weiterhin als palliativ anzusehen ist, sollten frühzeitig zusätzlich palliative und psychoonkologische Therapiekonzepte miteinbezogen werden. Nach abgeschlossener Tumortherapie sollten regelmäßige Nachsorgeuntersuchungen erfolgen, um frühzeitig Rezidive oder Metastasen zu detektieren.

Abstract

Merkel cell carcinoma (MCC) is a rare malignant disease that is most commonly seen in elderly and immunosuppressed patients. The incidence has risen over the last few years and the prognosis is poor with 5-year survival rates between 0 – 18 % in stage III and IV. The Merkel cell polyoma virus and UV-exposition play a central role for the pathogenesis of MCC. It presents as a single, solid, dome-shaped and rapidly growing tumor. The diagnosis is verified by histologic and immunhistochemical examinations. In 2017 a new staging system based on the TNM-classification was released by the UICC. The prognosis depends on the stage at primary diagnosis. Wide excision of the tumor and, if required, of lymph node metastases is important. Surgery should always be followed by radiation of the tumor region and the draining lymphatic system. Over the last few years chemotherapy was replaced by immunotherapy as first line therapy in advanced stage MCC. In prospective studies pembrolizumab showed a response rate of 56 % with a progression-free survival of 67 %. In October 2017 the anti-PD-L1-antibody avelumab was the first immune checkpoint inhibitor approved for the treatment of advanced MCC in Germany. However, the prognosis is still poor and palliative and psychooncological services should be offered to the patients at an early stage. After treatment patients should undergo regular follow-up examinations.

 

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