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Merkel-Zell-Karzinom

Ein hochaggressiver Tumor mit möglicher viraler Genese

Merkel cell carcinoma

A highly aggressive tumor with possible viral etiology

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Zusammenfassung

Das Merkel-Zell-Karzinom (MZK), auch als neuroendokrines Karzinom der Haut bezeichnet, ist ein seltener, hoch aggressiver Tumor. Es entwickelt sich meist auf chronisch lichtgeschädigter Haut, im höheren Lebensalter sowie unter Immunsuppression. Frühzeitig auftretende Lokalrezidive und Lymphknotenmetastasen prägen den klinischen Verlauf. Im Zentrum der Diskussion um die Ätiologie steht derzeit das kürzlich neu entdeckte Merkel-Zell-Polyomavirus (MCPyV). Die Infektion mit MCPyV erfolgt dabei offenbar sehr früh und das Virus findet sich auch im Gewebe Gesunder. Möglicherweise sorgt eine Mutation im MCPyV-Genom letztendlich für die Tumorentstehung. Die 5-Jahres-Überlebensrate von Patienten mit primärem MZK liegt nach rein chirurgischer Therapie bei nur 30–40%. Durch eine zusätzliche adjuvante Strahlentherapie erhöht sie sich auf ca. 75%. Im Falle von Lymphknoten- oder Fernmetastasen kommen neben Operation und Radiatio adjuvante Chemotherapien zum Einsatz, die sich an Protokolle anlehnen, wie sie beim kleinzelligen Bronchialkarzinom Anwendung finden. Trotz sehr hoher Remissionsraten unter diesen Therapien sind Rezidive im Stadium der Fernmetastasierung jedoch die Regel. MZK gelten als chemosensitiv, nicht jedoch als chemokurabel.

MZK-Patienten sollten einem aggressiven, gleichwohl jedoch individuell abgestimmten Therapieregime zugeführt werden. Insbesondere eine konsequente Integration der Strahlentherapie in das Behandlungskonzept kann die Prognose für die betroffenen Patienten wesentlich verbessern.

Abstract

Merkel cell carcinoma (MCC) or neuroendocrine carcinoma of the skin, is a rare and highly aggressive tumor which typically develops in chronically sun-damaged skin in aged or immunosuppressed patients. The clinical course is characterized by early local recurrence and lymphatic metastases. The current discussion on the etiology of MCC is dominated by the recently discovered Merkel cell polyoma virus (MCPyV). Apparently, MCPyV infection takes place early in life and the virus can also be found in healthy tissue. Possibly, a mutation of the viral genome is responsible for the development of the tumor. The 5 year survival rate of patients with primary MCC is only 30–40% after surgical therapy alone but can increase to about 75% after additional adjuvant radiotherapy. In cases with lymphatic or distant metastases various chemotherapy protocols in addition to operative and radiation therapy analogous to those for small cell lung cancer therapy have been found to be effective. Nevertheless, very high recurrence rates are typical in patients with distant metastases. Thus, MCC is regarded as chemosensitive but not chemocurable.

Patients with MCC should be treated with an aggressive but individually adapted concept. The consequent integration of radiotherapy into the therapeutic approach can improve the prognosis.

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Authors and Affiliations

  1. Universitätshautklinik, Ruprecht-Karls-Universität, Voßstr. 2, 69115, Heidelberg, Deutschland

    F. Toberer, S. Werchau, A. Enk &  P. Helmbold

  2. Radiologische Klinik, Abteilung Radioonkologie, Ruprecht-Karls-Universität, Heidelberg, Deutschland

    M. Bischof

  3. Abteilung für Allgemeine, Viszerale und Transplantationschirurgie, Ruprecht-Karls-Universität, Heidelberg, Deutschland

    M. Büchler

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  1. F. Toberer
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  2. S. Werchau
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  5. A. Enk
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  6. P. Helmbold
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Correspondence to P. Helmbold.

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Toberer, F., Werchau, S., Bischof, M. et al. Merkel-Zell-Karzinom. Chirurg 82, 653–660 (2011). https://doi.org/10.1007/s00104-010-2066-4

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