Merkelzellkarzinom

Zusammenfassung

Das Merkelzellkarzinom ist ein hochaggressives neuroendokrines Karzinom der Haut, das klinisch als rasch wachsender, rötlich-bläulich-livider Tumorknoten imponiert. Ulzerationen sind selten. Es betrifft bevorzugt die lichtexponierte Haut sehr alter oder langjährig immunsupprimierter Patienten.

Einen wichtigen Beitrag zur Pathogenese des Merkelzellkarzinoms gelang durch die Entdeckung des Merkelzell-Polyoma-Virus, welches in der Mehrzahl der Fälle nachweisbar und mutiert ist. Das mutierte sog. Large-T-Antigen kann aber noch in den Zellzyklus eingreifen und dadurch die Tumorigenese fördern.

Die Prognosemarker des Merkelzellkarzinoms sind noch umstritten, wichtig ist der Befall der regionalen Lymphknoten.

Therapiestandard ist im Stadium I die Exzision mit Sicherheitsabstand von 3 cm und die adjuvante Strahlentherapie der Tumorregion und evtl. der regionären Lymphknoten. Die Sentinel node biopsy sollte angestrebt werden. Auch im Stadium II ist chirurgische Therapie angezeigt mit nachfolgender Bestrahlung, bei inoperablen Zuständen ist die alleinige Radiatio vergleichbar effektiv. Chemotherapien – noch nicht gut evaluiert – sind Therapie der Wahl im Stadium III mit guten, allerdings meist nur kurzfristigen Remissionen. Daher sind eine Reihe von neuen sog. targeted therapies und Vakzinierungen beim Merkelzellkarzinom in der Diskussion und in klinischen Studien.

Das Merkelzellkarzinom, der bösartigste Tumor der Haut, hat trotz einiger neuerer Erkenntnisse noch immer eine Reihe von Geheimnissen!

Abstract

Merkel cell carcinomas (MCC) are highly aggressive neuroendocrine tumors of skin usually localized in UV-exposed skin of elderly and immunoincompetent patients. The isolation of a new virus, the Merkel cell polyoma virus, in most MCC is a main stay in clarifying the pathogenesis of MCC and especially its so-called Large T antigen seems tumorigenic. This antigen is mutated in MCC but still active in cell cycle regulation, inducing the proliferation of tumor cells.

There are no established markers of prognosis and no evidence based therapeutic procedures.

In stage I there is consent to remove the primary with 2 – 3 cm distance and applying radiation to the tumor bed and the regional lymph nodes. Sentinel node biopsy should be done if possible. Also in tumor stage II surgery and radiation are recommended. Chemotherapies are the first line therapies in stage III but not yet evidence based, usually of limited effect and palliative. Currently, innovative strategies such as targeted therapies and vaccinations are in discussion and under clinical trials. These aspects are discussed in this review paper.

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