Aktuelles Update zur Tumorbiologie und Behandlung des Merkelzell-Karzinoms

Zusammenfassung

Das Merkelzell-Karzinom (MCC) geht aus Merkelzellen hervor, die in der Basalschicht der Epidermis lokalisiert sind. Es imponiert am häufigsten im Kopf- und Halsbereich älterer hellhäutiger Patienten und wird als tödlichster Hautkrebs angesehen. Die radikale chirurgische Exzision verbunden mit der pathologischen Beurteilung des gesamten entnommenen Tumorgewebes stellt die empfohlene Behandlungsmethode dar. Frühe MCC-Stadien können durch chirurgische Maßnahmen mit oder ohne postoperative Radiotherapie geheilt werden, fortgeschrittene Stadien gelten hingegen als unheilbar. 2008 wurde ein neues humanes Polyoma-Virus im Genom des MCC Tumors entdeckt, das Merkelzell-Polyoma-Virus (MCPyV). Das MCPyV ist das erste Beispiel eines humanen onkogenen Polyoma-Virus. Die spezifischen Mutationen im viralen Genom und die klonale Integration in das Tumorgenom sind starke Beweise dafür, dass MCPyV kein sekundär infizierender Passagier-Virus ist. Ziel dieses Review-Artikels ist es, diesen seltenen Hautkrebs im Detail zu beschreiben und auf neueste Fortschritte in der Forschung zum MCC aufmerksam zu machen.

Abstract

Merkel cell carcinoma (MCC) is a carcinoma arising from Merkel cells located in the basal layer of the epidermis. The skin of the head and neck is a common site for MCC, occurring generally in fair-skinned elderly patients. MCC is considered as the most lethal skin cancer. Radical surgical excision with pathological verification of complete removal of the tumour is the recommended treatment. Early MCC can be cured by surgery with or without postoperative radiation therapy, whereas advanced MCC is currently considered to be incurable. In the year 2008, a new polyoma virus was found in the tumour genome of the MCC tumours. MCPyV (Merkel cell polyoma virus) appears to be the first example of a human oncogenic polyoma virus. Specific mutations in the viral genome and its clonal integration to the tumour genome are strong evidence against MCPyV as being a passenger virus that secondarily infects MCC tumours. The purpose of this review article is to shed light on this rare skin cancer and introduce the latest advances in research on MCC.

Schlüsselwörter

Merkelzell-Karzinom - Merkelzellen - Polyomavirus - Haut

Key words

Merkel cell carcinoma - Merkel cells - polyoma virus - skin

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