Das periokuläre Merkelzellkarzinom – eine Übersicht klinischer Aspekte und aktueller Therapieoptionen

Zusammenfassung

Das Merkel-Zell-Karzinom (MCC) stellt einen seltenen, jedoch hochaggressiven und rasch expandierenden malignen Hauttumor dar. Die periokuläre Region ist in etwa 10% der Fälle betroffen. Die aktuelle Therapieempfehlung des resektablen, nicht metastasierten MCC umfasst die chirurgische Totalexzision, allerdings sind bei Diagnosestellung bereits häufig Lymphknoten- oder Fernmetastasen vorhanden. Seit der Erstzulassung einer Immun-Checkpoint-Inhibitor-Therapie mit Avelumab für das metastasierte MCC im Jahr 2016 hat sich das mittlere Überleben im Vergleich zur zytostatischen Therapie erheblich gebessert bei gleichzeitig seltenerem Auftreten schwerwiegender therapieassoziierter unerwünschter Ereignisse. Weitere Immun-Checkpoint-Inhibitoren mit ersten vielversprechenden Ergebnissen sind derzeit noch in der klinischen Erprobung. Eine interdisziplinäre Betreuung an einem spezialisierten Zentrum mit Vorstellung in einem Tumorboard ist bei Patienten MCC aufgrund der komplexen Diagnostik, Therapie und Prognoseabschätzung essenziell.

Abstract

Merkel cell carcinoma (MCC) is a rare but highly aggressive and rapidly expanding malignant skin tumor. It affects the periocular region in approximately 10% of cases. The current treatment recommendation for resectable non-metastatic MCC comprises total surgical excision; however, lymph node or distant metastases are often already present by the time of the diagnosis. Since an immune checkpoint inhibitor therapy with avelumab was first approved for MCC in 2016, there has been considerable improvement in mean survival compared to cytostatic therapy; at the same time, there has been a reduction in serious treatment-associated adverse events. Other immune checkpoint inhibitors are currently still in clinical trials, with very promising initial results. Because of the complexity of the diagnosis, treatment, and prognosis, it is essential that MCC patients receive interdisciplinary care in a specialized center including consultation with a tumor review board.

Schlüsselwörter

Immuncheckpointinhibitor - Merkelzell-Polyomavirus - PD-1 - PD-L1 - Karzinom des Augenlids

Keywords

Immune checkpoint inhibitor - Merkel cell polyoma virus - PD-1 - PD-L1 - Carcinoma of the eyelid

Publikationsverlauf

Eingereicht: 30. Juni 2022

Angenommen: 16. August 2022

Artikel online veröffentlicht:
21. Dezember 2023

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