Schleimhautmelanome des Kopf-Hals-Bereichs

J. Thierauf; J. Veit; J. Döscher; M.-N. Theodoraki; J. Greve; T. K. Hoffmann

doi:10.1055/s-0042-105064

TumorDiagnostik & Therapie, Table of Contents

TumorDiagnostik & Therapie 2016; 37(03): 143-149
DOI: 10.1055/s-0042-105064

Thieme Onkologie aktuell

Schleimhautmelanome des Kopf-Hals-Bereichs

Mucosal melanoma of the head and neck

J. Thierauf

,

J. Veit

,

J. Döscher

,

M.-N. Theodoraki

,

J. Greve

,

T. K. Hoffmann

Abstract

Zusammenfassung

Mukosale Melanome des Kopf-Hals-Bereichs stellen sehr seltene, wenig erforschte Malignome dar, welche sich durch ein aggressives Wachstum, eine hohe Rate an lokoregionären Rezidiven und eine schlechte Prognose charakterisieren lassen. Bisher konnten keine Risikofaktoren für deren Entstehung identifiziert werden. Der Tumor tritt klassischerweise erst in fortgeschrittenem Stadium durch Symptome wie Epistaxis nasi oder nasale Obstruktion in Erscheinung und ist per Definition mindestens im T3 Stadium vorliegend. Der Goldstandard besteht in der radikalen Tumorresektion, welche bei knappen oder non in-sano Resektionen durch eine adjuvante Radiotherapie ergänzt wird. Die Anwendung klassischer Chemotherapeutika, Interferon oder zielgerichteter Antikörper konnte bislang zu keiner signifikanten Verbesserung der Prognose beitragen. Bemühungen, den Tumor besser biologisch und genomisch zu charakterisieren werden nach wie vor durch dessen geringe Inzidenz erschwert. Dennoch ist die molekulare Charakterisierung dieser prognostisch und therapeutisch klar vom kutanen Melanom abzugrenzenden Tumorentität dringend erforderlich, um innovative Therapiestrategien zu entwickeln, was wiederum interdisziplinärer und multizentrischer Anstrengungen bedarf.

Abstract

Primary mucosal malignant melanoma of the head and neck is a rare tumor entity with poor clinical outcome. Its growth pattern is characterized by an infiltrative and local destructive behavior. So far no risk factors could be identified. There are practically no early symptoms of the disease, as intermitting nose bleeding or nasal obstruction typically occur in advanced stage. The standard of care remains radical tumor resection with adjuvant radiation in cases of close margin resection. Other therapeutic options like the use of interferon, antibodies or conventional chemotherapeutics have not demonstrated significant clinical benefit so far. Current efforts to investigate the biological and genomic characteristics of these tumors have been constrained by its low incidence. In order to better characterize this rare tumor entity and to establish effective novel targeted therapies it will be necessary to establish an interdisciplinary and multicentric task force.

Schlüsselwörter

Schleimhautmelanom - molekulare Marker - Kopf-Hals-Region - Immuntherapie - Onkogene

Key words

mucosal melanoma - target therapy - head and neck

Full Text

References

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