Diagnostik und Therapie von Schleimhautmelanomen der Nase/Nasennebenhöhlen

Das sinunasale Schleimhautmelanom (SNSM) ist ein sehr selten vorkommendes Malignom [1], bei dem standardmäßig eine radikale chirurgische Tumorresektion und eine adjuvante Strahlentherapie durchgeführt werden. Die Rate an Lokalrezidiven ist hoch, und die Mehrheit der Patienten entwickelt Fernmetastasen. Beides trägt zu der sehr schlechten Prognose der Erkrankung bei. Immuncheckpoint-Inhibitoren konnten bisher vielversprechende Ergebnisse erzielen.

Abstract

Sinonasal mucosal melanoma (SNMM) is a rare and aggressive disease representing only 4% of all sinonasal malignancies and 1.4% of all melanomas. With an incidence of approximately 0.2 to 2 cases per million, the disease represents a very rare cancer type. As a result, there is a lack of data and most of the evidence for this highly aggressive disease is based on retrospective observations and analyses. The standard of care is radical tumor resection followed by an adjuvant radiotherapy. Nevertheless, the rate of local recurrence is high, up to 50%. In addition, the majority of patients (up to 70%) develop distant metastases during the course of their disease. Both contribute to the extremely poor prognosis of the disease. Mucosal melanomas (SM) and cutaneous melanomas (CM) behave differently with respect to biology, clinic presentation and prognosis. Compared to CM, survival rates are significantly lower for SM. The 5-year survival rate is around 25% in SNMM but 39–97% in cutaneous melanoma. Similar to CM, immune checkpoint inhibitors achieve promising results in SM. However, response rates are lower in SM compared to CM. The goal of this CME article is to provide an overview on biology, diagnosis, therapy, and prognosis of SNMM.

Kernaussagen

• Das sinunasale Schleimhautmelanom (SNSM) ist eine sehr seltene und äußerst aggressive Erkrankung.

• Das SNSM unterscheidet sich hinsichtlich Epidemiologie, Ätiologie, Prognose und molekularer Merkmale erheblich vom kutanen Melanom (CM). So ist das 5-Jahres-Überleben beim SNSM mit ca. 25% deutlich schlechter als beim CM.

• Die Therapie der Wahl beim SNSM besteht aus der chirurgischen Tumorresektion gefolgt von einer adjuvanten Strahlentherapie. Eine Neck Dissection erfolgt nur bei klinisch oder radiologisch suspekten Lymphknoten.

• In der Nachsorge ist aufgrund der hohen Rate an Lokalrezidiven in bis zu 50% der Fälle die HNO-ärztliche Untersuchung einschließlich der Nasenendoskopie von äußerster Wichtigkeit. Etwa 10% der Patienten mit SNSM weisen bereits bei der Diagnose Fernmetastasen auf, bis zu 70% entwickeln im Verlauf der Erkrankung Fernmetastasen. Aus diesem Grund sollten jährliche radiologische Kontrolluntersuchungen erfolgen.

• Zentrale Bedeutung in der palliativen Therapie des SNSM spielen Immuncheckpoint-Inhibitoren. Zudem sollten molekulargenetische Untersuchungen ergänzt werden, um das Vorliegen von medikamentös targetierbaren genetischen Veränderungen zu prüfen.

Schlüsselwörter

Melanom - Schleimhaumelanom - Nasennebenhöhlen - Mutation - Immuncheckpoint-Hemmung

Keywords

Melanoma - mucosal melanoma - paranasal sinus - mutation - immune checkpoint inhibition

Publication History

Article published online:
05 January 2024

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