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.
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