Primary mucosal melanomas of the head and neck

Mucosal melanomas of the head and neck are a rare disease of dismal prognosis. They account for less than 1 % of all cancers, less than 4 % of all melanomas, and more than half of all mucosal melanomas. Five-year survival is about 20-30 %, mainly because of deaths from distant metastases. Mucosal melanomas of the head and neck deserve central pathology reviews and case discussions within multidisciplinary rare disease networks, aware of all the diagnostic and therapeutic challenges. Early diagnosis followed by surgical excision remains the mainstay of treatment, and postoperative radiation therapy is often recommended. Current controversies include the role of minimal invasive endoscopic approaches, their challenges in terms of margin assessment, and radiation therapy dose. As for the modalities of radiation therapy, the current level of evidence pleads in favor of optimized tridimensional conventionally fractionated radiation therapy. However, technological advances suggest that carbon therapy might be preferred to proton therapy because of an expected better biological efficacy and charged particle therapy (proton or carbon therapy) to photon-based irradiation because of dose distributions and safer ability toward hypofractionation. The domain of medical oncology and genetic research may provide further clues, especially in light of benefits noted of KIT inhibitors and the proportion of mucosal melanomas with this genetic aberration. Adequate methodology needs to be developed for rare diseases with specific therapeutic challenges, and patient associations, patient-reported outcomes, and quality-adjusted life-years (QALY) should be part of the assessment of the treatment modalities.