Mucosal melanoma at the maxillar sinus– a rare disease

Background Mucosal melanoma is a rare disease, accounting 1.4 % of all melanomas and only 0.03 % of all new cancer diagnoses. 55 % of cases appear at the oral cavity, nasal cavity or sinuses. The only potentially curative option is a complete surgical excision. Radiotherapy improve local control, but there is no evidence it improves overall survival. Options for systemic therapy include immunotherapies and targeted treatments.

Case report A 71-year old man presented with the clinical picture of a ipsilateral chronic sinusitis and a protusion bulbi at the right side. The clinical examination showed a homogeneous tissue formation in the nasal cavity. The CT imaging revealed tumorous mass in the ethmoid bone, the maxillar sinus and partial in the right frontal sinus and the sphenoid sinus with destruction of the lamina papyracea and protrusion intraorbital and infiltration of the masticator space. There was shown a higher FDG accumulation in the whole-body PET/CT Imaging with Fluorodeoxyglucose. The rapid section examination revealed the cell picture of a malignant mucosal melanoma which was positiv for Ki-67, SOX-10 and HMB 45. In the gene sequencing a BRAF or c-KIT mutation was not detected. A complete surgical excision was impossible. A radiotherapy and the induction of specific immunotherapy with ipilimumab and nivolumab were recommended.

Conclusion Timely diagnosis can be complicated by the rarity of the tumor and the frequently occult site of origin, The only potentially curative option is complete surgical excision with negative margins. Clinical trials for patients with mucosal melanoma remain a key priority.

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Publication History

Article published online:
10 June 2020