Amelanotic melanoma of the nasal cavity: a rare entity

Mucosal melanomas of the head and neck are a rare and aggressive tumor entity. Both clinical and histopathological diagnostics, as well as the treatment of these malignancies, continue to pose a major challenge. About 40% of mucosal melanomas are amelanotic, compared to less than 10% amelanotic cutaneous melanomas. In the nasal cavity and the paranasal sinuses, primary surgery is currently the therapeutic gold standard, often supplemented by controversial adjuvant radiotherapy or immunotherapy. A 76-year-old patient presented to our clinic due to progressive right-sided nasal obstruction, with occasional unilateral epistaxis and rhinorrhea. We detected a polypous tumorous lesion of the right nasal septum. A CT-scan showed a big formation in the right nasal cavity. Endoscopic resection of the formation was performed. The histopathological assessment revealed an amelanotic melanoma. In the absence of evidence of distant metastasis in staging, we performed an extensive tumor resection and right-sided selective neck dissection. Subsequently, in case of BRAF-negativity, adjuvant therapy with Nivolumab was administered. The Patient developed a local tumor recurrence, so we performed a second radical resection and initiated a postoperative radiotherapy to improve regional tumor control. Currently, the patient is in close tumor aftercare. On the basis of this case, we would like to demonstrate the special features of this rare tumor entity and, in the absence of uniform therapeutic standards, despite the existing S3-guideline, encourage the discussion of therapy concepts.

Publication History

Article published online:
12 May 2023