Primary mucosal melanoma of the larynx: systematic review of the literature and qualitative synthesis

Purpose Primary mucosal melanoma of the larynx (PLM) is a rare entity among head and neck cancers. Due to its rarity, clear protocols of management are not available. A deeper knowledge of the clinical and biological behaviour of PLM is strongly needed. Methods According to PRISMA process, we searched through electronic databases case reports, case series and review articles providing relevant clinical data. The survival analysis was performed with Kaplan-Meier survival curves, using disease free survival (DFS) and overall survival (OS) as endpoints. Results 1074 articles were initially screened, of which 37 studies describing 44 PLM cases were selected and included in the analysis. Mean age was 59.7 years with a mean follow-up time of 25.4 months. The most common symptom at presentation was hoarseness (52%), while the most involved laryngeal subsite was supraglottic region (62%). Most patients presented with an advanced stage. Tumour (T) and node (N) status at presentation did not influence OS nor DFS, whereas distant metastases (M) status resulted significantly associated with the reduction of OS and DFS time (Mantel-Cox: p < 0.0001 and p = 0.001, respectively). The laryngeal subsite and the type of surgery performed did not significantly impact on OS and DFS. Conclusions Treatment for PLM remains debated. Surgery with safe margins is recommended due to the high rates of local recurrence. Systemic therapy is advised for metastatic disease. However, the prognosis remains poor even after radical resection or targeted therapy.