Background and Objectives: The respiratory apparatus, generally affected by highly aggressive tumors like lung cancer and mesothelioma, is rarely affected by primary malignant melanoma. The aim of this review was to identify cases of primary malignant melanoma of the lung (PMML) published in the modern scientific literature, and to describe their main clinical, pathological and therapeutic features. Materials and Methods: A systematic search of publications in the electronic database PubMed has been performed using keywords, and the references of the selected articles were checked to identify additional missing studies. Results: Globally 52 papers reporting on 76 cases were identified. Among them there were 47 reports of a single case, three papers reporting on two cases each, and two larger case series published in 1997 and 2005 including eight and 15 cases, respectively. Conclusions: PMML was generally diagnosed in middle-aged males, without any apparent correlation with cigarette smoking. It was more frequently found in the lower lobes and the left lung. The tumors were generally pigmented, composed by epithelial and/or spindle cells with large nuclei and prominent nucleoli, nuclear atypia, and numerous mitotic figures; they commonly showed immunostaining for S-100, HMB 45 and Melan-A. Early detection and surgical resection were the main determinants of survival from this rare malignancy.
Keywords: BRAF; HMB; cancer; lung; malignant melanoma; melan-A.