Primary gallbladder melanoma: A systematic review of literature

Primary gallbladder melanoma (PGM) is a rare malignancy with only sporadic cases reported in the English literature. We performed a systematic review of the cases published in the PubMed, Science Direct and Google Scholar databases with the aim of describing the reported clinicopathologic features of PGM. Thirty-six articles reporting on 39 patients were reviewed. There was a male predominance, with 23 (64 %) of 36 patients being males. The mean age at presentation was 55 +/- 16 years. Pain in the right upper quadrant was reported in 20/27 (74 %). The average size of the tumor was 3.5 x 1.9 x 1.4 cm. Gallbladder calculi were reported in 7/27 (26 %). A cholecystectomy was performed in 34/38 (89.5 %). Grossly, the tumor mostly (96.5 %) had polypoid appearances and on microscopic examination, the tumor were predominantly comprised of epithelioid cells 12/17 (70.6 %). Mitotic figures and prominent nucleoli were reportedly found in 8/8 (100 %) and 3/3 (100 %) respectively. Junctional melanocytic components were present in 13/21 (61.9 %). Tumor cells were reportedly immunoreactive for S-100 and HMB-45 in all tested cases. Metastasis were reported in 25/36 (69.4 %), with lymph nodes being the most common site (n = 8), followed by brain (n = 6) and liver (n = 4) for metastasis. At a mean follow-up period of 19 +/-3 months, 16 (48.5 %) of the 33 patients with available survival data were alive and 17/33 (51.5 %) were dead of disease. There is a lack of unified criteria for the diagnosis of PGM, and future studies should aim to resolve this.