Primary paraganglioma-like dermal melanocytic tumor (PDMT) is a rare dermal melanocytic tumor first recognized in 2004. The cases in previous reports suggested a benign course with a favorable prognosis. Herein, we describe a case of PDMT in the right neck from a 33-year-old man. The tumor cells with mild atypia and low level mitosis consisted of epithelioid cells or spindle cells and were divided into organoid or nest-like structures by fibrous strands and blood vessels, resembling a paraganglioma growth pattern. No necrosis was seen. These cells were positive for HMB45, Melan A, and S-100 with < 5% cells positive for Ki67. Although the morphology conformed to benign features, it recurred after 2 years. In conclusion, while more data are needed to confirm the biologic behavior of this tumor, at least a low malignant potential cannot be excluded.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6958257PMC

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