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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Cancer Res Commun
January 2025
University of British Columbia, Vancouver, BC, Canada.
NF1 encodes the multifunctional tumour suppressor protein, neurofibromin, which is best known for its causative role in Neurofibromatosis type 1 and in regulating MAPK signaling. Neurofibromin, in a context-specific manner, is involved in various tumorigenic processes, including those in melanocytes. This study investigated whether NF1 loss can collaborate with oncogenic GNAQ to promote melanoma in the dermis or eyes, where the G alpha q pathway is almost always activated.
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Department of Anesthesiology, Shenzhen Children's Hospital, Yitian Road 7019, Shenzhen, 518000, China.
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