Bednar tumors are rare pigmented variants of dermatofibrosarcoma protuberans (DFSP), accounting for less than 5% of DFSP cases. We report a rare case of a congenitally pigmented lesion that progressively enlarged during pregnancy and developed pain. A 32-year-old woman presented with a blue-black nodule on her right shoulder, which had been present since birth and was initially misdiagnosed as a blue nevus. Histopathological analysis revealed spindle cell proliferation in a storiform pattern with focal melanin pigment. Immunohistochemistry showed strong CD34 positivity and negative S100 staining, consistent with Bednar tumors. This case highlights the importance of considering Bednar tumors in the differential diagnosis of congenital pigmented lesions, particularly those showing interval growth or symptoms.
Chen et al. (Thu,) studied this question.