A 52-year-old woman was admitted with a palpable mass on the abdominal wall. This tumor had developed at the same site of a previously resected dermatofibrosarcoma protuberans. The biopsy specimen from the recurrent tumor revealed malignant fibrous histiocytoma. Immunohistochemistry with anti-CD34 antibody revealed diffuse linear positivity on the tumor cells of dermatofibrosarcoma protuberans and negativity on malignant fibrous histiocytoma. Anti CD34 antibody was valuable for a differential diagnosis of these two lesions.
Dermatofibrosarcoma protuberans (DFSP) typically presents during the early or mid-adult life, and the most common site of origin is the skin on the trunk and proximal extremities.
DFSP of the parotid gland is extremely rare and only one case has been reported in the literature. We present here a case of a 30-year-old woman with DFSP occurring in the parotid gland, and we discuss the differential diagnosis.
The patient is alive and doing well one year after her operation.
The authors examined a case of Bednar tumor(pigmented dermatofibrosarcoma protuberans). A 35-year-old woman presented with protruding black back mass 3x2.5 cm in surface dimension and 1 cm in heigh, which has grown slowly for 30 years. Microscopically the lesion is characterized by spindle cells arranged in storiform pattern and admixed with melanin containing dendritic cells. Ultrastructural study revealed three cell populations: (1) cells resembling fibroblasts, (2) cells resembling perineural cells, and (3) melanocytes. The immunohistochemical study using S-100 protein alpha-1 antitrypsin, antichymotrypsin and EMA revealed negative reaction in spindle fibroblastic tumor cells. The histogenesis of this neoplasm remains controversial; neuroectoderm, perineural cell, fibroblast and histiocyte. Through the ultrastructural study and immunohistochemical study, the authors suggested that Bedner tumor arises form the neuroectoderm with bidirectional to perineural and fibroblastic differentiation.