A Case of Histiocytic Sarcoma Arising in Head and Neck Region with Rhabdoid Differentiation / 대한이비인후과학회지

Head and neck sarcomas account for about 1% of malignant head and neck tumors, and histiocytic sarcomas (HSs) account for less than 1% of all hematolymphoid neoplasms. These rare hematopoietic neoplasms are the result of a malignant proliferation of cells. They have morphological and immunophenotypic features of mature tissue histiocytes, which play a major role in processing and presenting antigens to T or B lymphocytes. The mesenchymal differentiation of HSs has not been reported. We describe a case of HS with rhabdoid differentiation in a 55-year-old man who presented with a 3×2.5×2 cm mass in the left supraclavicular area. To the best of our knowledge, this is the first report worldwide of a primary HS with rhabdoid differentiation.