Dendritic cell sarcoma, not otherwise specified (NOS), is an entity that is poorly defined because of its rarity and poorly understood pathogenesis. It is characterized by positive immunohistochemical staining for S-100 and CD1a, along with an absence of cytoplasmic Birbeck granules on electron microscopy. We report the case of a surgically inaccessible nasopharyngeal dendritic cell sarcoma, NOS, in a 34-year-old man. Treatment with chemotherapy along with adjuvant radiation therapy was successful in decreasing the size of the nasopharyngeal mass, and the patient remained free of any evidence of recurrence nearly 5 years after treatment.
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