Interdigitating dendritic cell sarcoma (IDCS) is a malignant neoplasm that arises from the interdigitating reticular cells of the lymph node. Dendritic cells are a heterogeneous group of antigen-presenting cells that play a major role in innate and adaptive immune responses. Subtypes of dendritic cells include Langerhans cells, interdigitating dendritic cells, follicular dendritic cells, and dermal dendrocytes.1 Interdigitating dendritic cells reside in the T-cell areas of peripheral lymphoid tissue, including the paracortex of lymph node and tonsil, the splenic periarteriolar lymphoid sheaths, and the interfollicular areas of mucosa-associated lymphoid tissue, and are responsible for major histocompatibility complex–restricted stimulation of resting T cells. Interdigitating dendritic cell sarcoma is rare; to our knowledge, only 34 cases have been reported to date.1- 4 Lymphadenopathy is the most common physical finding at presentation, and 42% of cases occur in the head and neck.1,2,4 Otolaryngologists evaluating a head and neck mass should therefore include IDCS in the differential diagnosis.
Photomicrograph shows population of normal-appearing lymphocytes (right side) and large oval and polygonal tumor cells (left side) with atypical-appearing nuclei.
Photomicrograph shows immunohistochemically stained cells positive for S100 protein.
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