Abstract
Histiocytic sarcoma is a rare neoplasm of the lymphoreticular system. Cells show both morphologic and immunophenotypic features of mature tissue histiocytes. The gastrointestinal tract is the most commonly involved, but other sites include skin, soft tissue and lymph nodes. Tumours tend to present at an advanced stage and show an aggressive clinical course. We detail a case of histiocytic sarcoma in a 67-year-old lady, who initially presented due to difficulty fitting her dentures. Imaging revealed a solitary, destructive mass extending from the palate to the orbit. Biopsy, and later maxillectomy, showed a pleomorphic tumour cell population, with abundant eosinophilic cytoplasm and vesicular nuclei. Prominent emperipolesis was noted. Cells were positive for histiocytic markers by immunohistochemistry. Interestingly, the tumour also showed clonal T cell receptor gene rearrangement in one of two sets of PCR primers. The rarity of these tumours may pose some challenges forthe reporting pathologist, and we discuss the other potential differentials in the work up of a histiocytic rich neoplasm.
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