Malignant histiocytosis in the oropharynx

Malignant histiocytosis is characterized by systemic, progressive, and invasive proliferation of malignant histiocytes. The disorder is typically accompanied by fever, general fatigue, lymphadenopathy, and hepatosplenomegaly. A case of a 21-year-old woman with primary malignant histiocytosis of the oropharynx is reported. Histologic diagnosis from the biopsy specimen was confused by infiltration of normal-appearing histiocytes and inflammatory cells. The titers of Epstein-Barr virus and the Paul-Bunnell test were elevated without atypical lymphocytes. The patient died 3.5 months after the onset of symptoms. Autopsy revealed systemic neoplastic proliferation of malignant histiocytes. A review of literature on this subject revealed no cases of malignant histiocytosis primarily involving the oropharynx.