A rare histiocytic tumor characterized by a malignant proliferation of cells showing morphological and immunophenotypic features of mature tissue histiocytes. Most cases occur in extranodal sites most commonly the intestinal tract skin and soft tissue. Patients may present with a solitary mass lymphadenopathy a skin rash or numerous tumors on the trunk and extremities lytic bone lesions hepatosplenomegaly with pancytopenia intestinal obstruction and/or systemic symptoms. The neoplasm is aggressive with typically poor therapy response.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version May 2026
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Histiocytic sarcoma?
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