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Histiocytic sarcoma

Eric Jacobsen, MD
Section Editor
Arnold S Freedman, MD
Deputy Editor
Alan G Rosmarin, MD


Histiocytic sarcoma (HS) is an extremely rare non-Langerhans histiocyte disorder of unknown cause that most commonly presents with symptoms due to unifocal or multifocal extranodal tumors. HS may occur as a sporadic illness or may be clonally related to a separate synchronous or metachronous hematologic malignancy, such as follicular lymphoma or acute lymphoblastic leukemia.

Histocytic disorders are thought to be derived from mononuclear phagocytic cells (macrophages and dendritic cells) or histiocytes. This group has generally been divided into Langerhans cell histiocytosis and non-Langerhans histiocytosis. Langerhans cell histiocytosis is so named for its presumed derivation from the Langerhans cells, which are specialized dendritic cells found in the skin and mucosa. In contrast, non-Langerhans histiocytoses are thought to be derived from the monocyte-macrophage lineage.

The epidemiology, clinical manifestations, pathologic features, diagnosis, and management of HS will be presented here. The diagnosis and management of Langerhans cell histiocytosis and other non-Langerhans histiocytic disorders are presented separately. (See "Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis" and "Pulmonary Langerhans cell histiocytosis" and "Langerhans cell histiocytosis (eosinophilic granuloma) of bone in children and adolescents" and "Erdheim-Chester disease".)


HS is an extremely rare disease with only a few hundred cases reported in the literature. HS has been diagnosed in all age groups, but is most common in adults. In two case series, the median ages at diagnosis were 46 and 55 years [1,2]. There is no apparent gender difference.

There are no known environmental or hereditary genetic factors predisposing to the development of HS. HS can occur as an isolated disease or in the context of other hematologic neoplasms, such as follicular lymphoma, myelodysplasia, or acute lymphoblastic leukemia [2]. When HS occurs in the context of another hematologic malignancy, the two entities are often clonally related [3,4]. Of importance, tumors with pathologic features consistent with HS occurring in the context of acute monocytic leukemia are not considered HS.

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Literature review current through: Nov 2017. | This topic last updated: Oct 28, 2016.
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