Histiocytic sarcoma

Last revised by Frank Gaillard on 21 Oct 2021

Histiocytic sarcoma is a rare malignant hematopoietic neoplasm that has been reported in association with other hematological malignancies (particularly B and T cell lymphomas).

It comprises of tumor cells derived from the monocyte/macrophage lineage.

It usually occurs in lymph nodes, skin, and the gastrointestinal tract. Occurrence in the CNS system has also been rarely reported 3.

The overall prognosis is generally poor due to rapid progression and poor response to therapy. Tumor size may be a prognostic factor.

It was first described by Mathé et al. in 1970.

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