Overview

NCI Definition: An aggressive malignant neoplasm with a poor response to therapy, usually presenting as stage III/IV disease. It is characterized by the presence of neoplastic cells with morphologic and immunophenotypic characteristics similar to those seen in mature histiocytes. [1]

Histiocytic sarcomas most frequently harbor alterations in BCOR, TP53, SETD2, RARA, and PTPN11 [2].

Most Commonly Altered Genes in Histiocytic Sarcoma

BCOR Mutation, TP53 c.217-c.1178 Missense, TP53 Mutation, TP53 Missense, and TP53 L130F are the most common alterations in histiocytic sarcoma [2].

Top Alterations in Histiocytic Sarcoma

Significant Genes in Histiocytic Sarcoma

ATM +

BRAF +

BRCA1 +

BRCA2 +

RAD51C +

RAD51D +

Disease Details

Synonyms
SARCOMA, HISTIOCYTIC, MALIGNANT
Parent(s)
Non-langerhans-cell Histiocytosis
OncoTree Name
Histiocytic Sarcoma
OncoTree Code
HS

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.