Overview

NCI Definition: A malignant fibroblastic neoplasm arising from the soft tissue. It is characterized by the presence of spindle-shaped cells, cellular pleomorphism, thin-walled blood vessels, fibrous septa, and myxoid stroma. [1]

Myxofibrosarcomas most frequently harbor alterations in TP53, CDKN2A, CDKN2B, ATRX, and RB1 [2].

Most Commonly Altered Genes in Myxofibrosarcoma

CDKN2A Loss, CDKN2B Loss, TP53 Mutation, TP53 Loss, and TP53 c.217-c.1178 Missense are the most common alterations in myxofibrosarcoma [2].

Top Alterations in Myxofibrosarcoma

Significant Genes in Myxofibrosarcoma

ERBB2 +

PIK3CA +

PTEN +

Disease Details

Synonyms
Myxoid Fibrous Histiocytoma, High-grade myxofibrosarcoma, Myxofibrosarcoma, high-grade, Soft tissue myxofibrosarcoma, Myxoid Malignant Fibrous Histiocytoma, Myxoid MFH
Parent(s)
Soft Tissue Fibrosarcoma
OncoTree Name
Myxofibrosarcoma
OncoTree Code
MFS

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.