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, ATRX, CDKN2B, and NOTCH1 [2].

Most Commonly Altered Genes in Myxofibrosarcoma

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

Top Alterations in Myxofibrosarcoma

Significant Genes in Myxofibrosarcoma

PIK3CA +

PTEN +

Disease Details

Synonyms
Myxoid Fibrous Histiocytoma, 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 4. 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.