Overview

NCI Definition: An undifferentiated soft tissue sarcoma characterized by the presence of a pleomorphic malignant cellular infiltrate. It is also known as malignant fibrous histiocytoma. [1]

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

Most Commonly Altered Genes in Undifferentiated Pleomorphic Sarcoma

TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 c.142-c.212 Missense, TP53 c.1-c.137 Missense, and TP53 Missense are the most common alterations in undifferentiated pleomorphic sarcoma [2].

Top Alterations in Undifferentiated Pleomorphic Sarcoma

Significant Genes in Undifferentiated Pleomorphic Sarcoma

PIK3CA +

PTEN +

Disease Details

Synonyms
Malignant Fibrous Histiocytoma of the Soft Tissue and Bone, malignant fibrous cytoma, Unclassified Pleomorphic Sarcoma (Formerly "Malignant Fibrous Histiocytoma"), MFH, Malignant Fibroxanthoma, Storiform-Pleomorphic Malignant Fibrous Histiocytoma, Malignant Fibrous Histiocytoma, Fibroxanthosarcoma, Storiform-Pleomorphic MFH, UPS, Storiform-Pleomorphic Fibrous Histiocytoma, Unclassified Pleomorphic Sarcoma (Formerly "MFH"), HISTIOCYTOMA, FIBROUS, MALIGNANT, Unclassified Pleomorphic Sarcoma, Malignant Fibrous Histiocytoma of Soft Tissue and Bone, Undifferentiated Pleomorphic Soft Tissue Sarcoma
Parent(s)
Undifferentiated Sarcoma
Children
Cutaneous Undifferentiated Pleomorphic Sarcoma, Undifferentiated Pleomorphic Sarcoma, Inflammatory Variant, Undifferentiated High Grade Pleomorphic Sarcoma of Bone, and Undifferentiated Pleomorphic Sarcoma with Osteoclast-Like Giant Cells
OncoTree Name
Undifferentiated Pleomorphic Sarcoma/Malignant Fibrous Histiocytoma/High-Grade Spindle Cell Sarcoma
OncoTree Code
MFH

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.