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, CDKN2A, ATRX, RB1, and CDKN2B [2].

Most Commonly Altered Genes in Undifferentiated Pleomorphic Sarcoma

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

Top Alterations in Undifferentiated Pleomorphic Sarcoma

Significant Genes in Undifferentiated Pleomorphic Sarcoma

AKT1 +

AKT2 +

AKT3 +

CRKL +

EGFR +

ERBB2 +

IRS2 +

KIT +

MET +

MLH1 +

MLH3 +

MSH2 +

MSH3 +

MSH6 +

MTOR +

MYCN +

PDGFRA +

PIK3CA +

PIK3CG +

PIK3R1 +

PIK3R2 +

PMS1 +

PMS2 +

PTEN +

RICTOR +

RNF43 +

RPTOR +

TSC1 +

TSC2 +

Disease Details

Synonyms
Malignant Fibrous Histiocytoma of the Soft Tissue and Bone, Storiform-Pleomorphic MFH, Malignant Fibrous Histiocytoma of Soft Tissue and Bone, UPS, malignant fibrous cytoma, Undifferentiated Pleomorphic Soft Tissue Sarcoma, Unclassified Pleomorphic Sarcoma, Malignant Fibrous Histiocytoma, Fibroxanthosarcoma, Storiform-Pleomorphic Fibrous Histiocytoma, MFH, Unclassified Pleomorphic Sarcoma (Formerly "MFH"), Storiform-Pleomorphic Malignant Fibrous Histiocytoma, Unclassified Pleomorphic Sarcoma (Formerly "Malignant Fibrous Histiocytoma"), Malignant Fibroxanthoma, HISTIOCYTOMA, FIBROUS, MALIGNANT
Parent(s)
Undifferentiated/Unclassified Sarcoma
Children
Undifferentiated Pleomorphic Sarcoma with Osteoclast-Like Giant Cells, Undifferentiated High Grade Pleomorphic Sarcoma of Bone, Malignant Fibroxanthoma, Undifferentiated Pleomorphic Sarcoma, Inflammatory Variant, and Cutaneous Undifferentiated Pleomorphic Sarcoma
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 6. 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.