Overview

NCI Definition: A liposarcoma characterized by the presence of varying proportions of pleomorphic lipoblasts in a background that resembles undifferentiated pleomorphic sarcoma. It is the rarest liposarcoma variant and usually has an aggressive clinical course. [1]

Pleomorphic liposarcomas most frequently harbor alterations in TP53, RB1, ATRX, CYSLTR2, and SMARCA4 [2].

Most Commonly Altered Genes in Pleomorphic Liposarcoma

TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 Missense, RB1 Loss, and TP53fs are the most common alterations in pleomorphic liposarcoma [2].

Top Alterations in Pleomorphic Liposarcoma

Significant Genes in Pleomorphic Liposarcoma

PIK3CA +

PTEN +

Disease Details

Parent(s)
Liposarcoma
Children
Myxoid Pleomorphic Liposarcoma
OncoTree Name
Pleomorphic Liposarcoma
OncoTree Code
PLLS

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.