Overview

NCI Definition: An uncommon, aggressive malignant smooth muscle neoplasm, usually occurring in post-menopausal women. It is characterized by a proliferation of neoplastic spindle cells. Morphologic variants include epithelioid, granular cell, inflammatory and myxoid leimyosarcomas. [1]

Leiomyosarcomas most frequently harbor alterations in TP53, RB1, ATRX, PTEN, and MAP2K4 [2].

Most Commonly Altered Genes in Leiomyosarcoma

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

Top Alterations in Leiomyosarcoma

Significant Genes in Leiomyosarcoma

ARID1A +

ATM +

ATR +

ATRX +

BARD1 +

BRCA1 +

BRCA2 +

BRIP1 +

CCNE1 +

CDK12 +

CHEK1 +

CHEK2 +

ERBB2 +

FANCA +

FANCB +

FANCC +

FANCD2 +

FANCE +

FANCF +

FANCG +

FANCI +

FANCL +

FANCM +

FBXW7 +

MCPH1 +

MRE11A +

MYC +

MYCN +

NBN +

PALB2 +

PIK3CA +

PTEN +

RAD50 +

RAD51 +

RAD51B +

RAD51C +

RAD51D +

RAD54L +

SDHA +

SDHAF2 +

SDHB +

SDHC +

SDHD +

SLX4 +

Disease Details

Synonyms
Leiomyosarcoma (excluding uterine leiomyosarcoma), Unknown primary leiomyosarcoma, Soft tissue leiomyosarcoma, LEIOMYOSARCOMA, MALIGNANT, Leiomyosarcoma - not uterine, Leiomyosarcomas
Parent(s)
Sarcoma
Children
Bone Leiomyosarcoma, Cardiac Leiomyosarcoma, Extrahepatic Bile Duct Leiomyosarcoma, Inflammatory Leiomyosarcoma, Granular Cell Leiomyosarcoma, Vaginal Leiomyosarcoma, Pulmonary Vein Leiomyosarcoma, Colorectal Leiomyosarcoma, Vulvar Leiomyosarcoma, Leiomyosarcoma of Central Nervous System, Lung Leiomyosarcoma, Prostate Leiomyosarcoma, Breast Leiomyosarcoma, Ovarian Leiomyosarcoma, Anal Leiomyosarcoma, Fallopian Tube Leiomyosarcoma, Mediastinal Leiomyosarcoma, Gastric Leiomyosarcoma, Small Intestine Leiomyosarcoma, Superior Vena Cava Leiomyosarcoma, Esophageal Leiomyosarcoma, Retroperitoneal Leiomyosarcoma, Laryngeal Leiomyosarcoma, Conventional Leiomyosarcoma, Cutaneous Leiomyosarcoma, Pulmonary Artery Leiomyosarcoma, Epithelioid Leiomyosarcoma, Uterine Corpus Leiomyosarcoma, Gallbladder Leiomyosarcoma, Kidney Leiomyosarcoma, Liver Leiomyosarcoma, Myxoid Leiomyosarcoma, and Cervical Leiomyosarcoma
OncoTree Name
Leiomyosarcoma
OncoTree Code
LMS

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.