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, MAP2K4, and PTEN [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 +

MLH1 +

MLH3 +

MRE11A +

MSH2 +

MSH3 +

MSH6 +

MYC +

MYCN +

NBN +

PALB2 +

PIK3CA +

PMS1 +

PMS2 +

PTEN +

RAD50 +

RAD51 +

RAD51B +

RAD51C +

RAD51D +

SDHA +

SDHAF2 +

SDHB +

SDHC +

SDHD +

SLX4 +

Disease Details

Synonyms
Leiomyosarcomas, LEIOMYOSARCOMA, MALIGNANT, Leiomyosarcoma (excluding uterine leiomyosarcoma), Leiomyosarcoma - not uterine
Parent(s)
Malignant Smooth Muscle Neoplasm
Children
Leiomyosarcoma of Central Nervous System, Myxoid Leiomyosarcoma, Extrahepatic Bile Duct Leiomyosarcoma, Rectal Leiomyosarcoma, Conventional Leiomyosarcoma, Bone Leiomyosarcoma, Epithelioid Leiomyosarcoma, Kidney Leiomyosarcoma, Laryngeal Leiomyosarcoma, Vaginal Leiomyosarcoma, Retroperitoneal Leiomyosarcoma, Colon Leiomyosarcoma, Gallbladder Leiomyosarcoma, Cervical Leiomyosarcoma, Inflammatory Leiomyosarcoma, Breast Leiomyosarcoma, Small Intestine Leiomyosarcoma, Gastric Leiomyosarcoma, Cardiac Leiomyosarcoma, Cutaneous Leiomyosarcoma, Ovarian Leiomyosarcoma, Vulvar Leiomyosarcoma, Pulmonary Vein Leiomyosarcoma, Liver Leiomyosarcoma, Granular Cell Leiomyosarcoma, Uterine Corpus Leiomyosarcoma, Esophageal Leiomyosarcoma, Lung Leiomyosarcoma, Pulmonary Artery Leiomyosarcoma, Fallopian Tube Leiomyosarcoma, Mediastinal Leiomyosarcoma, Superior Vena Cava Leiomyosarcoma, Anal Leiomyosarcoma, and Prostate 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 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.