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 c.142-c.212 Missense, TP53 c.1-c.137 Missense, and TP53 Missense 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 +

NBN +

PALB2 +

PIK3CA +

PTEN +

RAD50 +

RAD51 +

RAD51B +

RAD51C +

RAD51D +

SLX4 +

Disease Details

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