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