Overview

NCI Definition: A poorly circumscribed morphologic variant of rhabdomyosarcoma. It is characterized by the presence of primitive skeletal muscle differentiation in any stage of myogenesis. [1]

Embryonal rhabdomyosarcomas most frequently harbor alterations in TP53, NRAS, NF1, MDM2, and HRAS [2].

Most Commonly Altered Genes in Embryonal Rhabdomyosarcoma

TP53 Mutation, NRAS Mutation, NRAS Exon 3 Mutation, NRAS Exon 3 Missense, and NRAS Codon 61 Missense are the most common alterations in embryonal rhabdomyosarcoma [2].

Top Alterations in Embryonal Rhabdomyosarcoma

Significant Genes in Embryonal Rhabdomyosarcoma

ARAF +

BRAF +

CRKL +

HRAS +

KRAS +

MAP2K1 +

MAP2K2 +

MAP2K4 +

MAP3K1 +

MAPK1 +

MYCN +

NF1 +

NRAS +

RAF1 +

SRC +

Disease Details

Synonyms
ERMS
Parent(s)
Rhabdomyosarcoma
Children
Extrahepatic Bile Duct Embryonal Rhabdomyosarcoma, Orbit Embryonal Rhabdomyosarcoma, Spindle Cell Rhabdomyosarcoma, and Botryoid-Type Embryonal Rhabdomyosarcoma
OncoTree Name
Embryonal Rhabdomyosarcoma
OncoTree Code
ERMS

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.