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
Botryoid-Type Embryonal Rhabdomyosarcoma, Orbit Embryonal Rhabdomyosarcoma, Spindle Cell Rhabdomyosarcoma, and Extrahepatic Bile Duct 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.