Overview

NCI Definition: A rapidly growing malignant mesenchymal neoplasm. It is characterized by the presence of round cells with myoblastic differentiation and a fibrovascular stroma resembling an alveolar growth pattern. The tumor usually presents in the extremities. [1]

Alveolar rhabdomyosarcomas most frequently harbor alterations in FOXO1, PAX3, MYCN, PAX7, and MYCL [2].

Most Commonly Altered Genes in Alveolar Rhabdomyosarcoma

FOXO1 Fusion, PAX3-FOXO1 Fusion, FOXO1-PAX3 Fusion, MYCN Amplification, and PAX7-FOXO1 Fusion are the most common alterations in alveolar rhabdomyosarcoma [2].

Top Alterations in Alveolar Rhabdomyosarcoma

Significant Genes in Alveolar Rhabdomyosarcoma

MYCN +

Disease Details

Synonyms
Monomorphous Round Cell Rhabdomyosarcoma, PEDIATRIC Soft tissue rhabdomyosarcoma alveolar, Soft tissue rhabdomyosarcoma alveolar, ARMS
Parent(s)
Rhabdomyosarcoma
Children
Orbit Alveolar Rhabdomyosarcoma
OncoTree Name
Alveolar Rhabdomyosarcoma
OncoTree Code
ARMS

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 8. 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.