Overview

NCI Definition: A neuroblastic tumor characterized by the presence of neuroblastic cells, ganglion cells, and a stroma with Schwannian differentiation constituting more than fifty-percent of the tumor volume. There are two histologic subtypes identified: ganglioneuroblastoma, intermixed and ganglioneuroblastoma, nodular. [1]

Ganglioneuroblastomas most frequently harbor alterations in RNF43, RBM10, RARA, PPM1D, and BRIP1 [2].

PPM1D Amplification, BRIP1 Amplification, ALK Mutation, ALK Missense, and ALK Codon 1174 Missense are the most common alterations in ganglioneuroblastoma [2].

Significant Genes in Ganglioneuroblastoma

Disease Details

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.