HIF and MYC signaling in adrenal neoplasms of the neural crest: implications for pediatrics

Research output: Contribution to journalReview articleContributedpeer-review



Pediatric neural crest-derived adrenal neoplasms include neuroblastoma and pheochromocytoma. Both entities are associated with a high degree of clinical heterogeneity, varying from spontaneous regression to malignant disease with poor outcome. Increased expression and stabilization of HIF2α appears to contribute to a more aggressive and undifferentiated phenotype in both adrenal neoplasms, whereas MYCN amplification is a valuable prognostic marker in neuroblastoma. The present review focuses on HIF- and MYC signaling in both neoplasms and discusses the interaction of associated pathways during neural crest and adrenal development as well as potential consequences on tumorigenesis. Emerging single-cell methods together with epigenetic and transcriptomic analyses provide further insights into the importance of a tight regulation of HIF and MYC signaling pathways during adrenal development and tumorigenesis. In this context, increased attention to HIF-MYC/MAX interactions may also provide new therapeutic options for these pediatric adrenal neoplasms.


Original languageEnglish
Article number1022192
Pages (from-to)1022192
JournalFrontiers in endocrinology
Publication statusPublished - 8 Jun 2023

External IDs

PubMedCentral PMC10286580
Scopus 85163610613
ORCID /0000-0002-6932-333X/work/142239674
WOS 001012461800001
Mendeley 3957b9a9-c0c8-363d-b1b2-7ca241353c45


Sustainable Development Goals


  • Humans, Child, Proto-Oncogene Proteins c-myc/metabolism, Neural Crest/metabolism, Signal Transduction/genetics, Adrenal Gland Neoplasms/metabolism, Neuroblastoma/metabolism, Carcinogenesis/metabolism, Pheochromocytoma, Sympathoadrenal cell lineage, Paraganglioma, Catecholamines, Neuroblastoma, Neural crest, Myc, Hypoxia, hypoxia, catecholamines, sympathoadrenal cell lineage, neural crest, neuroblastoma, pheochromocytoma, paraganglioma, MYC