AI Article Synopsis
- Phaeochromocytoma and paraganglioma (PPGL) are rare tumors that develop from specialized cells in the adrenal gland or nearby nerve tissues, affecting both adults and, less commonly, children.
- The annual incidence in children is approximately 0.5 cases per million, and there's been significant progress in understanding how to diagnose and treat these tumors, primarily based on adult studies.
- This article reviews the unique challenges and management strategies for diagnosing and treating pediatric PPGL, including recent international guidelines.
Article Abstract
Phaeochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumours which arise from chromaffin cells of the adrenal medulla or extra-adrenal autonomic ganglia. PPGL most commonly present in adulthood but can arise in childhood and adolescence with an estimated annual incidence of 0.5 cases per million children per year. There have been significant advances in the diagnosis and management of PPGL over the past 2-3 decades based largely on the study of adult patients. These advances in clinical knowledge can be applied to paediatric patients but like other cancers, paediatric PPGL must be viewed as a distinct subset with their own specific challenges and opportunities for improved clinical care. This review article provides an overview on the diagnosis and management of PPGL in children focusing on recent international guidance.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.beem.2024.101957 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!