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Characteristics of pediatric pheochromocytoma/paraganglioma
- Source :
- Indian Journal of Endocrinology and Metabolism, Vol 21, Iss 3, Pp 470-474 (2017), Indian Journal of Endocrinology and Metabolism
- Publication Year :
- 2017
- Publisher :
- Wolters Kluwer Medknow Publications, 2017.
-
Abstract
- The "rule of 10" used to describe pheochromocytoma/paragangliomas (PCC/PGLs) has been challenged. However, recent studies suggested that pediatric PCC/PGLs may follow a pattern. Hence, we reviewed the available literature to verify the same. We searched PubMed, Scopus, ProQuest, and Google Scholar for studies describing the genotype and/or phenotype characteristics of pediatric PCC/PGL cohorts published after 2000 in English language and those with sample size more than 35 were included in this review. Pediatric PCC/PGLs were malignant in 10%, synchronous bilateral in 20%, extra-adrenal in 30%, among which, 30% were extra-abdominal and familial in 40%. PCC/PGL diagnosed during pediatric age recurs in 50% by 30 years of follow-up and 60% cases occur in boys. Seventy percent of children with PCC/PGL are likely to have sustained hypertension. Germline mutations could be identified in 80% of children with PCC/PGL and 90% are secretory. The review concludes that pediatric PCC/PGLs follow a pattern, which we call "10%-90% rule." This new rule will help easily remember the characteristics of pediatric PCC/PGLs.
- Subjects :
- Pediatrics
medicine.medical_specialty
Endocrinology, Diabetes and Metabolism
Review Article
English language
macromolecular substances
lcsh:Diseases of the endocrine glands. Clinical endocrinology
Pheochromocytoma
03 medical and health sciences
paraganglioma
0302 clinical medicine
Endocrinology
Germline mutation
Paraganglioma
10%–90% rule
030225 pediatrics
Genotype
Medicine
lcsh:RC799-869
lcsh:RC648-665
business.industry
Pediatric age
medicine.disease
pheochromocytoma
pediatric
030220 oncology & carcinogenesis
bacteria
lcsh:Diseases of the digestive system. Gastroenterology
business
Subjects
Details
- Language :
- English
- ISSN :
- 22308210
- Volume :
- 21
- Issue :
- 3
- Database :
- OpenAIRE
- Journal :
- Indian Journal of Endocrinology and Metabolism
- Accession number :
- edsair.doi.dedup.....8c0dd663fb42fb37f01a0815c8c90af4