Characteristics of Pediatric Pheochromocytoma/paraganglioma

Overview

Journal Indian J Endocrinol Metab

Specialty Endocrinology

Date 2017 May 30

PMID 28553607

Citations 5

Authors

Vijaya Sarathi

Affiliations

Soon will be listed here.

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.

Citing Articles

Bilateral pheochromocytomas: clinical presentation and morbidity rate related to surgery technique and genetic status.

Lider Burciulescu S, Livia Gheorghiu M, Muresan A, Gherlan I, Patocs A, Badiu C Endocr Connect. 2024; 13(4).

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Unusual Cardiac Manifestations of a Pheochromocytoma in a Girl.

DAngelo L, Parent A, Derwael C, Hustinx R, Seghaye M Pediatr Rep. 2023; 15(1):237-244.

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Biochemically normal adrenal pheochromocytoma following extensive central necrosis in a child with von Hippel-Lindau (VHL) gene mutation.

Ng B, Wong J, Toh T BMJ Case Rep. 2021; 14(12).

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Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea.

Park H, Kim M, Lee J, Kim J, Jeong B, Lee S Front Endocrinol (Lausanne). 2021; 11:610746.

PMID: 33584544 PMC: 7879705. DOI: 10.3389/fendo.2020.610746.

Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management.

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