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Related Experiment Video

Updated: Jul 4, 2026

A Novel Method: Super-selective Adrenal Venous Sampling

A Novel Method: Super-selective Adrenal Venous Sampling

Published on: September 15, 2017

Update on pediatric pheochromocytoma.

Bas Havekes¹, Johannes A Romijn, Graeme Eisenhofer

¹Reproductive Biology and Medicine Branch, Section on Medical Neuroendocrinology, National Institute of Child Health and Human Development, NIH, Bethesda, MD, 20892-1109, USA.

Pediatric Nephrology (Berlin, Germany)

|June 21, 2008

Summary

Pediatric pheochromocytomas, rare tumors, require updated diagnostic and treatment strategies due to genetic factors and varied presentations. Early detection via metanephrine tests and genetic screening is crucial for managing these catecholamine-secreting tumors.

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Endoscopic Endonasal Trans-sphenoidal Approach: Minimally Invasive Surgery for Pituitary Adenomas

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Published on: January 17, 2018

Area of Science:

Pediatric Endocrinology
Oncology
Genetics

Background:

Pheochromocytomas are rare pediatric tumors originating from chromaffin cells, synthesizing and secreting catecholamines.
Pediatric cases often present as familial, extra-adrenal, bilateral, and multifocal, differing from adult presentations.
The clinical presentation is highly variable, earning pheochromocytoma the nickname 'the great mimic'.

Purpose of the Study:

To review recent advances in the biochemistry, genetics, and imaging of pediatric pheochromocytomas.
To outline updated recommendations for the evaluation and treatment of children with these tumors.
To emphasize the importance of genetic testing in pediatric pheochromocytoma management.

Main Methods:

Review of current literature on pheochromocytoma diagnosis, genetics, and treatment in children.
Emphasis on updated biochemical testing, including plasma or urinary fractionated metanephrines.
Discussion of genetic testing implications for hereditary forms and malignant potential.

Main Results:

Plasma or urinary fractionated metanephrines are recommended as the primary diagnostic biochemical tests.
Preoperative alpha-blockade is essential for all cases, even those synthesizing but not secreting catecholamines.
Approximately 40% of pediatric pheochromocytomas have a hereditary basis, necessitating genetic testing.

Conclusions:

Updated understanding of pheochromocytoma requires revised diagnostic and management guidelines.
Genetic testing is critical for identifying hereditary predispositions and guiding long-term care.
Tailored screening, surgical, and systemic treatment strategies are informed by underlying mutations and disease risk.