Pheochromocytomas arise from adrenal medulla; paragangliomas from extra-adrenal chromaffin tissue—both secrete catecholamines.

Pediatric cases often present with sustained hypertension, headache, sweating, and palpitations due to excess catecholamine release.

Genetic syndromes (MEN2, VHL, NF1, SDH mutations) account for majority of pediatric cases—family history and genetic testing crucial.

Preoperative alpha-blockade essential to prevent hypertensive crisis during surgical resection, the primary curative treatment.

Malignant potential exists; long-term surveillance required for metastatic disease and recurrence, especially in syndromic cases.

Pheochromocytoma and Paraganglioma PDQ® | GlobalCastMD Clinical Guidelines

Guideline document

This guideline is provided for in-page reading only. Please contact the publishing organization for downloadable copies.

Topic overview

Comprehensive clinical reference from NCI covering diagnosis, staging, and treatment approaches for childhood pheochromocytoma and paraganglioma. Provides evidence-based guidance for managing these rare catecholamine-secreting neuroendocrine tumors in pediatric patients.

Key Takeaways

Pheochromocytomas arise from adrenal medulla; paragangliomas from extra-adrenal chromaffin tissue—both secrete catecholamines.
Pediatric cases often present with sustained hypertension, headache, sweating, and palpitations due to excess catecholamine release.
Genetic syndromes (MEN2, VHL, NF1, SDH mutations) account for majority of pediatric cases—family history and genetic testing crucial.
Preoperative alpha-blockade essential to prevent hypertensive crisis during surgical resection, the primary curative treatment.
Malignant potential exists; long-term surveillance required for metastatic disease and recurrence, especially in syndromic cases.

Keywords

Hashtags

#Pheochromocytoma #Paraganglioma #PediatricOncology #NeuroendocrineTumors

Full guideline text

Full text not available for this guideline

How to cite: GlobalCastMD. Pheochromocytoma and Paraganglioma PDQ®. GlobalCastMD Medical Library. https://library.globalcastmd.com/guideline/2388