Pheochromocytoma and Paraganglioma PDQ®

In brief

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.

• 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.

Written by the GCMD Library team from the guideline.