03 April 2025 : Review article
Current Strategies for Managing Pheochromocytoma and Paraganglioma in Children and Adolescents
Aneta Michalczewska
ADEF 1*, Urszula Ambroziak DEF 2, Piotr Skrzypczyk ADEFG 3
DOI: 10.12659/MSM.947277
Med Sci Monit 2025; 31:e947277
Introduction
Incidence and Prevalence
Genetic Basis
Clinical Presentation
Biochemical Testing
Imaging Studies
Screening in Families
Pharmacological Treatment
Perioperative Management
Surgical Treatment
Treatment of Metastatic Tumors
Treatment of Recurrent Tumors
Surveillance
Personalized Management
Future Directions
Conclusions
References
Table 5 Medications used for preoperative management of arterial hypertension in pediatric PPGL adapted from Jain et al (2020) [15].
| Drug class | Dosage | Maximum recommended dose |
|---|---|---|
| α-adrenergic receptor blockers | ||
| Phenoxybenzamine | 0.2 mg/kg/day (max. 10 mg/dose)Increase the dose by 0.2 mg/kg/day every 4 daysGoal: 0.4–1.2 mg/kg/day ÷ 6–8 h | 2–4 mg/kg/day (60 mg/day) |
| Doxazosin | 1–2 mg/dayIncrease the dose by 2–4 mg/dayGoal: 2–4 mg/day ÷ 8–12 h | 4–16 mg/day |
| Prazosin | 0.05–0.1 mg/kg/day ÷ 8 h | 0.5 mg/kg/day (20 mg/day) |
| Terazosin | 1 mg/dayIncrease the dose to 1–4 mg/day | 20 mg/day |
| β-adrenergic receptor blockers | ||
| Propranolol | 1–2 mg/kg/day ÷ 6–12 hIncrease the dose to 4 mg/kg/day ÷ 6–12 h | 640 mg/day |
| Atenolol | 0.5–1 mg/kg/day ÷ 12–24 hIncrease the dose to 2 mg/kg/day ÷ 12–24 h | 100 mg/day |
| Metoprolol | 1–2 mg/kg/day ÷ 12–24 hIncrease the dose to 2 mg/kg/day ÷ 12–24 h | 200 mg/day |
| α- and β-adrenergic receptor blocker | ||
| Labetalol | 1–3 mg/kg/day ÷ 8–12 hIncrease the dose to 10–12 mg/kg/day ÷ 8–12 h | 1200 mg/day |
| Calcium channel blocker | ||
| Amlodipine | 0.05–0.1 mg/kg/day ÷ 12–24 hIncrease the dose to 0.3 mg/kg/day ÷ 12–24 h | 10 mg/day |
| Tyrosine hydroxylase inhibitor | ||
| Metyrosine | 20 mg/kg/day ÷ 6 hIncrease the dose to 60 mg/kg/day ÷ 6 h | 2500 mg/day |