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 4 Sensitivity, specificity, and limitations of various methods of radiological imaging of PPGL [21,28,37,51].
| Sensitivity | Specificity | Limitations | |
|---|---|---|---|
| Ultrasound | 76% () | ||
| Computed tomography (CT) | Small tumors: 90–92%.Big tumors: 100% ()90% ()88–100% () | 93% () | |
| Magnetic resonance imaging (MRI) | 93.3% ()Head and neck paragangliomas: 90–95% () | 93% () | |
| [I]-MIBG | 75–90% ()90.6% ()Pheochromocytomas: 85–88%.Paragangliomas: 56–75% () | 100% ()100% ()Pheochromocytomas: 70–100%.Paragangliomas: 84–100% () | |
| [F]-FDG PET | 74–100% ()66–78% () | ||
| [Ga]-DODATATE PET | 72–100% () | ||
| CT – computed tomography; MRI – magnetic resonance imaging; PPGL – pheo-chromocytoma and paraganglioma; MIBG – metaiodobenzylguanidine; FDG – fluorodeoxyglucose; FDOPA – fluorodihydroxy phenylalanine; DOTATATE – DOTA-Tyr3-Octreotate; PET – positron emission tomography. | |||