Current Strategies for Managing Pheochromocytoma and Paraganglioma in Children and Adolescents

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors that are an important cause of secondary hypertension. The most frequently reported symptom is persistent hypertension, exceeding paroxysmal hypertension. In children with elevated blood pressure, secondary hypertension should always be considered and investigated. These tumors exhibit a wide variety of clinical presentations, including syndromic presentations and life-threatening hypertensive crises. Recent advancements in genetic analysis have identified a growing number of pathogenic mutations. In the pediatric population, 70-80% of tumors exhibit germline mutations in known genes, predisposing children to metastatic and multifocal disease. The diagnosis and treatment remain challenging and often require a multidisciplinary approach at a highly specialized center. Plasma-free metanephrines measurement is considered the criterion standard for diagnosis due to its high sensitivity and reliable pediatric reference values. Functional imaging is helpful for pheochromocytoma and paraganglioma due to its high sensitivity and specificity, and it plays a crucial role in assessing regional extension, multifocality, and the presence of metastases, which are more frequently reported in children. Following definitive diagnosis and appropriate perioperative management, surgery remains the primary treatment. Genetic testing and counseling should be considered in all pediatric patients with confirmed pheochromocytoma and paraganglioma. Evidence and clinical trials in children are limited; therefore, the present report aims to review the presentation, associations, diagnosis, and management of pheochromocytoma and paraganglioma in children and adolescents.