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Pheochromocytoma in childhood
1Division of Pediatric Surgery, UCLA School of Medicine 90024.
Insights
Pediatric pheochromocytomas are rare tumors causing hypertension in children. Surgical removal is now safer, with a mortality rate under 3%, due to improved preoperative management.
Area of Science:
- Pediatric Endocrinology
- Surgical Oncology
- Pediatric Hypertension
Background:
- Pheochromocytomas are rare neuroendocrine tumors originating from chromaffin cells.
- In children, these tumors account for 1% of hypertension cases and often present as bilateral, multiple, or extra-adrenal.
- Malignancy is infrequent in pediatric pheochromocytomas.
Purpose of the Study:
- To review the characteristics and management of pediatric pheochromocytomas.
- To highlight advancements in surgical outcomes for this rare condition.
Main Methods:
- Review of clinical presentation, diagnostic approaches, and treatment strategies for pediatric pheochromocytomas.
- Analysis of surgical outcomes and complications, particularly focusing on preoperative management and surgical approaches.
Main Results:
- Children with pheochromocytomas frequently exhibit bilateral, multiple, or extra-adrenal tumors.
- Preoperative administration of alpha- and beta-blocking agents has significantly reduced surgical complications.
- Transabdominal resection is the preferred surgical approach due to tumor characteristics in children.
- The current mortality rate for pheochromocytoma removal in children is less than 3%.
Conclusions:
- Pheochromocytomas in children, while uncommon, require specific management considerations.
- Multidisciplinary care involving endocrinologists and surgeons is crucial for optimal outcomes.
- Advances in medical and surgical management have dramatically improved the safety and success of tumor resection.
Abstract:
Pheochromocytomas are uncommon tumors of childhood, accounting for 1% of hypertension cases in this age-group. Children have a high incidence of bilateral, multiple, or extra-adrenal tumors and a low incidence of malignancy. Alpha- and beta-blocking agents administered preoperatively have reduced complications markedly. Transabdominal resection is recommended because of the frequent extra-adrenal sites and multicentricity of the tumor in children. The mortality for removal of pheochromocytomas in childhood is currently less than 3%.