Two boys with four pheochromocytomas each.

Area of Science:

• Pediatric Endocrinology
• Surgical Oncology
• Nephrology

Background:

• Pheochromocytomas are rare neuroendocrine tumors arising from chromaffin cells, typically in the adrenal medulla.
• While often solitary, multiple or recurrent pheochromocytomas can occur, posing diagnostic and therapeutic challenges, particularly in pediatric patients.
• This case series examines two adolescent males with multiple, distinct pheochromocytomas requiring surgical management over extended periods.

Observation:

• Two teenage boys presented with severe hypertensive encephalopathy, necessitating surgical removal of pheochromocytomas.
• One patient developed four separate benign pheochromocytomas (adrenal and left adrenal) over 15 years, with initial surgery at ages 9-11.
• The second patient had multiple tumors (right renal artery, left para-aortic, right chest paravertebral, left adrenal) removed over time, starting at age 11.

Findings:

• All identified pheochromocytomas were pathologically benign.
• The absence of residual tumor and long disease-free intervals confirmed the distinct nature of the multiple tumors, not recurrence.
• Despite successful tumor removal, one patient experienced residual hemiplegia from the initial hypertensive crisis.

Implications:

• Multiple, distinct pheochromocytomas can occur in adolescents without a positive family history.
• Adolescents presenting with severe hypertension or related symptoms warrant thorough investigation for pheochromocytoma.
• Long-term surveillance is essential for pediatric patients with a history of pheochromocytoma due to the potential for new tumor development.