From the Research
Pheochromocytomas in toddlers typically present with irritability, failure to thrive, pallor, and severe hypertension, which can be resistant to standard antihypertensive medications, and require early recognition and treatment to prevent cardiovascular complications and potentially fatal hypertensive crisis. The clinical presentation of pheochromocytoma in toddlers can be distinct from adult presentations, with symptoms such as episodic headaches, sweating, and tachycardia being difficult to elicit in young children 1. Toddlers may show signs of hypertensive crisis, including seizures, visual disturbances, or altered mental status, as well as excessive sweating, flushing, vomiting, and unexplained fever. Weight loss despite normal appetite and behavioral changes like anxiety, restlessness, or sleep disturbances may also occur, but can be challenging to distinguish from normal toddler behavior.
Key considerations in the diagnosis of pheochromocytoma in toddlers include:
- Family history, as approximately 30-40% of pheochromocytomas in children are associated with genetic syndromes like MEN2, von Hippel-Lindau disease, or neurofibromatosis type 1 2, 1
- Biochemical testing, including plasma or urine metanephrines and catecholamines, as the gold standard for diagnosis 1
- Imaging studies, such as MR imaging, for localization and identification of metastatic lesions 3
- Preoperative therapy with alpha-blocking agents, beta blockers, and potentially tyrosine hydroxylase inhibitors to aid in a safe pre-, intra- and postoperative course 1
Early recognition and treatment of pheochromocytoma in toddlers are crucial to prevent cardiovascular complications, developmental issues, and potentially fatal hypertensive crisis, and a multidisciplinary team approach is recommended for optimal healthcare delivery. Genetic counseling should be undertaken in all cases, and lifelong follow-up for recurrent tumors is necessary, particularly in children with genetic syndromes associated with pheochromocytoma 1, 4.