From the Guidelines
Pheochromocytoma in toddlers requires prompt diagnosis and treatment with alpha-adrenergic blockade using phenoxybenzamine, followed by surgical removal of the tumor, as stated in the most recent guidelines 1. The initial management involves stabilization with alpha-adrenergic blockade using phenoxybenzamine starting at 0.2 mg/kg/day divided twice daily, gradually increasing to 0.4-1.2 mg/kg/day as needed. This must be started 7-14 days before any surgical intervention to control hypertension and prevent crisis.
Key Considerations
- Beta-blockers like propranolol (0.5-1 mg/kg/day divided three times daily) should only be added after adequate alpha blockade to prevent paradoxical hypertension.
- Definitive treatment is surgical removal of the tumor, which should be performed by an experienced pediatric surgeon at a center with pediatric endocrinology and intensive care support.
- Preoperative preparation includes adequate hydration and careful monitoring of blood pressure.
- Genetic testing is essential as up to 80% of pediatric pheochromocytomas are associated with hereditary syndromes like MEN2, VHL, or neurofibromatosis.
- Long-term follow-up is necessary as recurrence rates are higher in children than adults.
- Parents should be educated about symptoms requiring immediate attention, including severe headaches, sweating, palpitations, and hypertension. The diagnosis of pheochromocytoma should be entertained in a hypertensive patient with a combination of headaches, palpitations, and sweating, typically occurring in an episodic fashion, with a diagnostic specificity of 90% 1.
Diagnostic Approach
- The best screening test for pheochromocytoma is plasma free metanephrines (normetanephrine and metanephrine), which carries a 99% sensitivity and an 89% specificity.
- Despite improved diagnostic techniques, there remains an average of 3 years between the initial symptoms and final diagnosis, highlighting the need for prompt recognition and referral to a specialist center. In the context of pheochromocytoma, the use of alpha-1-blockers such as phentolamine, doxazosin, or terazosin is recommended for the management of adrenergic crises, as stated in the most recent guidelines 1.
Management of Adrenergic Crises
- Labetalol can also be used, which has the advantage of allowing titration of the infusion based on the BP response and avoids tachycardia via beta-blockade.
- Administering doxazosin or terazosin, followed by a beta-blocker, usually controls BP and adrenergic crises.
- Patients with pheochromocytoma have peripheral hypovolaemia, which exposes them to the risk of profound hypotension, particularly right after tumour excision, emphasizing the need for careful fluid management.
From the Research
Characteristics of Pheochromocytoma in Toddlers
- Pheochromocytoma is an unusual tumor in the pediatric age group, including toddlers 2
- Children with pheochromocytoma have fewer malignant tumors, more extraadrenal tumors, and greater bilaterality and multiplicity of tumor compared to adults 2
- Toddlers with pheochromocytoma may present with hypertension, headache, and sweating, similar to older children and adults 3, 4
Diagnosis and Treatment
- Diagnosis of pheochromocytoma in toddlers is made by laboratory and radiologic studies, including biochemical tests and imaging studies 2, 3, 4
- Preoperative medical therapy, including alpha-adrenergic blockade, is essential in the management of pheochromocytoma in toddlers 5, 6, 3
- Surgical treatment, including adrenalectomy, is the mainstay of treatment for pheochromocytoma in toddlers 2, 3, 4
Prognosis and Outcome
- The prognosis for toddlers with pheochromocytoma is generally good, with early diagnosis and total excision of the tumor being the most important aspects of accurate treatment 3
- However, incomplete excision and advanced-stage disease are major determinants of poor outcome 3
- Long-term follow-up is essential to monitor for recurrence and metastasis 3, 4