Management of functioning pediatric adrenal tumors

Area of Science:

• Pediatric Endocrinology
• Pediatric Oncology
• Surgical Oncology

Background:

• Hormonally active adrenal tumors in children are rare but can cause significant morbidity.
• Effective management requires a multidisciplinary approach and specialized expertise.
• Adrenocortical tumors (ACTs) and pheochromocytomas (PCCs) are the primary types encountered.

Purpose of the Study:

• To present the institutional experience in diagnosing and managing pediatric hormonally active adrenal tumors.
• To evaluate the outcomes of surgical and medical interventions.
• To identify factors influencing prognosis and recurrence.

Main Methods:

• Retrospective chart review of pediatric patients with hormonally active adrenal tumors (1983-2019).
• Inclusion criteria: evaluated at endocrinology clinic and operated at the institution.
• Data collected on tumor type, clinical presentation, pathology, treatment, and follow-up.

Main Results:

• 75 patients included: 58 with ACTs and 17 with PCCs.
• ACTs: 67.2% virilization, 8.6% Cushing syndrome; 18.9% positive family history; 10.3% recurrence, 5% disease-related death.
• PCCs: 41.2% positive family history; 35.3% recurrence, 5.9% disease-related death.

Conclusions:

• Specialized care at a referral center leads to high cure rates for pediatric adrenal tumors, including malignant types.
• Early diagnosis and appropriate management are key to favorable outcomes.
• Genetic counseling and familial surveillance are recommended due to a notable incidence of positive family history.