A CASE OF CHILDHOOD ADRENOCORTICAL TUMOR

A TICKING TIME BOMB

Abstract

INTRODUCTION/BACKGROUND
Childhood adrenocortical tumours (ACTs) are rare. Majority of children have clinical evidence of hormone hypersecretion at diagnosis which varies from virilization to Cushing’s syndrome. Markers for risk stratification in ACTs are not well-defined and differentiating adenomas from carcinomas remain challenging.

CASE
We describe a child with adrenocortical tumour presenting with virilization and hypertensive crisis. A 6-year-old male was initially admitted for status epilepticus attributed to rotavirus acute gastroenteritis. He had hirsutism, pubic hair, penile enlargement, and gynecomastia, which started 2 years ago. His weight and height were at the 50th centile. He developed hypertensive crisis requiring five anti-hypertensive agents including parenteral infusion. Blood tests showed high testosterone, renin and aldosterone. 24-hour urinary sample
demonstrated hypercortisolism. Magnetic resonance imaging of the upper abdomen showed a left adrenal mass. He underwent complete resection of the solid mass, which measured 83 x 82 x 59 mm and showed no signs of infiltration. The tumour scored 2/9 using the Wieneke algorithm, features compatible with adrenocortical adenoma. Post-resection, his blood pressure normalized with a single oral anti-hypertensive. He was counselled for adjuvant chemotherapy, but the family opted for watchful management.

CONCLUSION
The paucity of data on prognostic factors, disease staging, and treatment hinders the clinician’s ability in identifying patients at high risk of relapse even after complete tumour resection. More targeted predictive models for recurrence or malignant risk will be helpful in guiding treatment strategies.