Objective:

To enhance diagnostic awareness of ACTH-secreting pituitary adenomas with apoplexy in pediatric cases through a detailed case analysis.

Approach:

• Case Presentation: A 13-year-old boy presented with severe headache, anuria, hypertensive crisis, and rapid weight gain. Biochemical evaluation and MRI confirmed an ACTH-secreting adenoma, leading to surgical intervention.

Key Findings:

• The patient exhibited severe hypertension (180/120 mmHg), anuria, and rapid weight gain (20 kg over 2 months).
• Biochemical tests showed elevated ACTH (190 pg/mL), disrupted cortisol rhythm, and hypokalemia (2.16–3.0 mmol/L).
• MRI revealed a 6mm×5mm cystic lesion in the left adenohypophysis with mixed T1/T2 signals and no post-contrast enhancement, consistent with apoplexy.
• Histopathology confirmed an ACTH-secreting adenoma with hemorrhagic necrosis.
• Postoperative follow-up at 3 years showed normal blood pressure, weight, kidney function, and endocrine function, with no sign of recurrence.

Interpretation:

Pediatric ACTH-secreting pituitary apoplexy presents diagnostic challenges due to nonspecific symptoms and atypical imaging features. MRI is crucial for initial evaluation, while histopathology provides confirmation.

Limitations:

• Pediatric cases of ACTH-secreting adenomas are rare, limiting generalizability.
• Current evidence is predominantly derived from adult studies, with limited pediatric data.

Conclusion:

Early surgical intervention and hormonal management are critical for favorable outcomes in pediatric patients with ACTH-secreting pituitary adenomas.