Frontiers in endocrinology · 2026
Case Report: Pediatric ACTH-secreting pituitary adenoma presenting with hypertension and anuria.
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Li Jiaxin, Xia Wei, Wu Mengxing
Аннотация
BACKGROUND: Adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas are rare endocrine neoplasms. Pituitary apoplexy, caused by acute hemorrhage or infarction within the tumor, is an uncommon complication. Pediatric cases of ACTH-secreting adenomas with apoplexy are exceptionally rare, with limited understanding of their clinical and imaging manifestations. This report aims to enhance diagnostic awareness through a detailed case analysis.
CASE PRESENTATION: A 13-year-old boy presented with acute-onset severe headache, anuria, hypertensive crisis (180/120 mmHg), and rapid weight gain (20 kg over 2 months). Biochemical evaluation revealed elevated ACTH (190 pg/mL), disrupted cortisol rhythm, and hypokalemia (2.16-3.0 mmol/L). Pituitary magnetic resonance imaging (MRI) demonstrated a 6mm×5 mm 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. Transsphenoidal resection and hormone replacement achieved stable remission at the 3-year follow-up.
CONCLUSIONS: Pediatric ACTH-secreting pituitary apoplexy presents diagnostic challenges due to nonspecific symptoms and atypical imaging features. MRI remains pivotal for initial evaluation, though histopathology is confirmatory. Early surgical intervention and hormonal management are critical for favorable outcomes.
CASE PRESENTATION: A 13-year-old boy presented with acute-onset severe headache, anuria, hypertensive crisis (180/120 mmHg), and rapid weight gain (20 kg over 2 months). Biochemical evaluation revealed elevated ACTH (190 pg/mL), disrupted cortisol rhythm, and hypokalemia (2.16-3.0 mmol/L). Pituitary magnetic resonance imaging (MRI) demonstrated a 6mm×5 mm 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. Transsphenoidal resection and hormone replacement achieved stable remission at the 3-year follow-up.
CONCLUSIONS: Pediatric ACTH-secreting pituitary apoplexy presents diagnostic challenges due to nonspecific symptoms and atypical imaging features. MRI remains pivotal for initial evaluation, though histopathology is confirmatory. Early surgical intervention and hormonal management are critical for favorable outcomes.