Marked hypokalemia at presentation in an adolescent with ectopic ACTH syndrome secondary to a bronchial carcinoid tumor: a case report.

INTRODUCTION: Ectopic ACTH syndrome (EAS) is a rare cause of Cushing's syndrome in children and adolescents, accounting for less than 1% of pediatric cases. Compared with Cushing's disease, EAS follows a more rapid clinical course with more pronounced metabolic disturbances, among which hypokalemia is one of the most distinctive features. CASE PRESENTATION: A 14.7-year-old male presented with a three-month history of rapid weight gain, violaceous striae, hypertension, and proximal muscle weakness. Marked hypokalemia (2.8 mmol/L) prompted endocrine work-up, which revealed ACTH-dependent hypercortisolism with loss of diurnal cortisol rhythm, elevated midnight salivary cortisol, and non-suppression on low- and high-dose dexamethasone suppression tests. Pituitary MRI was unremarkable, and the marked hypokalemia raised suspicion of an ectopic source. Thoracic CT identified a nodular lesion in the right middle lobe, with corresponding uptake on ⁶⁸Ga-DOTATATE PET/CT. Right middle lobectomy was performed, and histopathology confirmed a typical bronchial carcinoid tumor. Postoperatively, hypercortisolism resolved, electrolyte balance normalized, and Cushingoid features regressed. CONCLUSION: Marked hypokalemia at presentation may serve as an early biochemical clue to ectopic ACTH secretion in pediatric ACTH-dependent hypercortisolism, and should prompt timely evaluation for extrapituitary sources, including bronchial carcinoid tumors.

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Publication Details

Journal
PubMed
Published
2026-10-05
DOI
https://doi.org/10.1159/hrp/adaag035
Primary Topic
Pituitary Gland Disorders and Treatments
Type
article
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article

Marked hypokalemia at presentation in an adolescent with ectopic ACTH syndrome secondary to a bronchial carcinoid tumor: a case report.

Şükran Poyrazoğlu, Doğu Vurallı Bakkaloğlu, Firdevs Baş, Zeynep Gözde Özkan et al.
PubMed
Pituitary Gland Disorders and Treatments
article

Marked hypokalemia at presentation in an adolescent with ectopic ACTH syndrome secondary to a bronchial carcinoid tumor: a case report.

Şükran Poyrazoğlu, Doğu Vurallı Bakkaloğlu, Firdevs Baş, Zeynep Gözde Özkan, Melek Yıldız, Aslı Derya Kardelen, Salih Duman, Hasan Yanik, Berker Ozkan
article en

Abstract

INTRODUCTION: Ectopic ACTH syndrome (EAS) is a rare cause of Cushing's syndrome in children and adolescents, accounting for less than 1% of pediatric cases. Compared with Cushing's disease, EAS follows a more rapid clinical course with more pronounced metabolic disturbances, among which hypokalemia is one of the most distinctive features. CASE PRESENTATION: A 14.7-year-old male presented with a three-month history of rapid weight gain, violaceous striae, hypertension, and proximal muscle weakness. Marked hypokalemia (2.8 mmol/L) prompted endocrine work-up, which revealed ACTH-dependent hypercortisolism with loss of diurnal cortisol rhythm, elevated midnight salivary cortisol, and non-suppression on low- and high-dose dexamethasone suppression tests. Pituitary MRI was unremarkable, and the marked hypokalemia raised suspicion of an ectopic source. Thoracic CT identified a nodular lesion in the right middle lobe, with corresponding uptake on ⁶⁸Ga-DOTATATE PET/CT. Right middle lobectomy was performed, and histopathology confirmed a typical bronchial carcinoid tumor. Postoperatively, hypercortisolism resolved, electrolyte balance normalized, and Cushingoid features regressed. CONCLUSION: Marked hypokalemia at presentation may serve as an early biochemical clue to ectopic ACTH secretion in pediatric ACTH-dependent hypercortisolism, and should prompt timely evaluation for extrapituitary sources, including bronchial carcinoid tumors.

PubMed
Openalex Percentile: Top 11%
Pituitary Gland Disorders and Treatments
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