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Updated: May 17, 2025

A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Ectopic ACTH-secreting pheochromocytoma without typical signs of Cushing syndrome
Zeltzin Soto-Montes1, David Medina-Julio2,3, Orlando D Solis-Coronado4
1Department of Endocrinology, Instituto Nacional de Ciencias Medicas y Nutrición Salvador Zubiran, Vasco de Quiroga 15, Belisario Domínguez Secc 16, Tlalpan, Ciudad de Mexico 14080, Mexico.
Abstract:
This case report describes a 42-year-old female with a rare pheochromocytoma presenting without classic Cushingoid features but with uncontrolled hypertension, type 2 diabetes, and recurrent headaches. Despite the absence of typical signs, biochemical analysis revealed elevated cortisol and ACTH levels, and imaging showed a 6 cm adrenal mass. The patient was stabilized preoperatively with alpha-blockers and metyrapone before undergoing a successful laparoscopic adrenalectomy. Histopathology confirmed pheochromocytoma with aggressive features. Postoperatively, her blood pressure and symptoms improved, and her cortisol levels normalized. This case underscores the diagnostic challenges of ACTH-secreting pheochromocytomas without classic hypercortisolism signs and emphasizes the need for thorough endocrine and imaging assessments. Surgical resection remains the definitive treatment, with long-term follow-up essential to monitor for recurrence. This case contributes to the limited literature on the coexistence of pheochromocytoma and ectopic ACTH secretion.
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