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Corticotropin-secreting pheochromocytoma: A case report
Pedro Iglesias1, Juan González González2, Emiliano González-Vioque3
1Department of Endocrinology and Nutrition, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain; Instituto de Investigación Sanitaria Puerta de Hierro Segovia de Arana, Majadahonda, Madrid, Spain.
None:
Corticotropin (ACTH)-secreting pheochromocytomas are rare neuroendocrine tumors that combine catecholamine and cortisol excess, posing major diagnostic and therapeutic challenges. We report the case of a 38-year-old man with hypertension, episodic adrenergic symptoms, and biochemical evidence of elevated metanephrines and ACTH-dependent hypercortisolism. Imaging revealed a right adrenal mass compatible with pheochromocytoma, but metaiodobenzylguanidine (MIBG) scintigraphy was negative. After preoperative preparation with alpha- and beta-blockade and intraoperative steroid coverage, the patient underwent laparoscopic adrenalectomy. Histopathology confirmed pheochromocytoma with focal ACTH expression and malignant potential (PASS 5). Postoperatively, he required transient glucocorticoid replacement and achieved normotension without antihypertensives. This case illustrates the complexity of diagnosing and managing ACTH-secreting pheochromocytomas, highlights the need for careful preoperative stabilization, and underscores the importance of multidisciplinary follow-up to detect recurrence and manage adrenal insufficiency.
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