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Scholars Journal of Medical Case Reports | Volume-14 | Issue-03
Ectopic ACTH-Secreting Cushing Syndrome of Pancreatic Origin: A Fatal Outcome Despite Early Diagnosis - A Case Report
Nada El Idrissi Dafali, Sana Rafi, Sara Ijdda, Ghiz lane El Mghari, Nawal EL Ansari
Published: March 24, 2026 |
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43
Pages: 475-477
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Abstract
Ectopic Cushing syndrome is a rare cause of ACTH-dependent hypercortisolism, with pancreatic neuroendocrine tumors representing an exceptional origin. We report the case of a 48-year-old woman presenting with severe Cushing syndrome, characterized by central obesity, hirsutism, secondary amenorrhea, and marked skin fragility. Laboratory evaluation confirmed pronounced ACTH-dependent hypercortisolism, and imaging revealed a suspicious pancreatic lesion. The patient was being prepared for surgical resection; however, her condition deteriorated rapidly, culminating in fatal septic shock before surgery. Pancreatic ectopic Cushing syndrome is associated with high morbidity due to metabolic, infectious, and cardiovascular complications. Diagnosis relies on elevated plasma ACTH, normal pituitary MRI, and targeted functional imaging. Optimal management involves surgical excision, with medical therapy to control hypercortisolism while awaiting intervention. This case underscores the potentially severe course of pancreatic ectopic Cushing syndrome and highlights the importance of early recognition and multidisciplinary management.


