Case Report

Ectopic ACTH Secretion by Islet Cell Neuroendocrine Carcinoma: Case Report and Review of the Literature

Chertman Marcos and Chertman Lila
World Journal of Medical and Surgical Case Reports 2013, 2:13

Abstract

Objective

To review the literature and describe a case of ectopic Cushing’s syndrome caused by a neuroendocrine carcinoma of the pancreas.

Methods

We present the clinical, laboratory, radiologic, and pathologic details of the case and review the relevant literature.

Results

A 65 year old woman presented with Cushing’s syndrome, hyperglycemia, hypokalemia, and a pancreatic mass. CT-guided aspiration and laparoscopic distal pancreatectomy were performed. Pathological examination revealed a neuroendocrine carcinoma with ectopic production of ACTH. She was treated initially with Ketoconazole and then monthly Sandostatin, and Cushingoid features have resolved.

Conclusion

Pancreatic neuroendocrine tumors that secrete ACTH causing Cushing’s syndrome are rare, posing a diagnostic and therapeutic challenge to the practitioner. We present a case of a rapidly evolving neuroendocrine Cushing’s syndrome that was treated successfully with surgery and medical management.

Key Words

Pancreas, Neuroendocrine, Ectopic, Cushing’s syndrome, Octreotide

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