Ann Hepatobiliary Pancreat Surg.  2017 Feb;21(1):61-65. 10.14701/ahbps.2017.21.1.61.

ACTH-producing neuroendocrine tumor of the pancreas: a case report and literature review

• Byun J
• Kim SH
• Jeong HS
• Rhee Y
• Lee WJ
• Kang CM

• KMID: 2371570
• DOI: http://doi.org/10.14701/ahbps.2017.21.1.61

Abstract

Tumors that arise from the endocrine pancreas, or the islets of pancreas, are called pancreatic neuroendocrine tumors (NETs). Pancreatic NET have an incidence of <0.1 per one million persons, and can lead to secretion of ectopic adrenocorticotropic hormone (ACTH). Herein, we presented a case of patient with Cushing's syndrome as a result of ACTH-producing pancreatic NET, who underwent successful laparoscopic distal pancreatosplenectomy. A 40-year-old Korean female patient with ophthalmologic discomfort, osteoporosis, and unexplained hypokalemia was admitted to our hospital. Under the suspicion of ACTH producing pancreatic NET after the diagnostic workup, we decided to perform surgical resection. Laparoscopic distal pancreatosplenectomy was performed; and the pathological examination revealed a 1.5 cm-sized grade 2 neuroendocrine tumor of the pancreas, which was encapsulated within the pancreatic parenchyma. After the operation, the patient no longer displayed cushingoid features. ACTH-producing pancreatic NET is rare, but can be one of the causes of Cushing's syndrome. Surgical resection is a feasible option in treating ACTH-producing pancreatic NET.

Keyword

ACTH; Neuroendocrine tumor; Pancreas; Surgery

MeSH Terms

Adrenocorticotropic Hormone
Adult
Cushing Syndrome
Female
Humans
Hypokalemia
Incidence
Islets of Langerhans
Neuroendocrine Tumors*
Osteoporosis
Pancreas*
Adrenocorticotropic Hormone

Figure

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  Fig. 1 Patient follow-up. (A) Preoperation, (B) Postoperation, (C) ACTH and cortisol.

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  Fig. 2 Preoperative findings. (A) Adrenal Computed Tomography: No remarkable finding in both adrenal glands. (B) Endoscopic ultrasonography: 18 mm-sized solid mass was localized to the pancreatic tail. Echo intensity was hypoechoic and echo texture was homogenous (black arrows). (C) Uptake at the pancreatic tail in positron emission tomography (white arrows). (D) ACTH sampling from the pancreatic, splenic, and portal vein.

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  Fig. 3 Postoperative findings. (A) Gross specimen, (B) Ki-67, (C) Chromogranin A, (D) Synaptophysin, and (E) ACTH (×100).

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