Byun Justin, Kim Sung Hyun, Jeong Hyang Sook, Rhee Yumie, Lee Woo Jung, Kang Chang Moo
Medical Course, Yonsei University College of Medicine, Seoul, Korea.
Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Yonsei University College of Medicine, Seoul, Korea.; Pancreaticobiliary Cancer Clinic, Yonsei Cancer Center, Severance Hospital, Seoul, Korea.
Ann Hepatobiliary Pancreat Surg. 2017 Feb;21(1):61-65. doi: 10.14701/ahbps.2017.21.1.61. Epub 2017 Feb 28.
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.
起源于胰腺内分泌部或胰岛的肿瘤被称为胰腺神经内分泌肿瘤（NETs）。胰腺NET的发病率为每百万人中少于0.1例，并且可导致异位促肾上腺皮质激素（ACTH）分泌。在此，我们报告了一例因产生ACTH的胰腺NET导致库欣综合征的患者，该患者接受了成功的腹腔镜远端胰腺脾切除术。一名40岁的韩国女性患者因眼部不适、骨质疏松和不明原因的低钾血症入院。在诊断性检查后怀疑为产生ACTH的胰腺NET，我们决定进行手术切除。实施了腹腔镜远端胰腺脾切除术；病理检查显示为一个1.5厘米大小的胰腺2级神经内分泌肿瘤，包被于胰腺实质内。术后，患者不再表现出库欣样特征。产生ACTH的胰腺NET很罕见，但可能是库欣综合征的病因之一。手术切除是治疗产生ACTH的胰腺NET的一种可行选择。
