Hyperinsulinemic hypoglycemia associated with ectopic Cushing鈥檚 syndrome due to a pancreatic endocrine tumor in a Type 2 diabetes mellitus patient: Clinical implications of a rare association

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• 作者：M. Filippella ; M. V. Dav矛 ; G. Doveri…
• 关键词：Hypoglycemic hyperinsulinemia ; ectopic Cushing鈥檚 syndrome ; neuroendocrine tumor ; pancreatic endocrine tumor ; Type 2 diabetes mellitus
• 刊名：Journal of Endocrinological Investigation
• 出版年：2011
• 出版时间：March 2011
• 年：2011
• 卷：34
• 期：3
• 页码：175-179
• 全文大小：
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• 作者单位：M. Filippella (1)
  M. V. Dav矛 (2)
  G. Doveri (1)
  E. Lillaz (1)
  A. Ciccarelli (1)
  E. Massimetti (1)
  M. Toaiari (2)
  M. Falconi (3)
  A. Colao (4)
  A. Faggiano MD, PhD (5)
  
  1. S.C. Internal Medicine Unit of Diabetology and Endocrinology, 鈥淧arini鈥?Hospital, Aosta
  2. Clinic of Internal Medicine D, University of Verona, Verona
  3. Surgical Unit B, University of Verona, Verona
  4. Department of Molecular and Clinical Endocrinology and Oncology, 鈥淔ederico II鈥?University, Italy
  5. IRCCS Fondazione SDN, Via E. Gianturco 113, 80143, Napoli, Italy
  
• 刊物类别：Medicine/Public Health, general; Endocrinology; Metabolic Diseases;
• 刊物主题：Medicine/Public Health, general; Endocrinology; Metabolic Diseases;
• 出版者：Springer International Publishing
• ISSN：1720-8386

文摘

Background: The coexistence of insulin and ACTH hypersecretion in the same patient is extremely rare. A diabetic patient with a pancreatic endocrine tumor (PET) co-secreting insulin and ACTH is even rarer and has never been described. The combination of these two endocrine syndromes results in a peculiar clinical picture. Aim: To determine the cause of glycemic variations in a patient with previously stable diabetes mellitus. Subjects and methods: This is a clinical case report from the Endocrinology Unit of Aosta Hospital and Internal Medicine and Surgical Unit of Verona University. A 69-yr-old diabetic patient was hospitalized for recurrent severe hypoglycemic events persistent after withdrawal of anti-diabetic drugs. The causes of hypoglycemia and subsequent resumption of hyperglycemia were investigated. Results: An insulin-secreting PET was diagnosed. Diazoxide and octreotide therapy initially was able to control hypoglycemic symptoms, then, a Cushing鈥檚 syndrome occurred resulting in worsening of diabetes control. ACTH was found to be released by the PET previously diagnosed as an insulin-secreting tumor. The tumor was removed and the histology was consistent with a well differentiated endocrine carcinoma. After surgery, adrenal function was normal and insulin therapy was again necessary to control diabetes. Conclusions: A single PET may be responsible for both a hyperinsulinemic and a Cushing鈥檚 syndrome. When this rare association occurs, each of the two syndromes may affect the other resulting in a peculiar clinical course. Finally, an insulin-secreting PET has to be kept in mind as a rare cause of hypoglycemia in diabetic patients. Key-words Hypoglycemic hyperinsulinemia ectopic Cushing鈥檚 syndrome neuroendocrine tumor pancreatic endocrine tumor Type 2 diabetes mellitus