Pancreatic neuroendocrine tumors with transformation to insulinoma: an unusual presentation of a rare disease

[摘要] Approximately 35% of the pancreatic neuroendocrine tumors (pNETs) are functional, the most common of which is aninsulinoma. Rarely can initially nonfunctioning tumor undergo biological transformation to a hormone-secreting tumor withsubsequent changes in the clinical picture. We present here three unique patients with long-standing pNETs who developedlife-threatening hyperinsulinemic hypoglycemia along with tumor progression. In two of the patients, everolimus (Afinitor)was administered in an attempt to control both tumor growth and hypoglycemia. In two cases everolimus therapy resulted inthe abolishment of hypoglycemia and induced significant tumor regression; however these beneficial responses weretransient. These cases highlight the exceptional ability of pNETs to change biological behavior in parallel with diseaseprogression. Our experience concurs with recently published studies demonstrating the utility of everolimus for the controlof both hypoglycemia and tumor progression.

[学科分类] 血液学

[关键词] Adult;Female;White;Israel;Pancreas;Endocrine-related cancer;Insulin;Neuroendocrine tumour;Insulinoma;Pancreatic neuroendocrine tumour;Hypoglycaemia;Abdominal pain;Abdominal lump;Diarrhoea;Weight loss;Hepatic metastases;PET scan;CT scan;Octreotide scan;Insulin;Glucose (blood);C-peptide (blood);Gastrin;Chromogranin A;Radionuclide therapy;Pancreaticoduodenectomy;Chemoembolisation;Radiofrequency ablation;Resection of tumour;Everolimus;Lanreotide;Octreotide;Somatostatin analogues;Fluorouracil;Streptozotocin;Diazoxide;Dexamethasone;Glucocorticoids;Unique/unexpected symptoms or presentations of a disease;June;2015