Diffuse nesidioblastosis underwent reoperation after streptozotocin treatment / 대한내분비학회지

ABSTRACT

Nesidioblatosis is a term that describes small clusters of pancreatic islet cells budding off exocrine ducts, and is commonly reported in infants with intractable idiopathic hypoglucemia. The onset of nesidioblastosis in adults is an extremely rare entity associated with hypersecretion of insulin and the treatment of choice is pancreatic resection. Medical treatment, including somatostatin, propranolol, diazoxide, hydrochlorthiazide and streptozotocin have achieved limited success. We experienced a case of adult nesidioblastosis that underwent reoperation after the failure of medical treatment following an inappropriate first operation. A 54-year old man was admitted due to intermittent hypoglycemic symptoms, which had been relieved by carbohydrate ingestion. Hyperinsulinemic hypoglycemia was documented during a prolonged fast. Image studies found no localized lesion, so a distal pancreatectomy was performed. The pathological examination of the resected pancreas revealed irregularly sized islets and a scattering of small endocrine cell clusters throughout the acinar tissue and ductuloinsular complex. After a partial pancreatectomy the hypoglycemia had not disappeared. The patient did not want to undergo a reoperaton due to the post operative wound infection that occurred after the distal pancreatectomy. Therefore, diazoxide, somatostatin, propranolol, and streptozotocin was used as the alternative to an operation. However, the hypoglycemia persisted during and after the medical treatment. Finally, he underwent a near total pancreatectomy (85%), and the hypoglycemia disappeared. The extent of pancreatectomy is important in clinical outcome of patients with nesidioblastosis