Focal nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. Because it is difficult to localize and detect with current imaging modalities, nesidioblastosis is challenging for biliary-pancreatic surgeons. ⁶⁸Gallium-DOTA-D-Phe¹-Tyr³-octreotide PET scanning and ¹¹¹indium-pentetreotide diethylene triamine pentaacetic acid octreotide scanning may be superior to conventional imaging modalities in determining the localization of nesidioblastosis. We report the successful surgical treatment of a 54-year-old woman with focal hyperplasia of the islets of Langerhans, who experienced frequent hypoglycemic symptoms and underwent various diagnostic examinations with different results.
Adult ; Diagnosis* ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Islets of Langerhans ; Middle Aged ; Nesidioblastosis* ; Octreotide ; Positron-Emission Tomography* ; Surgeons

Focal nesidioblastosis is a rare cause of endogenous hyperinsulinemic hypoglycemia in adults. Because it is difficult to localize and detect with current imaging modalities, nesidioblastosis is challenging for biliary-pancreatic surgeons. ⁶⁸Gallium-DOTA-D-Phe¹-Tyr³-octreotide PET scanning and ¹¹¹indium-pentetreotide diethylene triamine pentaacetic acid octreotide scanning may be superior to conventional imaging modalities in determining the localization of nesidioblastosis. We report the successful surgical treatment of a 54-year-old woman with focal hyperplasia of the islets of Langerhans, who experienced frequent hypoglycemic symptoms and underwent various diagnostic examinations with different results.
Adult ; Diagnosis* ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Islets of Langerhans ; Middle Aged ; Nesidioblastosis* ; Octreotide ; Positron-Emission Tomography* ; Surgeons

BACKGROUND: Endogenous hyperinsulinemic hypoglycemia (EHH) is characterized by an inappropriately high plasma insulin level, despite a low plasma glucose level. Most of the EHH cases are caused by insulinoma, whereas nesidioblastosis and insulin autoimmune syndrome (IAS) are relatively rare. METHODS: To evaluate the relative frequencies of various causes of EHH in Korea, we retrospectively analyzed 84 patients who were diagnosed with EHH from 1998 to 2012 in a university hospital. RESULTS: Among the 84 EHH patients, 74 patients (88%), five (6%), and five (6%) were diagnosed with insulinoma, nesidioblastosis or IAS, respectively. The most common clinical manifestation of EHH was neuroglycopenic symptoms. Symptom duration before diagnosis was 14.5 months (range, 1 to 120 months) for insulinoma, 1.0 months (range, 6 days to 7 months) for nesidioblastosis, and 2.0 months (range, 1 to 12 months) for IAS. One patient, who was diagnosed with nesidioblastosis in 2006, underwent distal pancreatectomy but was later determined to be positive for insulin autoantibodies. Except for one patient who was diagnosed in 2007, the remaining three patients with nesidioblastosis demonstrated severe hyperinsulinemia (157 to 2,719 microIU/mL), which suggests that these patients might have had IAS, rather than nesidioblastosis. CONCLUSION: The results of this study suggest that the prevalence of IAS may be higher in Korea than previously thought. Therefore, measurement of insulin autoantibody levels is warranted for EHH patients, especially in patients with very high plasma insulin levels.
Autoantibodies ; Autoimmune Diseases ; Blood Glucose ; Diagnosis ; Humans ; Hyperinsulinism ; Hypoglycemia* ; Insulin ; Insulin Antibodies ; Insulinoma ; Korea ; Nesidioblastosis ; Pancreatectomy ; Plasma ; Prevalence ; Retrospective Studies

Nesidioblastosis is a term used to describe pathologic overgrowth of pancreatic islet cells. It also means maldistribution of islet cells within the ductules of exocrine pancreas. Generally, nesidioblastosis occurs in beta-cell and causes neonatal hyperinsulinemic hypoglycemia or adult noninsulinoma pancreatogenous hypoglycemia syndrome. Alpha-cell nesidioblastosis and hyperplasia is an extremely rare disorder. It often accompanies glucagon-producing marco- and mircoadenoma without typical glucagonoma syndrome. A 35-year-old female was referred to our hospital with recurrent acute pancreatitis. On radiologic studies, 1.5 cm sized mass was noted in pancreas tail. Cytological evaluation with EUS-fine-needle aspiration suggested serous cystadenoma. She received distal pancreatectomy. The histologic examination revealed a 1.7 cm sized neuroendocrine tumor positive for immunohistochemical staining with glucagon antibody. Multiple glucagon-producing micro endocrine cell tumors were scattered next to the main tumor. Additionally, diffuse hyperplasia of pancreatic islets and ectopic proliferation of islet cells in centroacinar area, findings compatible to nesidioblastosis, were seen. These hyperplasia and almost all nesidioblastic cells were positive for glucagon immunochemistry. Even though serum glucagon level still remained higher than the reference value, she has been followed-up without any evidence of recurrence or hormone related symptoms. Herein, we report a case of alpha-cell nesidioblastosis and hyperplasia combined with glucagon-producing neuroendocrine tumor with literature review.
Adult ; Chromogranin A/blood ; Female ; Glucagon/*metabolism ; Glucagon-Secreting Cells/metabolism ; Humans ; Hyperplasia/complications/*diagnosis ; Islets of Langerhans/metabolism/ultrasonography ; Nesidioblastosis/complications/*diagnosis ; Neuroendocrine Tumors/complications/*diagnosis/pathology ; Pancreas/*pathology ; Tomography, X-Ray Computed

No abstract available.
Adenoma, Islet Cell* ; Adult* ; Diabetes Mellitus, Type 2* ; Humans ; Islets of Langerhans* ; Nesidioblastosis*

Hyperinsulinemic hypoglycemia in the absence of exogenous insulin use is caused by disorders such as insulinoma, diffuse beta-cell hyperplasia/nesidioblastosis, and autoimmune hypoglycemia. Nesidioblastosis is a rare cause of hypoglycemia in adults, accounting for 0.5~7.0% of organic hyperinsulinemia cases. Although pancreatic resection is considered the best treatment modality for curing nesidioblastosis, there is no consensus regarding the indications for and extent of the surgery due to its high risk and complication rate. A 75-year-old woman presented with an altered mental state, a mass suspected of being an insulinoma, and insulin receptor antibodies. The patient underwent surgery because of recurrent life-threatening hypoglycemia. Postoperative pathology of her pancreas revealed nesidioblastosis.
Accounting ; Adult ; Aged ; Antibodies ; Consensus ; Female ; Humans ; Hyperinsulinism ; Hypoglycemia ; Insulin ; Insulinoma ; Nesidioblastosis ; Pancreas ; Receptor, Insulin

PURPOSE: Insulinomas and nesidioblastosis are surgically curarable hyperinsulinemic hypoglycemic diseases; however the symptoms, diagnosis, and localization of these diseases are sometimes difficult. METHODS: From April 1990 to December 2008, patients with diagnosis of pancreatic insulinoma and nesidioblastosis were retrospectively reviewed. RESULTS: In the entire cohort, there were 6 patients with multiple endocrine neoplasia type 1 (MEN 1), 7 patients with nesidioblastosis, and 7 patients with multiple lesions. The incidence of neuroglycopenic symptoms (71.5%) was more prevalent than autonomic symptoms (26.5%). The pre-operative localization methods were computed tomography (CT), transabdominal ultrasonography, endoscopic ultrasonography (EUS), magnetic resonance imaging (MRI), angiography, selective arterial calcium stimulation with venous sampling (ASVS), and transhepatic portal venous sampling (THPVS) which had sensitivities of 75%, 40%, 75%, 64%, 48%, 20%, and 87%, respectively. During the second half of the study period, CT had a sensitivity of 83%. THPVS was the most sensitive method of all the diagnostic tools. Intra-operative palpation and ultrasonography were the most powerful modalities for tumor localization. The surgical treatments included enucleation (20%), distal pancreatectomy (51%), central pancreatectomy (12%), pancreaticoduodenectomy (15%), and near-total pancreatectomy (1.5%). From 2006, laparascopic surgery was used as the preferred treatment modality for insulinomas. CONCLUSION: Early diagnosis and precise localization is the most important aspect of treating insulinomas. For better localization, dual phase spiral CT and intra-operative ultrasonography play an important role in tumor localization.
Angiography ; Calcium ; Cohort Studies ; Early Diagnosis ; Endosonography ; Humans ; Incidence ; Insulinoma ; Magnetic Resonance Imaging ; Multiple Endocrine Neoplasia Type 1 ; Nesidioblastosis ; Palpation ; Pancreatectomy ; Pancreaticoduodenectomy ; Retrospective Studies ; Tomography, Spiral Computed

PURPOSE: Insulinomas and nesidioblastosis are surgically curarable hyperinsulinemic hypoglycemic diseases; however the symptoms, diagnosis, and localization of these diseases are sometimes difficult. METHODS: From April 1990 to December 2008, patients with diagnosis of pancreatic insulinoma and nesidioblastosis were retrospectively reviewed. RESULTS: In the entire cohort, there were 6 patients with multiple endocrine neoplasia type 1 (MEN 1), 7 patients with nesidioblastosis, and 7 patients with multiple lesions. The incidence of neuroglycopenic symptoms (71.5%) was more prevalent than autonomic symptoms (26.5%). The pre-operative localization methods were computed tomography (CT), transabdominal ultrasonography, endoscopic ultrasonography (EUS), magnetic resonance imaging (MRI), angiography, selective arterial calcium stimulation with venous sampling (ASVS), and transhepatic portal venous sampling (THPVS) which had sensitivities of 75%, 40%, 75%, 64%, 48%, 20%, and 87%, respectively. During the second half of the study period, CT had a sensitivity of 83%. THPVS was the most sensitive method of all the diagnostic tools. Intra-operative palpation and ultrasonography were the most powerful modalities for tumor localization. The surgical treatments included enucleation (20%), distal pancreatectomy (51%), central pancreatectomy (12%), pancreaticoduodenectomy (15%), and near-total pancreatectomy (1.5%). From 2006, laparascopic surgery was used as the preferred treatment modality for insulinomas. CONCLUSION: Early diagnosis and precise localization is the most important aspect of treating insulinomas. For better localization, dual phase spiral CT and intra-operative ultrasonography play an important role in tumor localization.
Angiography ; Calcium ; Cohort Studies ; Early Diagnosis ; Endosonography ; Humans ; Incidence ; Insulinoma ; Magnetic Resonance Imaging ; Multiple Endocrine Neoplasia Type 1 ; Nesidioblastosis ; Palpation ; Pancreatectomy ; Pancreaticoduodenectomy ; Retrospective Studies ; Tomography, Spiral Computed

We report a 45-year-old man with type 2 diabetes who presented with recurrent hypoglycemia. Biochemical and imagingstudies did not show any mass-like lesion in the pancreas, so prednisolone and diazoxide were administered for the treatment of hypoglycemia. However, the hypoglycemia persisted during and after the medical treatment. A selective arterial calcium stimulation test was performed and revealed a suspicious lesion at the head of the pancreas. The patient underwent enucleation of the pancreas head lesion. The lesion was confirmed histologically to be focal nesidioblastosis and surgical resection was successfully performed. The patient showed no hypoglycemic symptoms postoperatively.
Calcium ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Diazoxide ; Head ; Humans ; Hypoglycemia ; Middle Aged ; Nesidioblastosis ; Pancreas ; Prednisolone

Nesidioblastosis is a rare disorder, and it usually considered as a cause of neonatal hyperinsulinemic hypoglycemia. A 35 year-old-woman with hyperinsulinemic hypoglycemia was admitted in an unconscious condition. Abdominal CT, pancreas MRI and celiac angiography with an intra-arterial calcium stimulation test revealed a suspicious insulin-producing tumorous lesion in the head of pancreas. The patient underwent enucleation of the pancreas head tumor under the initial diagnosis of insulinoma. However, the tumor was confirmed histologically as nesidioblastosis that showed ductoendocrine proliferations and numerous small endocrine cell groups. Nesidioblastosis is classified into a focal type and a diffuse type, which are characterized by different clinical outcomes. The patient in our case showed a normal blood glucose level after operation, which is often the case for the focal type. Herein, we report this very rare case of adult nesiodioblastosis that was successfully treated by surgical resection.
Adult ; Angiography ; Blood Glucose ; Calcium ; Endocrine Cells ; Head ; Humans ; Hyperinsulinism ; Hypoglycemia ; Insulinoma ; Nesidioblastosis ; Pancreas ; Unconscious (Psychology)