BACKGROUND Nesidioblastosis usually refers to a series of clinical manifestations caused by the proliferation ofβ-cells in pancreatic islets,and these clinical manifestations are hyperinsulinemia and persistent hypog...BACKGROUND Nesidioblastosis usually refers to a series of clinical manifestations caused by the proliferation ofβ-cells in pancreatic islets,and these clinical manifestations are hyperinsulinemia and persistent hypoglycemia.According to the size of the lesion,nesidioblastosis is divided into focal nesidioblastosis,diffuse nesidioblastosis and atypical nesidioblastosis,and its pathogenesis is still unclear.Nesidioblastosis is mainly seen in infants and rarely reported in adults,especially focal nesidioblastosis,which is difficult to distinguish from insulinoma.CASE SUMMARY We report a case of adult focalβ-cell nesidioblastosis in which the preoperative diagnosis was insulinoma.The patient was a 48-year-old male who suffered from repeated morning and fasting palpitations,sweating,and severe disturbance of consciousness for 5 years.His blood glucose was found to be as low as 1.79 mmol/L during an attack.However,abdominal computed tomography showed no abnormalities.Magnetic resonance imaging and endoscopic ultrasonography demonstrated a nodular mass in the head of the pancreas,combined with hyperinsulinemia and high serum C-peptide.The patient was diagnosed with insulinoma and underwent Beger surgery;however,the postoperative pathological results showed nesidioblastosis.CONCLUSION Although surgical resection is the preferred option for nesidioblastosis,some cases can be treated non-surgically.In order to increase clinicians’understanding of nesidioblastosis,it is necessary to review the pathogenesis,diagnosis and treatment of this disease.展开更多
In neonates, persistent hyperinsulinemic hypoglycemia (PHH) is associated with nesidioblastosis. In adults, PHH is usually caused by solitary benign insulinomas. We report on an adult patient who suffered from insulin...In neonates, persistent hyperinsulinemic hypoglycemia (PHH) is associated with nesidioblastosis. In adults, PHH is usually caused by solitary benign insulinomas. We report on an adult patient who suffered from insulin-de- pendent diabetes mellitus, and subsequently developed PHH caused by diffuse nesidioblastosis. Mutations of the MEN1 and Mody 2/3 genes were ruled out. Preoperative diagnostic procedures, the histopathological criteria and the surgical treatment options of adult nesidioblastosis are discussed. So far only one similar case of adult nesidioblastosis subsequent to diabetes mellitus Ⅱ has been reported in the literature. In case of conversion of diabetes into hyperinsulinemic hypoglycemia syndrome, nesidioblastosis in addition to insulinoma should be con- sidered.展开更多
Persistent hyperinsulinemic hypoglycemia is caused most commonly by an insulinoma in adults or by nesidioblastosis in neonates. In adults, nesidioblastosis is a rare disorder characterized by diffuse or disseminated p...Persistent hyperinsulinemic hypoglycemia is caused most commonly by an insulinoma in adults or by nesidioblastosis in neonates. In adults, nesidioblastosis is a rare disorder characterized by diffuse or disseminated proliferation of islet cells. We recently encountered a case of nesidioblastosis in an adult. A 71-year-old man was admitted due to intermittent general weakness, abdominal pain, and mild dyspnea. The patient underwent a subtotal gastrectomy for a gastric adenocarcinoma two years ago. After 5 d of admission, the patient showed symptoms of cold sweating, chilling, and hypotension 30 min after eating. Thereafter, he frequently showed similar symptoms accounting for hypoglycemia regardless of food consumption. Laboratory findings revealed a low fasting blood glucose level （25 mg/dL）, and a high insulin level （47 μIU/mL）. Selective intra-arterial calcium stimulation with hepatic venous sampling （ASVS） was performed to localize a mass and revealed an increased insulin level about fourfold that of the normal fasting level at 60 s in the splenic artery, which suggested the presence of an insulinoma in the tail of pancreas. A distal pancreatectomy was performed. Neither intraoperative exploration nor a frozen biopsy specimen detected any mass-forming lesion. On the histological examination, many of the islets were enlarged and irregularly shaped in all specimens, the arrangement of which was a Iobulated islet pattern. Cytologically, a considerable subpopulation of endocrine cells showed enlarged and hyperchromatic nuclei. By immunohistochemistry, the cells were identified as p-cells. These clinical, radiological, microscopic and immuno-histochemical findings are consistent with diffuse nesidioblastosis in adults.展开更多
Insulinoma is a pancreatic endocrine tumor lower in size than 20 mm in 80% of the cases and his treatment is chirurgical. However, in certain circumstances such as an occult location or circumstances of metastases, me...Insulinoma is a pancreatic endocrine tumor lower in size than 20 mm in 80% of the cases and his treatment is chirurgical. However, in certain circumstances such as an occult location or circumstances of metastases, medical treatment is called for. Observation: A 29 years old patient with no specific pathological antecedents has presented severe hypoglycemia mainly in the morning. A patient was in a generally good condition. The fasting test revealed an inappropriate secretion of insulin at a venous glycemia of 0.35 g/l;which was corroborated by Turner index and altered glucose insulin index that we calculated. Moreover, the 8 h cortisolemia was normal at 90.13 ng/l, the TSH was normal at 1.44 μui/l, anti-insulin antibodies were negative at 6.7 U/l;the search of hypoglycemic sulfonamides was negative. Morphologically, she had three pancreatic tomodensitometry these were normal. She also had echo-endoscopy which showed a normal pancreas. The surgical exploration with preoperative echo is advised only after surgeon’s assessment when the technical conditions are not put together. The diagnosis of the occult insulinoma or of nesidioblastosis was retained. The medical treatment was retained. Due to the unavailability of diazoxide in our pharmacies and the high cost of analogs of somatostatine, she was provided with prednisone 0.5 mg/kg/24h which was 40 mg/day after common agreement. The evolution was favorable. Conclusion: It should be noticed that medical treatment can be suggested if insulinoma is not localized. This observation proves that the localization of the insulinoma can be unsuccessful. It should also be noticed that our experience is the fourth described in literature, where hypoglycemia in insulinomas is controlled by prednisone.展开更多
Pathologic hyperplasia of various pancreatic endocrine cells is rare but has been long known.β cell hyperplasia contributes to persistent hyperinsulinemic hypoglycemia of infancy,which is commonly caused by mutations...Pathologic hyperplasia of various pancreatic endocrine cells is rare but has been long known.β cell hyperplasia contributes to persistent hyperinsulinemic hypoglycemia of infancy,which is commonly caused by mutations in the islet ATP-sensitive potassium channel,and to noninsulinoma pancreatogenous hypoglycemia in adults,which may or may not be associated with bariatric surgery.α cell hyperplasia may cause glucagonoma syndrome or induce pancreatic neuroendocrine tumors.An inactivating mutation of the glucagon receptor causes α cell hyperplasia and asymptomatic hyperglucagonemia.Pancreatic polypeptide cell hyperplasia has been described without a clearly-characterized clinical syndrome and hyperplasia of other endocrine cells inside the pancreas has not been reported to our knowledge. Based on morphological evidence,the main pathogenetic mechanism for pancreatic endocrine cell hyperplasia is increased endocrine cell neogenesis from exocrine ductal epithelium.Pancreatic endocrine cell hyperplasia should be considered in the diagnosis and management of hypoglycemia,elevated islet hormone levels,and pancreatic neuroendocrine tumors.Further studies of pathologic pancreatic endocrine cell hyperplasia will likely yield insights into the pathogenesis and treatment of diabetes and pancreatic neuroendocrine tumors.展开更多
文摘BACKGROUND Nesidioblastosis usually refers to a series of clinical manifestations caused by the proliferation ofβ-cells in pancreatic islets,and these clinical manifestations are hyperinsulinemia and persistent hypoglycemia.According to the size of the lesion,nesidioblastosis is divided into focal nesidioblastosis,diffuse nesidioblastosis and atypical nesidioblastosis,and its pathogenesis is still unclear.Nesidioblastosis is mainly seen in infants and rarely reported in adults,especially focal nesidioblastosis,which is difficult to distinguish from insulinoma.CASE SUMMARY We report a case of adult focalβ-cell nesidioblastosis in which the preoperative diagnosis was insulinoma.The patient was a 48-year-old male who suffered from repeated morning and fasting palpitations,sweating,and severe disturbance of consciousness for 5 years.His blood glucose was found to be as low as 1.79 mmol/L during an attack.However,abdominal computed tomography showed no abnormalities.Magnetic resonance imaging and endoscopic ultrasonography demonstrated a nodular mass in the head of the pancreas,combined with hyperinsulinemia and high serum C-peptide.The patient was diagnosed with insulinoma and underwent Beger surgery;however,the postoperative pathological results showed nesidioblastosis.CONCLUSION Although surgical resection is the preferred option for nesidioblastosis,some cases can be treated non-surgically.In order to increase clinicians’understanding of nesidioblastosis,it is necessary to review the pathogenesis,diagnosis and treatment of this disease.
文摘In neonates, persistent hyperinsulinemic hypoglycemia (PHH) is associated with nesidioblastosis. In adults, PHH is usually caused by solitary benign insulinomas. We report on an adult patient who suffered from insulin-de- pendent diabetes mellitus, and subsequently developed PHH caused by diffuse nesidioblastosis. Mutations of the MEN1 and Mody 2/3 genes were ruled out. Preoperative diagnostic procedures, the histopathological criteria and the surgical treatment options of adult nesidioblastosis are discussed. So far only one similar case of adult nesidioblastosis subsequent to diabetes mellitus Ⅱ has been reported in the literature. In case of conversion of diabetes into hyperinsulinemic hypoglycemia syndrome, nesidioblastosis in addition to insulinoma should be con- sidered.
文摘Persistent hyperinsulinemic hypoglycemia is caused most commonly by an insulinoma in adults or by nesidioblastosis in neonates. In adults, nesidioblastosis is a rare disorder characterized by diffuse or disseminated proliferation of islet cells. We recently encountered a case of nesidioblastosis in an adult. A 71-year-old man was admitted due to intermittent general weakness, abdominal pain, and mild dyspnea. The patient underwent a subtotal gastrectomy for a gastric adenocarcinoma two years ago. After 5 d of admission, the patient showed symptoms of cold sweating, chilling, and hypotension 30 min after eating. Thereafter, he frequently showed similar symptoms accounting for hypoglycemia regardless of food consumption. Laboratory findings revealed a low fasting blood glucose level （25 mg/dL）, and a high insulin level （47 μIU/mL）. Selective intra-arterial calcium stimulation with hepatic venous sampling （ASVS） was performed to localize a mass and revealed an increased insulin level about fourfold that of the normal fasting level at 60 s in the splenic artery, which suggested the presence of an insulinoma in the tail of pancreas. A distal pancreatectomy was performed. Neither intraoperative exploration nor a frozen biopsy specimen detected any mass-forming lesion. On the histological examination, many of the islets were enlarged and irregularly shaped in all specimens, the arrangement of which was a Iobulated islet pattern. Cytologically, a considerable subpopulation of endocrine cells showed enlarged and hyperchromatic nuclei. By immunohistochemistry, the cells were identified as p-cells. These clinical, radiological, microscopic and immuno-histochemical findings are consistent with diffuse nesidioblastosis in adults.
文摘Insulinoma is a pancreatic endocrine tumor lower in size than 20 mm in 80% of the cases and his treatment is chirurgical. However, in certain circumstances such as an occult location or circumstances of metastases, medical treatment is called for. Observation: A 29 years old patient with no specific pathological antecedents has presented severe hypoglycemia mainly in the morning. A patient was in a generally good condition. The fasting test revealed an inappropriate secretion of insulin at a venous glycemia of 0.35 g/l;which was corroborated by Turner index and altered glucose insulin index that we calculated. Moreover, the 8 h cortisolemia was normal at 90.13 ng/l, the TSH was normal at 1.44 μui/l, anti-insulin antibodies were negative at 6.7 U/l;the search of hypoglycemic sulfonamides was negative. Morphologically, she had three pancreatic tomodensitometry these were normal. She also had echo-endoscopy which showed a normal pancreas. The surgical exploration with preoperative echo is advised only after surgeon’s assessment when the technical conditions are not put together. The diagnosis of the occult insulinoma or of nesidioblastosis was retained. The medical treatment was retained. Due to the unavailability of diazoxide in our pharmacies and the high cost of analogs of somatostatine, she was provided with prednisone 0.5 mg/kg/24h which was 40 mg/day after common agreement. The evolution was favorable. Conclusion: It should be noticed that medical treatment can be suggested if insulinoma is not localized. This observation proves that the localization of the insulinoma can be unsuccessful. It should also be noticed that our experience is the fourth described in literature, where hypoglycemia in insulinomas is controlled by prednisone.
文摘Pathologic hyperplasia of various pancreatic endocrine cells is rare but has been long known.β cell hyperplasia contributes to persistent hyperinsulinemic hypoglycemia of infancy,which is commonly caused by mutations in the islet ATP-sensitive potassium channel,and to noninsulinoma pancreatogenous hypoglycemia in adults,which may or may not be associated with bariatric surgery.α cell hyperplasia may cause glucagonoma syndrome or induce pancreatic neuroendocrine tumors.An inactivating mutation of the glucagon receptor causes α cell hyperplasia and asymptomatic hyperglucagonemia.Pancreatic polypeptide cell hyperplasia has been described without a clearly-characterized clinical syndrome and hyperplasia of other endocrine cells inside the pancreas has not been reported to our knowledge. Based on morphological evidence,the main pathogenetic mechanism for pancreatic endocrine cell hyperplasia is increased endocrine cell neogenesis from exocrine ductal epithelium.Pancreatic endocrine cell hyperplasia should be considered in the diagnosis and management of hypoglycemia,elevated islet hormone levels,and pancreatic neuroendocrine tumors.Further studies of pathologic pancreatic endocrine cell hyperplasia will likely yield insights into the pathogenesis and treatment of diabetes and pancreatic neuroendocrine tumors.
