Search

Pancreatogenous hyperinsulinemic hypoglycemia (PHH) is a rare disorder determined by an abnormally high secretion of insulin in the pancreas, in the absence of other medical or pharmacological factors. Either β-cell tumors (insulinomas) or β-cell hyperplasia (nesidioblastosis) can determine this pathology. Most publications on insulinomas or nesidioblastosis approached these subjects from a clinical point of view. This paper aims to analyze pathological aspects underlying pancreatogenous hyperinsulinemic hypoglycemia. We present two cases of insulinomas with unusual pancreatic localization and size, one of them showing amyloid deposits in the stroma. In both cases, immunohistochemistry confirmed the clinical and imagistic supposition. The third reported case refers to a 57-year-old patient with nesidioblastosis with isolated disposition of endocrine cells and areas of focal organization, both morphological aspects being extremely rare in adults. Although clinical and laboratory data are usually identical in the two forms of PHH, histopathological and immunohistochemical diagnosis is essential in differentiating insulinomas from nesidioblastosis, as the surgical management is different: enucleation for insulinomas and total or subtotal pancreatectomy for nesidioblastosis.

A 2-day-old Simmental calf with arthrogryposis and astasia was subjected to necropsy examination. The calf was normoglycaemic and normoinsulinaemic. Microscopically, pancreatic tissue was hyperplastic with an irregular lobular arrangement of pancreatic islets. Newly-formed islet cells budded from intralobular or intercalated ducts (so-called ductulo-insular complexes) and there were prominent blood vessels with telangiectatic features surrounded by rows of cuboidal-columnar islet cells. The newly-formed islets expressed insulin antigen immunohistochemically. The lesion was diagnosed as nesidioblastosis, an uncommon abnormality previously associated with the double muscling trait in cattle., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Over the past 20 years bariatric surgery proved to be a valid treatment for reduction and elimination of obesity-related diseases and long-term sustainable weight loss. Minimally invasive or laparoscopic techniques such as laparoscopic Roux-en-Y (LRNY) have replaced open procedures. Many factors play important roles in the small intricacies and variations of the procedure, chief of which is the creation and size of the gastrojejunostomy. Regardless of the variations in technique, the LRNY remains the gold standard for the surgical treatment of clinically severe or morbid obesity, with relatively low morbidity and mortality., (Copyright © 2011. Published by Elsevier Inc.)

Objective: To report a case of metastatic insulinoma presenting 11 yr after enucleation of an isolated insulinoma, and 5 yr after distal pancreatectomy for nesidioblastosis., Methods: We present the clinical, laboratory, radiological and pathological findings in a 34 yr-old-man with recurrent hypoglycemia. The pertinent literature is reviewed., Results: A 34- yr-old man presented in 1992 with symptoms of recurrent hypoglycemia. Laboratory and imaging findings were consistent with insulinoma. After enucleation of the tumor, his symptoms resolved. In 1998 hypoglycemia recurred and biochemical work-up was consistent with endogenous hyperinsulinism. Imaging for recurrent or metastatic insulinoma revealed no mass. He underwent a distal pancreatectomy and pathology revealed islet cell hyperplasia, or nesidioblastosis. However, the patient reported minimal symptomatic improvement. He was started on diazoxide but was poorly compliant and ate frequently to avoid hypoglycemic symptoms. In 2003 he presented with hypoglycemia-induced seizure activity. Imaging showed hepatic and pulmonary lesions, but no pancreatic mass. An octreotide scan revealed increased hepatic uptake and fine needle aspiration of a liver lesion confirmed metastatic insulinoma. Arterial-stimulation venous sampling revealed increased insulin output from the liver and normal insulin output from the pancreas. After failure of medical therapy, radiofrequency ablation of hepatic lesions was performed with subsequent improvement in clinical symptoms. The patient was discharged on a low dose of dexamethasone., Conclusion: Although this patient had nesidioblastosis, his recurrent hyperinsulinism was most likely secondary to metastatic insulinoma. Radiofrequency ablation was successfully used for palliative purposes in managing metastatic insulinoma refractory to medical therapy.

Background: Nesidioblastosis is often resistant to medical therapy and requires near-total pancreatectomy. There is little information on the postoperative imaging findings of these patients., Objective: To demonstrate by US the late imaging findings in these patients., Materials and Methods: Children diagnosed with nesidioblastosis and who had undergone 90-95% pancreatectomy received preoperative, immediate-postoperative (within 10 days of surgery) and long-term annual US examinations. In the preoperative study, three anterior-posterior (AP) measurements were obtained of the head, body and tail of the pancreas. In the postoperative and long-term follow-up US, AP and transverse measurements of the pancreatic remnant were obtained. Pancreatic echogenicity was also assessed. The results were compared with normal pancreatic dimensions as a function of age. Glucose metabolism and pancreatic enzymes were also analysed., Results: The study group comprised 22 patients (aged 9 days to 2 years). The pancreas was normal in all preoperative US examinations. The first postoperative examination showed, in all patients, a remnant of the pancreatic head measuring 8-13 mm. The last follow-up US was similar to the first postoperative study in 6 patients, and 12 showed complete pancreatic regeneration (normal size, echogenicity and function), and 4 had incomplete regeneration with head and body normal in size, but lack of a pancreatic tail. All patients were asymptomatic and showed normal laboratory tests., Conclusions: US measurements indicated normal age-dependent growth after near-total resection in 54% of patients. The function and echogenicity of the regenerated pancreas indicate that the increase in organ size was due to normal pancreatic tissue.