review of literature on clinical pancreatology - The Pancreapedia

Insulinomas are rare neuroendocrine tumours with an incidence <strong>of</strong> four cases per million ayear. Only few cases <strong>of</strong> insulinoma in patients with preexisting diabetes mellitus have beenreported. It was presented a 50-year-old male with type 2 diabetes mellitus who sufferedfrom recurring hypoglycemia. He had gained 20 kilograms <strong>of</strong> weight in five years. 72-hourfast revealed hypoglycaemia in the presence <strong>of</strong> inadequately high C-peptide and insulinlevels. Magnetic resonance imaging and selective arterial calcium stimulation test confirmeda mass in the body <strong>of</strong> the pancreas. The tumor was removed surgically. Pathologicalexamination demonstrated a benign insulinoma. Postoperatively, blood glucose levels werewithin the therapeutic range. The HbA (1c) value was 6.8 percent three months after theintervention. Clinicians should be alert to insulinoma as a, though rare, differential diagnosis<strong>of</strong> hypoglycaemia in diabetes, in particular in patients with recurrent, otherwise unexplainedhypoglycaemia [678].Non-functioning tumorA 52-year-old man was admitted for detailed examination <strong>of</strong> a mass with extensivecalcification in the tail <strong>of</strong> the pancreas by fluoro-deoxy glucose-positron emissiontomography/computed tomography (FDG-PET/CT). Abdominal CT and magnetic resonanceimaging (MRI) findings showed a calcified tumor 5 cm in diameter with a smooth surface. Thetumor mainly showed calcification at it center and a partially solid element around it marginwhich was enhanced in the early phase. Pathological and immunohistochemical studiesrevealed a nonfunctioning islet cell tumor with calcification. A nonfunctioning islet cell tumorwith central calcification formation as it grew to a maximum diameter <strong>of</strong> 7 cm is rare. Whendiagnosing pancreatic tumors it must be kept in mind that some nonfunctioning islet celltumors <strong>of</strong> the pancreas can show nontypical features such as calcification formation [679].Tumors <strong>of</strong> the papilla <strong>of</strong> VaterEndocrine tumors <strong>of</strong> the ampullary region are rare, and accurate indications for theirmanagement are lacking. It was <strong>review</strong>ed all patients who 1982-2003 were submitted to apancreaticoduodenectomy for ampullary endocrine tumors. Eight patients, 3 men and 5women, with a mean age <strong>of</strong> 48 years were included. Two patients presented with Zollinger-Ellison syndrome, and 1 had neur<strong>of</strong>ibromatosis. Operative mortality was nil. The mean size<strong>of</strong> the tumors was 17 mm (range, 5-40 mm). There were 7 well-differentiated and 1 poorlydifferentiated endocrine carcinomas. Seven patients had satellite lymph node metastases,and 1 had diffuse liver metastases. Median follow-up was 131 months (range, 17-315months). At the end <strong>of</strong> the follow-up period, 5 patients were alive and disease-free; 1 patientwas alive with stable liver metastases. Two patients died 17 months and 13 years aftersurgery, respectively, from metastasis and an unrelated cause. This study demonstrates thehigh frequency <strong>of</strong> lymph node invasion in these uncommon tumors, even at an early clinicalstage. Pancreaticoduodenectomy may result in prolonged survival <strong>of</strong> patients with welldifferentiatedtumors [680].SurgerySurgery represents the only chance <strong>of</strong> cure for a patient with a neuroendocrine tumour(NET). The main indications for surgery lie in the risk <strong>of</strong> developing metastatic disease withincreasing tumour diameter and for a functioning NET also in control <strong>of</strong> the hormonalsyndrome. However, only a small minority <strong>of</strong> patients presents with a potentially resectableprimary NET without metastatic disease. An R0-resection is mandatory, which may be