Endocrine pancreatic neoplasia

Focal endocrine differentiation, especially in the form of scattered cells, is quite common in pancreatic neoplasia of ductal and acinar nature and is of no known biologic significance. However, if a tumor is predominantly composed of cells with endocrine lineage, it is classified as endocrine. ... 

The clinical presentation of patients with endocrine pancreatic tumors will vary according to the type of hormone released. Hyperinsulinism manifested with diarrhea, abdominal pain, and low levels of glucose will be found in the majority of patients. Nesidioblastosis is a primitive pancreatic B cells hyperplasia. Approximately 5% of patients with hyperinsulinism may have this type of tumor. Hunger, jitteriness, lethargy, apnea, and seizures are common manifestations in newborns with nesidioblastosis, while older children may show diaphoresis, confusion, or unusual behavior. Zollinger-Elli-son syndrome will present with intractable peptic ulcers. Patients with vipomas will have watery diarrhea, hypokalemia, and achlorhydria, while multiple endocrine neoplasias have been reported with multiple endocrine neoplasia type 1 (MEN 1). 

The initial hazard identification of pancreatic toxicity in a prechnical study is always concerning because of the potentially serious human health risks, which may include fulminant, hfe-threatening cases of DIAP or a lifelong iUness, such as CP, type 1 diabetes, or pancreatic neoplasia. Because both the exocrine and the endocrine pancreas have enormous reserve capacities and there are limited sensitive and specific biomarkers to detect pancreatic injury in both animals and humans, there is always the concern that subclinical DIAP may initially go undetected in humans. Further, AP may progress until a threshold of severity is evident in minimally or asymptomatic healthy human volunteers exposed to drug candidates with unknown risks and/or in previously healthy patients that receive standard-of-care agents with marked... 

Multiple endocrine neoplasia type I with pituitary and pancreatic tumors... 

There is epidemiologic evidence to suggest an increased prevalence of duodenal ulcers in patients with certain chronic diseases, but the pathophysiologic mechanisms of these associations are uncertain. A strong association exists in patients with systemic mastocytosis, multiple endocrine neoplasia type 1, chronic pulmonary diseases, chronic renal failure, kidney stones, hepatic cirrhosis, and ai-antitrypsin deficiency. An association may exist in patients with cystic fibrosis, chronic pancreatitis, Crohn s disease, coronary artery disease, polycythemia vera, and hyperparathyroidism. 

Le Bodic M-F, Heyman M-F, Lecomete M, et al. Immuno histochemical study of 100 pancreatic tumors in 28 patients with multiple endocrine neoplasia type I. Am J Surg Pathol. 1996 20 1378-1384. 

Pancreatic endocrine neoplasms (PENs) are the majority of the endocrine neoplasms in the pancreas.Most PENs are sporadic. However, these tumors also constitute one of the major components of the multiple endocrine neoplasia I (MENl) syndrome or may arise in patients with vHL syndrome. Those that are associated with increased serum levels of hormones and lead to corresponding symptoms are referred to as functional and are named according to which hormone they secrete (e.g., insulinoma—42% of all functional variants, gastrinoma—24%, glucagonoma—14%,... 

This is one differential in which immunohistochemistry can be of utmost importance. Numerous cytologic and architectural similarities exist between HCC and metastatic well-differentiated neuroendocrine neoplasia (carcinoids and pancreatic endocrine neoplasia) They are both cellular, stroma-poor tumors that have a delicate sinusoidal vasculature and relatively monotonous cells with fair amount of cytoplasm and round nuclei. A panel of "neuroendocrine markers" (chromogranin, synapto-physin, and CD56) combined with "hepatocytic" markers (Hep Par-1 and polyclonal-CEA, and FISH for albumin) can be helpful. 

In the pediatric population, pancreatic tumors are very unusual entities. They account for less than 5% of all malignancies in children. These tumors may arise from the exocrine or endocrine pancreas. Exocrine neoplasias include ductal adenocarcinoma, acinar adenocarcinoma, pancreaticoblastoma, or infantile adenocarcinoma. Endocrine tumors or islet cell tumors are named after the hormone produced, with insulinoma being the most common in children (Walker 1996). 

Zollinger-Ellinson syndrome is a severe form of peptic ulcer disease related to the presence of a gastrin secreting tumour. These tumours can develop from pancreatic islets or the endocrine cells of duodenal or gastric mucosa and therefore may form part of multiple endocrine neoplasia type I (MENl)... 

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