Pancreatic endocrine neoplasms

• Also referred to as ‘islet cell tumours;
• Rare accounting for 2% of all pancreatic neoplasms
• Have an appearance which resembles carcinoid tumours
• May be single, multiple, benign or malignant. If malignant they metastasise to the lymph nodes and liver
• May be non-functional but most produce pancreatic hormones

 

• The three most common and distinctive clinical syndrome associated with functional pancreatic endocrive neoplasms are;
  • Hyperinsulinaemia
  • Hypergastraemia and Zollinger-Ellison syndrome
  • Multiple endocrine neoplasia

 

Hyperinsulinism (Insulinoma)

• Î’-cell tumours are the most common pancreatic endocrine neoplasia
• Generally benign
• They result in a clinical triad;
  • Attacks of hypoglycaemia with blood glucose levels below 40mg/dL
  • The attacks consist of CNS manifestations – confusion, stupor, LOC
  • Attacks are precipitated by fasting or exercise and are relieved quickly by glucose administration

 

Zollinger-Ellison syndrome

• Hypersecretion of gastrin
• May also arise from the duodenum and peripancreatic soft tissues as well as the pancreas
• The hypersecretion of gastric acid causes peptic ulcers in around 90% of patients
• Over half are invasive and metastasise
• In around 25% of patients gastromas arise in conjunction with other endocrine tumours thus conforming to MEN-1 syndrome
• More than 50% of patients have diarrhoea
• Treatment involves PPI’s and surgical excision

 

Other rare pancreatic endocrine neoplasms

 

α- cell tumours (glucagonomas)

• Associated with increased serum levels of glucagons and a syndrome resembling mild diabetes, a characteristic skin rash called necrolytic migratory erythema and anaemia
• Most often occurs in menopausal or peri-menopausal women

 

δ- cell tumours (somatostatinomas)

• Associated with diabetes mellitus, cholelithiasis, steatorrhoea and hypochlorhydria (low levels of gastric acid)

 

VIPoma

• WHDA syndrome – watery diarrhoea, hypokalaemia, achlohdyria (absent gastric acid)

 

• Some pancreatic and extrapancreatic endocrine tumours can produce two or more hormones usually simulataneously. In addition to glucogon, insulin and gastrin, they can also produce, ACTH, MSH, vasopressin, serotonin and noradrenalin