- Pancreas is derived embryologically from the foregut
- Has both exocrine and endocrine components
- The endocrine component is within the Islets of Langerhans
- Alpha cells secrete glucagon
- Beta cells secrete insulin
- Delta cells secret somatostatin
- Endocrine tumours arise from the Islets of Langerhans
- They may produce hormones
- May be associated with Multiple Endocrine Neoplasia (MEN) syndromes
Insulinomas
- Insulinomas are rare
- The annual incidence in the UK is 1-2 per million population
- They are usually solitary
- They can occur at any age
- Slightly more common in women
- 90% less than 2 cm in diameter
- 90% are benign
- 10% associated with MEN Type 1 syndrome
Clinical features
- Symptoms are non-specific and variable
- May be induced by exercise
- Symptoms are those of hypoglycaemia
Investigation
- Diagnosis can be difficult
- Need to demonstrate hypoglycaemia in the presence of symptoms
- Insulin levels are normal or raised
- C-peptide levels are usually increased
- Diagnosis can be confirmed by CT
Management
- Resection offer the only chance of cure
- 10 year survival rates are over 90%
- Hepatic artery embolisation and chemotherapy maybe required in
metastatic disease
Gastrinomas
- Gastrinomas occur in both the duodenum and pancreas
- Gastrin over production results in Zollinger-Ellison syndrome
- Presents with severe peptic ulcer disease and diarrhoea
- 20% patients have MEN type 1 disease
- Gastric acid hypersecretion can be controlled by either PPI or
surgery
- Historically total gastrectomy was performed and the tumour was left
in situ
- Now the tumour is removed by either
- Distal pancreatectomy
- Enucleation
- Duodenectomy
- Whipple's procedure
- Patients with metastatic disease require
- Chemotherapy
- Alpha-interferon
Glucagonomas
- Glucagonomas are rare
- Occur as part of the MEN type 1 syndrome
- Metastatic disease often present at presentation
- Symptoms are often non-specific
- Patients may have a characteristic rash and mucositis
- Diagnosis confirmed by the detection of raised serum glucagon
- Surgery may be beneficial even in the presence of metastatic disease
- Octreotide may help control symptoms
Bibliography
Azimuddin K, Chamberlain R S. The surgical management of
pancreatic neuroendocrine tumors. Surg Clin North Am 2001;
81: 511-525.
Finlayson E, Clarke O H. Surgical treatment of insulinomas.
Surg Clin North Am 2004; 84: 775-785.
Norton J A, Jensen R T. Resolved and unresolved
controversies in the surgical management of patients with
Zollinger-Ellison syndrome. Ann Surg 2004; 240:
757-773.
Tucker O N, Crotty P L, Conlon K C. Management of
insulinoma. Br J Surg 2006; 93: 264-275 |