This is a result of a tumour of the alpha 2 cells of the islets of Langerhans
- the majority of these tumours are malignant
- about 90% of patients already have liver or lymph node metastases at presentation
- 5–20% of glucagonomas occur as component of MEN-I syndrome
This tumour may present with attacks of hyperglycaemia (diabetes mellitus occurs in more than 50% of cases), anaemia, rash and diarrhoea.
Necrolytic migratory erythema is associated with this condition (in more than 70% of patients)
- may also affect the mucous membranes, leading to cheilitis, glossitis and stomatitis.
Other possible features include:
- weight loss or cachexia in more than 60% of patients
- psychiatric disturbances, such as depression or psychosis
- tendency to venous thrombosis in about 11% of patients
Elevated fasting plasma glucagon levels are present in all patients. Also raised pancreatic polypeptide values are present in approximately 50% of patients.
Tumour localization is via techniques such as transabdominal ultrasonography, CT, MRI, selective abdominal angiography, endoscopic ultrasonography or somatostatin receptor scintigraphy.
Management:
- directed by a specialist
- insulin therapy may be required for diabetics
- aspirin therapy has been used as thrombosis prophylaxis
- octreotide is effective in controlling the rash - however this treatment modality is less effective in the management of diabetes mellitus and weight loss
- octreotide is ineffective in reducing the incidence of venous thrombosis
- combination chemotherapy is often given in advanced disease
- surgical removal may be curative only for patients with local, benign disease
- single or repeated hepatic artery embolization of the metastases initially results in symptomatic relief in the majority (more than 80%) of patients - however in more than 50% of patients the symptoms will aggravate within 6 months
Notes:
- Zollinger-Ellison may also develop many years after the initial diagnosis (as part of MEN-I) - annual gut hormone screening has been recommended
Reference:
- de Herder WW and Lamberts SWJ. Best Practice & Research Clinical Endocrinology & Metabolism 2004; Volume 18(4): 477-495.