glucagonoma

Last reviewed 01/2018

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:

1. de Herder WW and Lamberts SWJ. Best Practice & Research Clinical Endocrinology & Metabolism 2004; Volume 18(4): 477-495.