Glucagonoma

Epidemiology of glucagonoma
The estimated incidence of glucagonomas is 1 per 20 million of the population per year. Glucagonomas are uncommon, representing approxametely 10% of all functioning pancreatic NETs. The tumors are usually diagnosed between the ages of 40 and 70 years.

Symptoms of glucagonoma
The syndrome reflects the catabolic effect of excess glucagon production. The most characteristic symptom is a skin rash called necrolytic migratory erythema, starting in the groins and ingues, and migrating to extremities (Fig.3). The syndrome also includes mild glucose intolerance, anemia, weight loss, sometimes cachexia and trombo-embolic complications. Glucagonomas are typically located in the distal pancreas, and are often large, between 4-15 cm. Approximately 60-70% of glucagonomas have metastasized at the time of diagnosis.

Diagnosis is settled by demonstration of raised plasma glucagon and is often delayed, even in presence of a typical skin lesion. The patients can have marked palliation by nutritional supplement, which can heal the skin lesion prior to surgery.

FiG. 3 Typical skin rash, migrating necrolytic erythema in gucagonoma patient
FiG. 3 Typical skin rash, migrating necrolytic erythema in gucagonoma patient

 

Treatment of glucagonoma
The treatment is operation, and during surgery the patients require antithrombotic medication because of high risk for thrombosis and pulmonary embolism. Treatment is left-sided subtotal pancreatic resection and clearance of regional lymph node metastases. Disease progression may be slow despite metastases. The patients can often during an extended disease course require sequential excision of lymph nodes and liver metastases with 5-10 years or more between recurrent lesions.