All of the following are true about glucagnoma except:
1- The tumore arises from the alpha cells of the pancreas.
2- The tumor most often arise from the tail of the pancrease.
3- Necrolytic migratory erythema rash is specific to glucagonoma.
4- Glucagon level > 1000pg/ml is diagnostic.
5- CT abdomen with contrast is intial study for localization.
6- Endoscopic US of pancreas will help both the localization and obtaining biopsy of the tumor.
7- It is among endocrine tumors that is uniquely associated with venous thromboembolism.
All are true except 3, this rash can occur in other conditions, however it is commonly the clue to this rare diagnosis. Weight loss and diabetes mellitus are common as well. Neuropsychiatric symptoms are common. The diagnosis is usally established late after the tumor have metastasized. The diagnosis is usually made after the skin rash occured and glucagon level is commonly quite elevated > 500 the normal is 100 level can be at high normal in few patients. CT is usually the test ordered to localize the tumor and potential metastasis which are commonly to the liver. Octreotoide scan will also show the tumor and metastasis however rarely needed since the CT will visualize the tumor in most cases. The second study is usually through endoscopic US that can identify tumors and as well allow needle biopsy of the tumors in the pancreas. In the early cases with no metastasis surgical removal of the tumor is done. In hepatic predominant disease from metastasis resection of hepatic metastasis or hepatic artery embolization may be effective in slowing the disease. Octretoide is quite effective in controlling the symptoms however regress of tumor is not clearly shown. Alpha interferon and chemotherapy are other options. Disease is monitored regularly with serum glucagon and CT. It is not uncoomon to have 5 year survival of 50% in metastatic slowly progressing disease.
