Glucagonoma : Differential

Comments:
Differential Diagnosis of Glucagonoma: The skin lesions (necrolytic migratory erythema, NME) of glucagonoma can precede the full-blown clinical syndrome by several years. In the absence of other clinical features, NME is often misdiagnosed as pemphigus foliaceus, pemphigoid, vasculitis, acrodermatitis enteropathica, psoriasis, herpes, seborrheic or contact dermatitis, or eczema.

Elevated glucagon levels without glucagonoma syndrome may be seen in an autosomal recessive benign condition called glucagon cell hyperplasia and neoplasia (GCHN) resulting from germline mutations in GCGR. Grossly, there is diffuse involvement of pancreas by variably-sized nodules instead of a single dominant mass.

Several multisystem diseases can show mild elevation of plasma glucagon levels (200 to 500 pg/ml), including cirrhosis, chronic renal disease, diabetic ketoacidosis, prolonged starvation, acute pancreatitis, acromegaly, hypercorticism, severe burns, severe stress, septicemia, and celiac disease.

The image shows a well-differentiated pancreatic neuroendocrine tumor with copious hyalinized stroma. The patient had full-blown glucagonoma syndrome.