Spinal Metastasis as the Initial Manifestation of a Nonsecretory Glucagonoma

Anjana Aggarwal, MD, Jennifer Brainard, MD, Daniel J. Brotman, MD


South Med J. 2003;96(2) 

In This Article

Abstract and Introduction

Glucagonomas are rare functional endocrine tumors of the pancreas that classically present with symptoms of glucagon excess, including rash, hyperglycemia, diarrhea, and weight loss. Metastatic disease at presentation is common but is often limited to the liver and regional lymph nodes. We describe an unusual case of a patient with glucagonoma who presented with a pathologic vertebral fracture. This tumor had no evidence of active hormone secretion but tested positive for glucagon by immunohistochemical staining.

Glucagonomas are neuroendocrine tumors that arise from cells of the pancreatic islets. These tumors are often associated with a clinical syndrome caused by the metabolic effects of glucagon. The glucagonoma syndrome includes increased hepatic gluconeogenesis and hyperglycemia, muscle catabolism with associated wasting, and an unusual cutaneous eruption known as necrolytic migratory erythema, sometimes with associated stomatitis or cheilosis.[1,2,3] Diarrhea and gastrointestinal complaints are common, especially when the tumor secretes other peptide hormones in addition to glucagon, such as vasoactive intestinal peptide or gastrin.[2] Venous thromboembolism is unusually common in patients with this malignancy.[2,4] Glucagonomas are rarely benign and often metastasize to the liver and the lymph nodes[2,5] but can also metastasize to distant organs, including bone, in the later stages of the disease.[2,6,7] We describe a case of a glucagonoma metastatic to bone at presentation. The patient had no evidence of systemic glucagon excess, so the diagnosis was made on the basis of positive immunohistochemical staining of the tumor for glucagon. This case illustrates that glucagonomas can metastasize widely and that they are not always associated with clinical evidence of glucagon excess.


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