BACKGROUND: Pancreatic endocrine tumors are uncommon neoplasms and can lead to systemic disorder including glucagonoma syndrome, a very rare prototypical paraneoplastic phenomenon. The aim of this study was to assess the diagnosis and surgical strategy for the treatment of glucagonoma syndrome.
METHODS: The clinical data of a case of pancreatic head tumor with typical glucagonoma syndrome of necrolytic migratory erythema (NME), diabetes mellitus (DM), anemia, and glossitis were retrospectively analyzed.
RESULTS: Cutaneous eruption occurred mainly in the groin, extremities, thighs, buttocks, and perineum. A highly elevated level of serum glucagon was detected by radioimmunoassay. A tumor located in the head of the pancreas was well-defined by pre and intra-operative ultrasonography, contrast enhanced computed tomography, and magnetic resonance imaging. Tumor enucleation was performed, showing significantly improved symptoms. Near complete resolution of NME was shown one week after surgery.  Surgical complications or recurrence was not found.
CONCLUSIONS: The diagnosis of glucagonoma syndrome is established by marked clinical features such as NME as the hallmark clinical finding, hyperglucagonemia, and radiographically demonstrated neuroendocrine tumor. The topographic diagnosis of glucagonoma can be achieved by combined imaging methods. Enucleation of tumor is a valuable treatment for solitary pancreatic tumor without peripancreatic invasion, liver metastasis, and pancreatic duct compression.