Diagnosis and treatment of pancreatic glucagonoma presenting with multiple metastases and recurring postoperatively: Report of two patients

Abstract

Background: Pancreatic glucagonoma is a rare neuroendocrine neoplasm with malignant potential. Its etiology is unclear, and its course is protracted, often fraught by recurrences and greatly diminished quality of life. Case presentation: Herein, we report two patients with such tumors, one a 60-year-old woman hospitalized for blood glucose elevation and recurrent necrolytic migratory erythema (NME) of the trunk and limbs. Imaging studies showed an area (~3 cm) of reduced enhancement at head of pancreas. Examination of the subsequent pancreaticoduodenectomy specimen confirmed a pancreatic neuroendocrine tumor (G2). The patient’s blood glucose normalized postoperatively, and her skin erythema cleared. During follow-up, however, intramuscular injections (30 mg) of long-acting release (LAR) octreotide acetate microspheres were required at 28-day intervals for recurrent NME; and there was apparent hepatic and retroperitoneal nodal involvement after 36 months. The second patient was a 32-year-old woman hospitalized for recurrent glossitis, perioral dermatitis, and NME of both lower limbs. Imaging studies revealed a low-density nodule (~2 cm) in tail of pancreas, as well as multiple space-occupying lesions of liver. Distal pancreatectomy was undertaken, with splenectomy and palliative resection of hepatic nodules, confirmingmetastasis of a primary pancreatic neuroendocrine tumor (G2) to liver.The NME resolved postoperatively, but this patient also received intramuscular octreotide LAR injections (30 mg) at 28-day intervals. After 6 months, percutaneous radiofrequency ablation of residual liver lesions took place. Conclusions: The present accounts detail our diagnostic and therapeutic experiences with this rare type of tumor, especially the management of multiple metastases upfront and postoperative recurrences.