Diagnosis and comprehensive treatment of pancreatic glucagonoma presenting with multiple metastases and recurring postoperatively

Abstract

Background: Pancreatic glucagonoma is a rare neuroendocrine neoplasm with malignant potential. At present, clinicians have a poor understanding of the disease, and the current epidemiology, diagnosis and comprehensive treatment of the disease have not been fully established. 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 retroperitioneal 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, confirming metastasis 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： In patients with multiple liver metastases, palliative surgical treatment may confer patient benefits and prolong survival. Those with hormonal-based symptoms are often well controlled through somatostatin analog (SSA) administration. postoperative adjuvant therapy of metastatic pancreatic glucagonoma has also obvious effects on the prognosis.