Medical Oncology

, Volume 24, Issue 3, pp 330–337

Endocrine pancreatic tumors with glucagon hypersecretion: a retrospective study of 23 cases during 20 years

Authors

    • Department of Medicine/Endocrine OncologyUniversity Hospital
  • Anders Sundin
    • Department of RadiologyUniversity Hospital
  • Dan Granberg
    • Department of Medicine/Endocrine OncologyUniversity Hospital
  • Kristina Dunder
    • Department of Medicine/Endocrine OncologyUniversity Hospital
  • Britt Skogseid
    • Department of Medicine/Endocrine OncologyUniversity Hospital
  • Eva Tiensuu Janson
    • Department of Medicine/Endocrine OncologyUniversity Hospital
  • Staffan Welin
    • Department of Medicine/Endocrine OncologyUniversity Hospital
  • Kjell Öberg
    • Department of Medicine/Endocrine OncologyUniversity Hospital
  • Barbro Eriksson
    • Department of Medicine/Endocrine OncologyUniversity Hospital
Original Paper

DOI: 10.1007/s12032-007-0011-2

Cite this article as:
Kindmark, H., Sundin, A., Granberg, D. et al. Med Oncol (2007) 24: 330. doi:10.1007/s12032-007-0011-2

Abstract

Background

Glucagon-secreting endocrine pancreatic tumor is a rare disease, hence controlled studies on clinical management are lacking. In an attempt to assess the efficacy of diagnostic and therapeutic measures in patients with glucagonoma, a retrospective study was performed using the archives of a tertiary care center.

Patients and methods

Records from 340 patients with endocrine pancreatic tumors were reassessed and 23 patients with malignant endocrine pancreatic tumor and elevated plasma glucagon levels were identified.

Results

About 7% of patients with histologically verified tumors fullfilled our criteria for glucagonoma. Only 22% of these patients had developed diabetes prior to the diagnosis of glucagonoma. Seventy eight percent had metastatic disease to the liver at diagnosis. Necrolytic migratory erythema was diagnosed or clinically suspected in 52%. Somatostatin receptor scintigraphy was positive in 95%. Nineteen patients received chemotherapy at some point, in 18 cases streptozotocin and 5 FU. With this treatment, objective radiological responses were seen in 50% of evaluable patients. Other treatment modalities used were interferon, somatostatin analogs, hepatic artery embolization, radio-frequency ablation of liver metastases, and radiolabeled somatostatin analogs. During the study period, 11 patients died at a median of 80 months from diagnosis whereas 11 patients are still alive after a median follow up of 52 months. One patient was lost to follow-up.

Conclusions

Glucagonomas represent 7% of our comprehensive referal material of endocrine pancreatic tumors. Necrolytic migratory erythema was a common finding (52%) and diabetes less frequent at presentation than previously reported. Tumors were positive on somatostatin receptor scintigraphy and objective responses were seen to chemotherapy.

Keywords

Neuroendocrine tumorSomatostatin receptor scintigraphyChemotherapySurvivalCT

Copyright information

© Humana Press Inc. 2007