title: Endocrine pancreatic tumors with glucagon hypersecretion: a retrospective study of 23 cases during 20 years.
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.
  
  
  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 referral 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.
authors.affiliation: Department of Medicine/Endocrine Oncology, University Hospital, Uppsala, Sweden. henrik.kindmark@medsci.uu.se
authors.forename: Henrik
authors.initials: H
authors.lastname: Kindmark
authors.forename: Anders
authors.initials: A
authors.lastname: Sundin
authors.forename: Dan
authors.initials: D
authors.lastname: Granberg
authors.forename: Kristina
authors.initials: K
authors.lastname: Dunder
authors.forename: Britt
authors.initials: B
authors.lastname: Skogseid
authors.forename: Eva Tiensuu
authors.initials: ET
authors.lastname: Janson
authors.forename: Staffan
authors.initials: S
authors.lastname: Welin
authors.forename: Kjell
authors.initials: K
authors.lastname: Oberg
authors.forename: Barbro
authors.initials: B
authors.lastname: Eriksson
mesh_terms: D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D004890:Erythema / Q000150:complications* / Q000175:diagnosis; D005260:Female; D005934:Glucagon / Q000097:blood; D005935:Glucagonoma / Q000150:complications / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007372:Interferons; D008113:Liver Neoplasms / Q000556:secondary; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000150:complications / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D017481:Receptors, Somatostatin / Q000378:metabolism; D012189:Retrospective Studies; D012737:Sex Factors; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D017481:Receptors, Somatostatin; D005934:Glucagon; D007372:Interferons