Clinical Background
Glucagonomas are neuroendocrine tumors that produce excessive amounts of glucagon and are associated with a distinctive syndrome.
Epidemiology
- Incidence - <1/million
- Age - median age is 50-70 years
- Sex - equal gender distribution
- Occurrence - sporadic most of the time
Risk Factors
- Genetic - rarely associated with MEN 1
Pathophysiology
- Tumor of the alpha cells of the pancreatic islets
- Most frequently in the body and tail of the pancreas
- Tumor is usually large when discovered
- Most tumors are malignant
Clinical Presentation
- Diabetes mellitus, skin rash, hypoaminoacidemia, tendency for deep vein thrombosis (DVT)
- Skin rash
- Migratory necrolytic erythema
- Starts as annular erythema at intertriginous sites
- Rash progresses to papulobullous stage that waxes and wanes
- Frequently metastatic at presentation - liver is the most common site
See Also
Diagnosis
Diagnosis
- Laboratory testing
- Glucagon level >500 pg/mL
- Imaging studies
Differential Diagnosis
- Diabetes mellitus (type 1 or type 2)
- Thrombophilic syndrome
- Hereditary thrombophilia
- Paraneoplastic syndromes
Tests generally appear in the order most useful for common clinical situations
| Test name: Glucagon
|
| ARUP #: 0099165 |
| Methodology: Radioimmunoassay
|
| Use: Diagnose glucagonoma |
| Limitations: |
| Follow-up:
|
| Test name: Immunohistochemistry Stain Offering
|
| ARUP #: arup005 |
| Methodology: Immunohistochemistry
|
| Use: For fixed tissue samples, consultative services as well as immunohistochemical staining for glucagon, PGP9.5, synaptophysin and CAM5.2 (LMW) are available |
References
General References
Chastain MA. The glucagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci.
2001;
321(
5):
306-320.
Cruz-Bautista I, Lerman I, Perez-Enriquez B, Padilla LS, Torres CL, Lopez A, Cabrera T, Mehta RP, Gomez-Perez FJ, Rull JA, Orozco-Topete R. Diagnostic challenge of glucagonoma: case report and literature review. Endocr Pract.
2006;
12(
4):
422-426.
Jabbour SA, Davidovici BB, Wolf R. Rare syndromes. Clin Dermatol.
2006;
24(
4):
299-316.
O'Grady HL, Conlon KC. Pancreatic neuroendocrine tumours. Eur J Surg Oncol.
2008;
34(
3):
324-332.
Oberg K, Eriksson B. Endocrine tumours of the pancreas. Best Pract Res Clin Gastroenterol.
2005;
19(
5):
753-781.
Soga J, Yakuwa Y. Glucagonomas/diabetico-dermatogenic syndrome (DDS): a statistical evaluation of 407 reported cases. J Hepatobiliary Pancreat Surg.
1998;
5(
3):
312-319.
Tierney EP, Badger J. Etiology and pathogenesis of necrolytic migratory erythema: review of the literature. MedGenMed.
2004;
6(
3):
4-.
van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR. The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. Eur J Endocrinol.
2004;
151(
5):
531-537.
Medical Reviewers
Meikle, A. Wayne, M.D. Medical Director, RIA and Endocrinology at ARUP Laboratories; Professor of Internal Medicine and Pathology, University of Utah
Comprehensive Review: July 2008
Last Update: July 2008
