|
Definition
|
pancreatic
NET (99 %) with excessively elevated secretion of glucagon
original lit.: Becker et al. 1942 (Arch
Dermatol Syph), Unger et al. 1963 (J Clin Invest), McGavran et
al 1966 (N Engl J Med) |
| |
©
www.gep-net.com / www.gep-net.de
|
|
Incidence
|
Incidence:
0.01 - 0.1 per 1 Mill. / year (
= 0.25% - 10% insulinoma-incidence) |
|
Pathology
- Pathophysiology
|
katabolic
glucagon effects:
glykogenolysis, gluconeogenesis, lipolysis (weight loss),
hypoaminoacidemia (genesis of cutaneous erythema) |
insulin
secretion: mild
diabetes without ketogenesis |
often
large ( < 5 cm) tumors, mostly located in the pancreatic
tail, frequently malignant (~ 70 %) |
|
Clinical
|
|
Typical
clinical syndrom (Glucagonoma-Syndrome) :
|
|
1.
specific erythema of
skin ("necrolytic migratory erythema / NME",
"rash") - 65-90 %
|
| 2.
weight loss -
60-100 % |
| 3.
glucose - intolerance or mild
diabetes - 80-90 % |
| 4.
anemia - 50-85 % |
| other
symptoms:
diarrhea, abdominal pain, thromboembolism, glossitis |
|
|
Diagnosis
|
Laboratory:
plasma glucagon conc. > 500 pg/ml,
frequently > 1000 pg/ml |
mild
hyperglycemia (never ketonemic), grossly reduced amino acids
(glycin and alanine, < 25 % normal); hypocholesterolemia |
|
|
Therapy-Strategies
|
surgical
resection - surgery of the liver (metastases) - chemoperfusion
and -embolisation - chemotherapy |
symptoms
- rash: hyperalimentation, substitution of amino acids
iv., parenteral nutrition, somatostatin
analogs |
|
|
Images
|
|
