Glucagonoma is a rare functioning pancreatic neuroendocrine tumor (PNET) that hypersecretes glucagon, leading to a syndrome comprising migratory necrolytic erythema , diabetes mellitus , anemia , weight loss, mucosal abnormalities, thromboembolism, gastrointestinal and neuropsychiatric symptoms.
Summary
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- 1 Synonyms
- 2 Prevalence
- 3 Inheritance
- 4 Age of onset or onset
- 5 Epidemiology
- 6 Clinical description
- 7 Etiology
- 8 Diagnostic methods
- 1 Differential diagnosis
- 9 Management and treatment
- 10 Forecast
- 11 Sources
Synonyms
Glucagonoma syndrome
Prevalence
<1 / 1,000,000
Heritage
Not applicable
Age of onset or onset
Adulthood
epidemiology
The estimated incidence in the general population is 1 / 20,000,000.
Clinical description
Glucagonoma usually occurs in the fifth decade of life, with migrating necrolytic erythema often the initial symptom. This skin condition is characterized by a rash of reddish, blistering and migratory lesions, associated with severe itching and located primarily in the lower extremities and groin. In most cases diabetes is present and therapy with insulin is required. Other frequent manifestations are weight loss, anemia , mucosal abnormalities ( glossitis , cheilitis , stomatitis ), gastrointestinal disorders, thromboembolism and neuropsychiatric symptoms (depression ). Most glucagonomas have already metastasized by the time they are diagnosed, primarily in the liver . In some cases, glucagonoma may be associated with multiple endocrine neoplasia type 1 (MEN1). Occasionally, the glucagonoma may be non-functioning.
Etiology
The etiology is unknown. These 2-25 cm tumors mainly originate from the tail of the pancreas . Synthesize and secrete glucagon , responsible for compensating the effects of insulin , and therefore essential to regulate the levels of sugar in blood .
Diagnostic methods
Diagnosis is based on clinical findings and endocrine tests. Levels glucagon serum are substantially higher (> 500 pg / mL), considered diagnostic levels above 1000 pg / mL if the patient also shows features of the syndrome of the glucagonoma. The level of other hormones such as insulin , somatostatin, and the intestinal vasoactive peptide may also be elevated. Serum chromogranin A levels are increased. Tumor localization is possible by computed tomography (CT), octreotide scan, magnetic resonance imaging (MRI), and / or endoscopic ultrasound.
Differential diagnosis
Differential diagnosis includes familial hyperglucagonemia, hereditary and autoimmune chronic pancreatitis, Mahvash disease, acrodermatitis enteropathica, and cirrhosis .
Management and treatment
Somatostatin analogues (octreotide and lanreotide) are generally effective in remission of the rash in most patients as well as in improving some symptoms (weight loss, abdominal pain , and diarrhea ). Hyperglycemia is treated with insulin injections or antidiabetic drugs. Parenteral nutrition (with essential fatty acids, amino acids, vitamins, and minerals) may also be necessary. Surgical resection is the only curative option in localized cases. Surgical debulking followed by chemotherapy may be an option in large tumors. Resection of the lymph nodes, spleen, and parts of the liver affected by metastasisIt may also be necessary. Since thromboembolic complications can occur, antiplatelet agents and anticoagulants may be recommended.
Forecast
In general, the prognosis of glucagonoma is unfavorable since most have metastasized to the diagnosis. Usually, those cases associated with MEN1 have a better prognosis since they are diagnosed earlier.
