Glucagonoma

Glucagonoma

Pancreas is an exoendocrine gland. Exocrine part releases pancreatic enzymes which help in digestion. Endocrine part consists of islets cells which have alpha cells which secrete hormone glucagon and beta cells which secrete hormone insulin into the blood. Glucagon is responsible for increasing blood sugar level while insulin is responsible for lowering blood sugar levels. Glucagonoma is a rare condition. It is a type of neuroendocrine tumour. It is a malignant tumor of the alpha cells of islets of the pancreas which leads to excess glucagon hormone in the blood.

Causes and risk factors

Causes of glucagonoma are unknown. Genetic factors, family history are also responsible for glucagonoma. Most of the glucagonoma are cancerous. 

Clinical presentation

Glucagonoma is characterized by presence of 4D, i.e., diabetes, dermatitis, DVT, and depression, though all 4 may not be present in every patient. Patient presents with symptoms like increased appetite, inflamed mouth and tongue, glucose intolerance, and excessive thirst. There is frequent urination, nighttime urination. There is unintentional weight loss. High blood sugar is present. Diarrhea can occur. Skin rash may be evident on face, abdomen, buttocks, or feet that comes and goes. There may be raised sores filled with clear fluid or pus. Skin rash is typical and is also known as necrolytic migratory erythema. Blood clots are common in the leg [DVT]. Depression is seen in many patients.

Investigation

Medical history by the patient and clinical examination by the doctor helps in diagnosis. CT/MRI scan of the abdomen is advised. Blood tests are done for glucose levels, glucagon levels. Biopsy of skin is done. Endoscopic ultrasound is recommended. Radioactive scan is done. 

Treatment 

First line of treatment consists of attaining symptomatic relief. Management of blood sugar levels, administration of hypoglycemic drugs or injections is are required. Medications for skin rashes, diarrheas are given. Further treatment consists of surgical removal of tumor.

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