What Is a Glucagonoma?

Islet alpha cell tumor, which secretes excessive glucagon, mainly manifested as skin migration, necrolytic erythema, diabetes, anemia, glossitis and cheilitis, vulvovaginitis, hypoamino acidemia, etc., also known as Hyperglycemia skin disease syndrome. Malignant, often metastasized early. Tumor diameter is generally 1.5 to 3 cm, and sometimes the entire pancreas is a tumor. After early surgical removal of the tumor, skin damage and diabetes can quickly disappear. This disease is more common in middle-aged and elderly people aged 40 to 70 years. Women are more common than men, and most of them are menopausal women. Most have a course of more than 1 year, and some more than 12 years.

Basic Information

English name: glucagonoma
Visiting department: Oncology
Multiple groups: Middle-aged and elderly women at the age of 40 to 70,

Common locations: Pancreatic tail, pancreatic body, pancreatic head
Common causes: Islet alpha cell hyperplasia
Common symptoms: Rash and diabetes

Causes of glucagonoma

Glucagon tumors are mostly isolated, 60% of which are malignant and occasionally caused by islet alpha cell hyperplasia. The most common tumors are pancreatic tail, followed by pancreatic body and pancreatic head.

Clinical manifestations of glucagonoma

The prominent symptoms of this disease are rash and diabetes. The appearance of the rash has certain characteristics. It is clinically called epidermal necrotizing migratory erythema, and it is mainly regional erythema, and it can also be desquamative red pimples and macular rashes, which are often ring-shaped or arc-shaped. Crusting, due to susceptibility to infection by bacteria and yeast, necrotizing lytic bullous rash. When the initial lesion site begins to heal, pigmentation remains at the healing site, and the lesion can be displaced from one site to another. Its erythema can occur in various parts of the body, but it is more common in the trunk, lower abdomen, groin, buttocks, perineum, lower limbs, and middle 1/3 of the face. It takes 1-2 weeks for the lesion to heal.

More than 95% of patients have symptoms of diabetes, and the symptoms are usually mild. They can often be controlled through diet control or oral medications. Occasionally, they are more severe and require large doses of insulin to control.

Glucagon tumor test

Laboratory inspection

(1) General laboratory tests Hypoaminoacidemia, urine glucose positive, elevated blood glucose or decreased glucose tolerance, rapid erythrocyte sedimentation, positive cell positive hemochromatemia, tumor cells can be found in duodenal fluid of pancreatic head tumors.

(2) Plasma glucagon radioimmunoassay The basic measurement of glucagon is significantly higher, which can exceed 1000 pg / ml, which is 5 to 10 times the normal value. Glucose is often not inhibited by oral or intravenous glucose injection. . Plasma glucagon challenge test Pancreatic -cell secretagogues can be used, such as arginine and alanine. Plasma glucagon increases after injection, but this response is also seen in primary or secondary islet alpha cell proliferation, so this test is not a specific diagnostic method for glucagonoma.

2. Other auxiliary inspections

(1) Barium meal examination and duodenal hypotonography are only helpful for the diagnosis of pancreatic head islet cell tumors, as the inner wall of the descending duodenum has changed, but this disease rarely occurs in the pancreatic head . In some cases, coarse folds of the jejunum and ileum appear.

(2) Celiac artery and pancreatic artery angiography The diagnosis of islet cell tumor can reach more than 70%, but it is not clear which cell the tumor came from.

(3) Ultrasound scan and CT scan of the liver and pancreas can diagnose the primary lesion of the pancreas and whether the liver has metastatic lesions.

(4) Observation of tumor tissue by electron microscope Tumor cells conform to the characteristics of islet cells; tumor tissues contain high concentrations of glucagon.

Glucagon tumor diagnosis

According to clinical manifestations, typical specific skin damage, weight loss that is very similar to diabetes in all cases, glossitis and angular cheilitis, diarrhea, etc., combined with laboratory and other auxiliary examinations can be diagnosed.

Differential diagnosis of glucagonoma

Glucagon tumors have skin damage and increased plasma glucagon levels, so cases with skin damage and elevated glucagon levels should be identified:

Disease of skin damage

(1) The skin damage of patients with glucagon tumors is necrotizing and dissolving transitional erythema: it should be associated with benign familial pemphigus, deciduous pemphigus, diffuse pustular psoriasis, toxic epidermal necrolysis Phase identification.

(2) Patients with skin lesions and tumors should be distinguished from diseases such as dermatomyositis, black acanthosis, and acquired ichthyosis.

2. Diseases with elevated plasma glucagon

Those with a significant increase are only seen in islet a-cell tumors, but those with a mild increase are seen in the following conditions and diseases: protein intake, starvation, insulin withdrawal, acidosis, uremia, infection, strenuous exercise, diabetes, liver cirrhosis, reservoir Hin syndrome, acromegaly, pheochromocytoma, acute pancreatitis, and adrenocortical hormone therapy should be identified.

Glucagon tumor complications

A small number of patients have mental neuropathies such as dementia, optic nerve atrophy, stuttering, nystagmus, ataxia, and abnormal reflexes.

Glucagon tumor treatment

Treatment of skin damage

For the early stage of the disease, no specific skin disease has been shown, or before the diagnosis has been clarified, oral corticosteroids, oxytetracycline, diiodine or azathioprine can be used to relieve skin lesions; in recent years, zinc agents and topical applications Corticosteroids can also relieve skin lesions, but they are not effective for other symptoms.

2. Surgical removal of tumor

After the diagnosis is confirmed, surgical treatment should be adopted in time, and those who have doubts should also be surgically explored. Symptoms can be rapidly improved after tumor resection, and skin lesions often improve significantly on the second day after surgery, and they can completely disappear after 2 weeks. Heavier skin lesions and fusion bullae also returned to normal within 3 weeks after surgery. Patients with diabetes can heal after a few days of insulin treatment, and glucose tolerance recovers later, and it has been reported to return to normal within 3 months.

3. Chemotherapy

Chemotherapy can be used for patients with metastasis who cannot be resected or who have resected the original lesion.