Glucagonoma is a very rare hormonally active tumor derived from the alpha cells of the pancreas, secreting glucagon. This tumor grows very slowly and its symptoms appear late. In most patients, the onset of glucagonoma syndrome occurs up to 2 years before the diagnosis is made. What is glucagonoma treatment? What's the prognosis?
Contents:
- Glucagonoma - symptoms
- Glukagonoma - diagnosis
- Glucagonoma - treatment
- Glucagonoma - prognosis
Glucagonomaaccounts for about 2% of all hormonally active neoplasms in the gastrointestinal tract. The incidence of this cancer is 1/20 million cases per year. Glucagon is a hormone secreted physiologically by the alpha cells of the pancreas. Its task is to maintain normal blood glucose levels in a state of hunger. It happens by increasing the breakdown of glycogen, which is a spare material present in the liver.
Due to the breakdown of glycogen, glucose is released into the blood, which helps to maintain its proper concentration in the body, despite hunger. The presence of a glucagon-secreting tumor causes an uncontrolled rise in blood glucose levels, irrespective of food intake.
Not infrequently, glucagonoma is a component of the multiple endocrine neoplasia syndrome (MEN1). The tumor is then accompanied by primary hyperparathyroidism and a tumor of the anterior pituitary gland. The glucagon-secreting tumor is a malignant tumor that can metastasize to distant organs.
Glucagonoma is a large, solid tumor most often located in the tail of the pancreas, very rarely this tumor has a different location. Glucagonoma is most common in postmenopausal women.
At the time of diagnosis, the vast majority of patients with glucagonoma already have metastases to the liver and regional lymph nodes. It is extremely rare that the symptoms of glucagonoma are not related to a pancreatic tumor but are associated with cirrhosis or chronic pancreatitis.
Glucagonoma - symptoms
Glucagonoma is a tumor with a very slow growth, therefore it begins to show up very late. The most common symptoms associated with glucagonoma are:
- diabetes osmooth running
Increased blood glucose levels may cause drowsiness, increased thirst, frequent urination, visual disturbances, and impaired wound healing. However, this type of diabetes tends to resolve after the glucagon-secreting tumor is removed.
- weight loss
- anemia
- diarrhea
- stomatitis
- erythema wandering necrotic
Wandering necrotic erythema is the most characteristic symptom of glucagonoma. It is a skin lesion that initially develops on the limbs and can appear all over the body as the disease progresses. Initially, erythema appears on the skin, a bladder appears in its central part, which after piercing creates a necrotic focus covered with a dark scab. It is accompanied by pain and itching. The lesion heals within 10 days, leaving a discoloration. Erythema appears periodically every 7-14 days.
- venous thrombosis and pulmonary embolism
- diffuse alopecia (Latin alopecia diffusa)
This symptom is characterized by excessive hair loss, baldness is even over the entire surface of the scalp, and is often accompanied by deformation of the nail plate.
- tongue reddening, chewing
- mental and neurological disorders such as irritability, apathy, memory problems
- nystagmus.
Glukagonoma - diagnosis
Clinical symptoms, especially the presence of migratory necrotic erythema, require the implementation of glucagonoma diagnostics. Diagnosis is based on the assessment of blood glucagon levels, a result above 1000 pg / ml is the basis for extending the diagnosis towards a glucagon-secreting tumor.
Laboratory tests also show a decreased level of zinc in the blood. In order to confirm the diagnosis and to deepen the diagnosis, imaging tests are performed to assess the size and location of the tumor. The tests indicated in the diagnostics of glucagonoma include ultrasound, computed tomography and somatostatin receptor scintigraphy.
Scintigraphy is a very sensitive test that detects somatostatin receptors in tumor cells. It allows the location of a tumor that is not visible in other imaging tests, and helps to qualify patients for treatment with somatostatin analogues.
Thanks to this test, it is possible to assess whether a given tumor has somatostatin receptors, which are a target for the drugs used.
The histopathological examination of the tumor biopsy is the basis for determining the tumor grade.
Glucagonoma - treatment
The basisTreatment is the surgical removal of the tumor with a margin of he althy tissue. Pharmacological treatment is based on the use of drugs that are somatostatin analogues, if their presence on tumor cells was found in the receptor scintigraphy test.
These drugs help to inhibit the progress of the disease and reduce clinical symptoms. They are given as intramuscular injections every few weeks as recommended by your doctor.
In the case of inoperable tumors, radioisotope treatment with radiolabelled somatostatin analogues is used, if scintigraphic examination has confirmed the presence of somatostatin receptors on the tumor surface.
In case of therapeutic failure with other methods, chemotherapy is used in a multi-drug regimen.
Glucagonoma - prognosis
The prognosis depends on the stage of the neoplasm at the time of diagnosis and the presence of metastases, it is assumed that in the case of successful tumor removal surgery, the 5-year survival rate in patients is 97%.
Read also: Pancreatic cancer - symptoms and treatment of pancreatic cancer
About the authorBow. Agnieszka MichalakA graduate of the First Faculty of Medicine at the Medical University of Lublin. Currently a doctor during postgraduate internship. In the future, she plans to start a specialization in pediatric hematooncology. She is particularly interested in paediatrics, hematology and oncology.Read more articles by this author