According to abbreviationfinder.org, the glucagonoma is a malignant tumor in the pancreas that grows from the A cells. Functionally active glucagonomas produce hormones and potentially cause a variety of symptoms, while functionally inactive glucagonomas can remain symptom-free. If surgical removal is not possible, chemotherapy is an option.
What is a glucagonoma?
A glucagonoma is a malignant tumor in the pancreas, which is located in the upper abdomen. Their secretions contain enzymes that help digest carbohydrates, proteins, and fats in the duodenum. In addition, it produces the hormones insulin, glucagon, somatostatin, ghrelin, and pancreatic polypeptide.
One of the hormones, glucagon, regulates blood glucose levels and is produced in the A cells of the pancreas; tumor growth in glucagonomas also originates from these cells. Depending on the behavior of the tumor, medicine differentiates between functionally active and functionally inactive glucagonomas.
The latter can be both benign and malignant and do not always lead to the development of symptoms. In contrast, functionally active glucagonomas are often malignant and present with a variety of symptoms. Overall, the disease is extremely rare: only around one percent of pancreatic tumors are glucagonomas, most of which are functionally inactive.
Causes
Type I multiple endocrine neoplasia can lead to the development of functionally inactive glucagonomas. This underlying condition, also known as Wermer syndrome, is an inherited condition. A gene on the eleventh chromosome that usually prevents the development of tumors is affected.
Type I multiple endocrine neoplasia can cause not only glucagonomas but also other pancreatic tumors such as insulinomas and gastrinomas. Neoplasms in the parathyroid glands, the pituitary gland and other organs are also possible. Irrespective of this, glucagonomas can occur without previous illnesses; the exact cause of the uncontrolled cell growth in the tumor can often not be determined in individual cases.
Symptoms, Ailments & Signs
Different signs are characteristic of functionally active and functionally inactive glucagonomas. Possible symptoms of functionally active glucagonoma include necrotizing migratory eczema (erythema necrolyticum migrans), anemia and diabetes mellitus.
Sweet-smelling urine, increased urine production, severe thirst, tiredness, subjective lack of energy, itching, dermatitis, slower wound healing and neurological symptoms can indicate diabetes.
In addition, patients often lose weight because the body cells cannot use the sugar despite sufficient glucose levels in the blood. Functionally inactive glucagonomas can develop without noticeable symptoms, since they do not produce additional glucagon and accordingly do not interfere with the metabolism of the human body.
Depending on its location and size, however, the tumor can indirectly cause symptoms by displacing healthy tissue, partially blocking the main bile duct (ductus choledochus) and thereby causing jaundice ( icterus ), provoking (pressure) pain or impairing the blood vessels and thereby leading to bleeding. Metastases to other organs, such as the liver, may cause other symptoms.
Diagnosis & History
Symptoms that may be present provide the first clues to the glucagonoma. Imaging methods enable the tumor to be viewed directly, which is also necessary for later removal. Ultrasound sonography, computed tomography (CT) or magnetic resonance imaging (MRT) are preferably used. In many cases, the glucagonoma is discovered by chance during such examinations.
An increased glucagon concentration can be found in the blood, especially in the case of a functionally active glucagonoma: the value can be ten to a thousand times higher than the normal range. In addition, the laboratory examination of the blood specifically searches for the tumor marker chromogranin A, the occurrence of which is typical for glucagonomas.
Complications
Since the glucagonoma is a malignant tumor, the further course depends heavily on the spread and severity of the tumor. In most cases, the glucagonoma causes diabetes, which places a heavy burden on the patient’s everyday life. The urine also smells very sweet and the patient suffers from increased thirst.
There is a constant feeling of tiredness, which cannot be compensated for with the help of sleep. Itching also occurs in different parts of the body and wounds heal more slowly. If the metastases spread to other regions of the body, complaints can also occur in other places and organs. In the worst case, this can lead to death.
The tumor is usually treated with either surgery or chemotherapy. This leads to nausea, vomiting and hair loss. However, it cannot be predicted whether the tumor can be completely cured and whether it will recur. In most cases, life expectancy is reduced by the glucagonoma.
When should you go to the doctor?
If symptoms such as a sweet smell of urine, severe thirst or fatigue are noticed, a doctor should be consulted. A glucagonoma must be recognized and treated early to enable a positive healing process. People who feel physically or mentally unwell and, for example, are constantly tired or experience unusual itching should have this clarified immediately.
Whether it is a glucagonoma can only be answered by a doctor. Patients suffering from multiple endocrine neoplasia type I are particularly susceptible to the development of a glucagonoma. If the above symptoms are noticed, often accompanied by signs of diabetes mellitus or anemia, medical advice is required.
If unusual symptoms occur during treatment, it is best to inform the responsible doctor. In the event of serious complications such as bleeding or severe pressure pain, the person concerned should be taken to the nearest hospital.
Treatment & Therapy
Removal of the glucagonoma is the standard treatment. If surgery is not possible or not advisable, chemotherapy can also be considered. The patient usually receives intravenous drugs that inhibit cell growth. The effect affects not only the tumor, but also all other cells.
Those treated therefore typically suffer from hair loss, since hair cells also divide very frequently and are particularly affected by the chemotherapeutic agent. An example of an active ingredient is streptozotocin, to which primarily pancreatic cells react. Common side effects of streptozotocin include nausea, diarrhea, vomiting, liver and kidney failure.
In addition, the active substance itself can have a carcinogenic effect and promote the development of tumors; So far, however, scientists have only been able to observe this effect in animals. In particular, untreated, functionally active glucagonomas can have fatal consequences. The tumor produces glucagon, which promotes the release of glucose.
Insulin, which the body also produces in the pancreas, acts like a key on the body’s cells and allows them to absorb glucose. An excess of glucagon effectively inhibits the effect of insulin – the result is on the one hand an oversupply of glucose in the blood and on the other hand cells that suffer from a lack of energy and ultimately starve. This condition corresponds to the metabolic disease diabetes mellitus.
Diabetes may need to be treated as well; the same applies to other symptoms. After removal of the tumor and taking the prescribed hormone substitutes, the consequences may disappear. Regular follow-up examinations ensure that the tumor does not return or spread to other organs due to metastasis.
Outlook & Forecast
The prognosis of the glucagonoma is linked to the progression of the disease at the time of diagnosis and the start of treatment. If left untreated, the malignant tumor of the pancreas leads to the premature death of the patient. Alternative healing methods that lead to freedom from symptoms are currently not known and spontaneous healing is not to be expected in these cases.
Glucagonoma requires surgery and, depending on the individual circumstances, the use of chemotherapy. If the cancer cells in the organism have already spread beyond the pancreas and infested other organs, the chance of a cure drops drastically. In these patients, the treatment plan is geared towards alleviating the existing symptoms.
So that the affected person feels as few impairments as possible and is not exposed to any unnecessary pain. If the tumor can be successfully removed in an operation, the chances of alleviating the symptoms increase. A cure can be achieved with subsequent follow-up treatment and further cancer prevention. Nevertheless, cancer therapy is necessary in the further course, which leads to severe restrictions in lifestyle.
Overall, the life expectancy of the patient with a glucagonoma is significantly reduced despite all current medical options. In many of those affected, a recurrence of the tumor disease is to be expected and numerous complications and secondary diseases are possible.
Prevention
General preventive measures that can help prevent glucagonoma and other cancers include avoidance of exposure to radiation, ultraviolet radiation, chemical carcinogens, and other factors. Personal lifestyle also has an influence on the individual cancer risk.
Smoking, obesity and poor eating habits are among the most important factors in this area. In addition, doctors and health insurance companies recommend regular check-ups for certain types of cancer in order to detect tumors at an early stage.
Aftercare
The options for aftercare are usually very limited in the case of a glucagonoma. The person concerned is primarily dependent on medical treatment by a doctor so that further complications and symptoms can be avoided and limited. A glucagonoma does not heal itself. In the worst case, the person affected dies as a result of this disease.
The earlier the tumor is detected, the better the further course. For this reason, early diagnosis and treatment is of great importance. The treatment itself usually takes the form of chemotherapy. Those affected depend on the support of friends and family, and they need not only physical but also psychological support.
A healthy diet and a healthy lifestyle in general can also have a positive effect on the course of this disease. Those affected are often dependent on taking medicines, whereby care must be taken to ensure that they are taken regularly and, above all, correctly.
Even after successful treatment, regular examinations of the body are necessary in order to detect further tumors at an early stage. Furthermore, contact with other people affected by this disease can have a positive effect on the further course.
You can do that yourself
Once a glucagonoma has been diagnosed, medical attention is required. However, those affected can also do some things themselves to relieve the symptoms and promote recovery.
First of all, it is important to treat the side effects individually. Measures such as bed rest, warm pads and a gentle diet help against nausea, diarrhea, vomiting and other side effects of the prescribed medication. Hair loss is usually not permanent. However, if it is perceived as annoying, a hairpiece or a gentle hair restorer is recommended.
However, the latter should be discussed beforehand with the responsible doctor in order to rule out interactions with the prescribed medication. In addition, it is important to treat the triggering disease. If the glucagonoma is based on diabetes, the diet must be changed. Any overweight should also be reduced and exercise should be done regularly. This also helps against accompanying symptoms such as fatigue, lack of energy and slower wound healing.
Physiotherapeutic measures such as physiotherapy, Pilates or yoga help against neurological complaints.
Since the appropriate self-help measures differ from case to case, you should talk to your doctor regularly. The doctor can provide additional tips that can be used to promote recovery independently.
