Glucagonoma

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?

The glucagonoma is a malignant tumor in the pancreas, which is located in the upper abdomen. Your secretions contain enzymes that support the digestion of carbohydrates, proteins and fats in the duodenum. It also produces the hormones insulin, glucagon, somatostatin, ghrelin and the pancreatic polypeptide.

One of the hormones, glucagon, regulates blood glucose levels and is produced in the A cells of the pancreas; It is from these cells that tumor growth in the glucagonoma also starts. 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 manifest themselves in a variety of complaints. Overall, the disease is extremely rare: only around one percent of pancreatic tumors are glucagonomas, most of which are functionally inactive.

causes

The multiple endocrine neoplasia of type I can lead to the development of functionally inactive glucagonomas. This underlying disease, also known as Wermer syndrome, is an inherited disease. Affected is a gene on the eleventh chromosome that usually prevents tumors from developing.

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. Regardless 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

Functionally active and functionally inactive glucagonomas are each characterized by different signs. Possible symptoms of the functionally active glucagonome include necrotizing migratory eczema (Erythema necrolyticum migrans), anemia, and diabetes mellitus.

Urine smelling sweet, increased urine production, severe thirst, tiredness, subjective lack of energy, itching, dermatitis, slow wound healing and neurological symptoms can indicate the diabetes.

In addition, patients often lose body weight because the body cells cannot use the sugar despite sufficient glucose levels in the blood. Functionally inactive glucagonomas can develop without noticeable symptoms, as they do not produce any additional glucagon and accordingly do not interfere with the metabolism of the human body.

Depending on the location and size, the tumor can indirectly cause discomfort by displacing healthy tissue, partially blocking the main bile duct (common bile duct) and thereby causing jaundice (jaundice), provoking (pressure) pain or impairing the blood vessels and thereby leading to bleeding . Metastases in other organs such as the liver may cause additional symptoms.

Diagnosis & course

Symptoms that may be present provide first indications of the glucagonoma. Imaging procedures enable the tumor to be viewed directly, which is also necessary for later removal. Ultrasound sonography, computed tomography (CT) or magnetic resonance tomography (MRT) are preferably used. In many cases, the glucagonome is discovered by chance during such examinations.

In the blood, especially with a functionally active glucagonome, an increased glucagon concentration can be determined: The value can be ten to a thousand times above 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 extent and severity of the tumor. In most cases, the glucagonoma causes diabetes, which puts a heavy strain on the everyday life of the person affected. The urine also smells very sweet and the patient suffers from increased thirst.

There is a constant feeling of tiredness, which cannot be compensated with the help of sleep. Itching also occurs in different parts of the body and wounds only heal more slowly. If the metastases spread to other regions of the body, complaints can also arise in other places and organs. In the worst case scenario, death can result.

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, the glucagonome will reduce life expectancy.

When should you go to the doctor?

If symptoms such as a sweetish urine odor, severe thirst, or fatigue are noticed, a doctor should be consulted. A glucagonoma must be recognized and treated early in order to enable a positive healing process. People who feel physically or mentally uncomfortable 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 with multiple endocrine neoplasia of type I are particularly prone to developing a glucagonoma. If the symptoms mentioned are noticed, often accompanied by signs of diabetes mellitus or anemia, medical advice is required.

If you experience unusual symptoms during treatment, it is best to inform your doctor. In the event of serious complications such as bleeding or severe pressure pain, the person concerned should be taken to the nearest hospital.

Doctors & therapists in your area

Treatment & Therapy

The removal of the glucagonoma is the standard treatment. If surgery is not possible or makes no sense, chemotherapy can also be considered. The patient is usually given intravenous drugs that inhibit cell growth. The effect affects not only the tumor, but also all other cells.

Treated patients therefore typically suffer from hair loss, as hair cells also divide very often and are particularly affected by the chemotherapeutic agent. Streptozotocin, for example, to which pancreatic cells react primarily, comes into question as an active ingredient. Common side effects of streptozotocin are nausea, diarrhea, vomiting, and liver and kidney failure.

In addition, the active ingredient itself can be carcinogenic and promote the development of tumors; So far, however, scientists have only been able to observe this effect in animals. Untreated, functionally active glucagonomas, in particular, can be fatal. The tumor produces glucagon, which promotes the release of glucose.

Insulin, which the organism also produces in the pancreas, acts like a key on the body's cells and allows them to absorb the glucose. An excess of glucagon inhibits the effect of insulin - the result is, on the one hand, an excess of glucose in the blood and, on the other hand, cells that suffer from a lack of energy and ultimately starve to death. This condition corresponds to the metabolic disease diabetes mellitus.

The diabetes may have to be treated as well; the same is true of other symptoms. After removal of the tumor and taking the prescribed hormone substitutes, the consequences may disappear. Regular follow-up exams 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.

The glucagonoma requires surgery and, depending on the individual circumstances, chemotherapy. If the cancer cells in the organism have already spread beyond the pancreas and attacked other organs, the prospect of a cure drops drastically. For these patients, the treatment plan is geared towards relieving the existing symptoms.

So that the person affected feels as few impairments as possible and is not exposed to unnecessary pain. If the tumor can be removed successfully in an operation, the prospect of relief increases. 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. A recurrence of the tumor disease is to be expected in many of those affected and numerous complications and secondary diseases are possible.

prevention

General preventive measures that can help prevent glucagonomas and other cancers include avoiding radioactive rays, UV rays, chemical carcinogens, and other factors.The personal lifestyle also has an influence on the individual risk of cancer.

Smoking, obesity and bad eating habits are among the most important factors in this area. In addition, doctors and health insurance companies recommend regular preventive examinations for certain types of cancer in order to detect tumors at an early stage.

Aftercare

The options for follow-up care 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 complaints are avoided and limited. Self-healing does not occur in a glucagonoma. In the worst case, the affected person dies from the consequences 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.

Furthermore, a healthy diet and a healthy lifestyle in general can have a positive effect on the course of this disease. Those affected are often dependent on taking medication, whereby it is important 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 sufferers of this disease can also have a positive effect on the further course.

You can do that yourself

If a glucagonoma is diagnosed, medical treatment is required. However, those affected can do some things themselves to alleviate 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. The 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. It is also important to treat the underlying disease. If the glucagonoma is caused by diabetes, the diet must be changed. Any excess weight should also be reduced and sport should be exercised regularly. This also helps against accompanying symptoms such as fatigue, lack of energy and slowed 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 give further tips with which the recovery can be promoted independently.