Paraneoplastic Syndrome

A paraneoplastic syndrome is a concomitant disease of cancer. However, it is not the result of the tumor, but develops parallel to it. Sometimes the typical symptoms of a paraneoplastic syndrome point to an as yet undiscovered and initially symptom-free malignant tumor.

What is Paraneoplastic Syndrome?

A paraneoplastic syndrome is always a concomitant disease to a malignant tumor. It never develops primarily as a result of neoplasia. Sometimes the symptoms of a paraneoplastic syndrome appear without any tumor-related symptoms being noticeable.

The typical symptoms of certain paraneoplastic syndromes can often facilitate the diagnosis of the actual cancer. The symptoms are not the direct result of masses or tissue damage caused by the tumor. Rather, they develop through the increased tumor-related release of certain active substances.

These can be hormones, antibodies, enzymes or inflammatory factors. The individual clinical pictures are correspondingly diverse. Paraneoplastic syndromes affect, among other things, the endocrine system, the skin, the digestive system, the nervous system, the blood or the joints.

Many typical symptoms occur even without an underlying cancer. However, some paraneoplastic syndromes are only found in association with cancer.

causes

The cause of the paraneoplastic syndrome is the increased formation and release of biologically active substances. There are paraneoplastic syndromes that resemble typical hormonal disorders because the tumor produces more certain hormones. Endocrine paraneoplastic syndromes are caused, among other things, by neuroendocrine tumors in the pancreas.

These include the so-called insuloma with an overproduction of insulin, the gastrinoma with an increased formation of gastrin, which stimulates gastric acid production, or the vipoma with severe therapy-resistant diarrhea. Around 20 percent of all tumor patients develop a paraneoplastic syndrome.

The largest proportion with up to 40 percent are bronchial carcinomas. In addition to the hormone-related paraneoplastic syndromes, the increased formation of antibodies against cancer cells can also lead to autoimmune-like diseases if these also attack healthy body cells as part of a cross-reaction.

The prognosis of tumors with antibody-related paraneoplastic syndromes is more favorable than tumors without accompanying symptoms, since the former have a stronger immune reaction against the cancer cells. Some tumors secrete inflammatory factors such as prostaglandins.

Skin changes, rheumatic complaints or gastrointestinal disorders can occur. Special paraneoplastic syndromes affect the nervous system. Autoantibodies attack nerve cells and cause neurological complaints.

In addition, certain tumors also produce substances that lead to haematological changes. Overall, a multitude of different paraneoplastic syndromes can develop in this way.

Symptoms, ailments & signs

Paraneoplastic syndromes are expressed by general and specific symptoms. Many cancers are accompanied by a general complex of symptoms consisting of cachexia, increased heat build-up, sweating, thrombosis, leukocytosis or anemia.

The cachexia manifests itself in constant emaciation, although enough calories are ingested. This leads to increased activity of mitochondria in cancer cells, which leads to very high energy consumption. The metabolism is greatly accelerated, the production of heat is increased and fat burning is boosted.

The emaciation in the context of cachexia is therefore not the result of a lack of food, but rather that of increased energy consumption. Special symptoms include hormone-related disorders. Certain forms of the so-called Cushing's syndrome occur in bronchial, liver cell or kidney carcinomas.

The increased release of ACTH stimulates the adrenal cortex to increase the release of cortisol. This leads to trunk obesity with a bull's neck and moon face and a weakening of the immune system with an increased susceptibility to infections. In an insuloma, too much insulin is produced. The blood sugar level drops dramatically. Other hormones that can be increased by carcinomas are parathyroid hormone (PTH), vasopressin (ADH), thyrotropin (TSH), calcitonin or serotonin. The calcitonin lowers the level of calcium in the blood. An increased calcium level is caused by the parathyroid hormone.

Increased serotonin concentrations lead to persistent diarrhea and abdominal pain, among other things, because the intestinal peristalsis is constantly stimulated.The TSH in turn stimulates the production of thyroid hormones in the thyroid gland, which causes the typical symptoms of an overactive thyroid gland. Antibody-mediated reactions can lead to autoimmune diseases.

This can lead to rheumatic joint inflammation, inflammatory processes in the gastrointestinal area or neurological failures. If the skin is involved, the main symptom is excruciating itching. Skin lesions, keratoses or an increased growth of body hair are also observed.

Diagnosis & course of disease

Depending on the symptom complex, hormone tests or tests for antibodies can reveal the cause of certain symptoms. Imaging techniques can reveal the underlying tumor.

Complications

In most cases, this syndrome is accompanied by various complications and complaints that mainly occur during cancer. The complaints themselves are very different and can vary greatly from one another. As a rule, for this reason, no general prediction can be made about the further course of the disease.

Those affected suffer from heavy sweats and also from thrombosis. It also results in anemia and thus bleeding or fatigue and exhaustion of the patient. The metabolism of those affected is also affected by this syndrome, so that in most cases the patients also lose weight.

The immune system is significantly weakened by the tumor disease, so that infections or inflammations occur more often, which have a very negative effect on the quality of life of the person affected. Itching or lesions on the skin can also occur. Treatment of this syndrome is carried out by treating the tumor. It cannot be predicted whether this will be successful. The tumor may also reduce the patient's life expectancy.

When should you go to the doctor?

Paraneoplastic syndrome only occurs in people who have cancer. Therefore, at the first irregularities as well as indications of a health impairment, a doctor should be visited. Those affected suffer from symptoms such as diarrhea, internal weakness and a general feeling of illness. If you lose weight or sweat, see a doctor. If the person concerned suffers from heat build-up, circulatory disorders and general malaise, a visit to a doctor is advisable.

Changes in the appearance of the skin, a decrease in physical performance and an increased need for sleep must be examined and treated by a doctor. Disorders of the digestive tract, rheumatic complaints, an increased susceptibility to infections as well as repeated fungal infections or an attack on other germs indicate a disease. A doctor should be informed of the observations and a follow-up visit should be made. Disorders of the endocrine system, changes in libido or emotional abnormalities should be discussed with a doctor.

A depressed mood, peculiarities of behavior as well as changes in personality are to be understood as warning signs of the organism. A visit to a doctor is advisable so that research into the cause can be initiated. In addition, regular participation in the preventive examinations offered is generally recommended in adulthood. In an early diagnosis, the first changes or abnormalities in health can be documented.

Treatment & Therapy

The cause of paraneoplastic syndromes can be treated by removing the tumor. This usually also makes the symptoms go away. This applies in particular to endocrine-related paraneoplastic syndromes. However, there are also cases where the antibody activity remains high after the tumor has been removed.

Of course, the individual tumor comorbidities can be treated separately. Drug therapies or immunosuppression therapies have proven effective in the case of antibody-related paraneoplastic syndromes. Each paraneoplastic syndrome requires its own therapeutic approach. However, if possible, the causal tumor treatment should be sought.

Outlook & forecast

Paraneoplastic syndrome is not an isolated case as there are several corresponding syndromes. These are viewed by medical professionals as complications of a wide variety of tumor diseases. Paraneoplastic syndromes are not caused by the tumor itself or directly by its metastases. Neither are tumor-related, vascular, metabolic, infectious or treatment-related effects involved in the development of a paraneoplastic syndrome.

However, paraneoplastic syndrome can occur without a recognizable tumor. In this case, it is standard practice to regularly check for a tumor over a period of two to four years. It has already been proven that paraneoplastic syndrome occurs in many cases in connection with tumor formation. A distinction must be made between idiopathic and true paraneoplastic syndromes.

The diagnosis of such syndromes is already very complex and difficult. Treatment is also difficult. But it is by no means impossible. Instead of conventional tumor therapy, immunosuppressants are usually used - but only if no tumor could be found. Symptomatic therapies such as pain therapy or other measures are also possible.

What is certain is that the type of paraneoplastic syndrome can be used to determine what type of tumor it could be. If this can be discovered at an early stage through regular searches and removed surgically, the prognosis improves.

prevention

There is no general recommendation for the prevention of a paraneoplastic syndrome, as this always accompanies a certain tumor disease. However, the focus of prevention is on the tumors. For example, the risk of developing bronchial cancer can be reduced by adopting a healthy lifestyle and not smoking.

Aftercare

Paraneoplastic syndrome is related to cancer. The effects are not directly attributable to the tumor and are likely to be due to an immune reaction or a hormonal change. The aftercare of the paraneoplastic syndrome is therefore connected with the aftercare of the actual cancer and merges with it.

Depending on the cause, even after the tumor has been removed, the paraneoplastic syndrome may not necessarily resolve itself. The body's reactions persist especially when the development of the disease is caused by antibodies that also attack healthy tissue in the body. In the follow-up care, the specialist, in this case an oncologist, will take care of the symptoms of the syndrome and treat them during appropriate check-ups.

Since the symptoms of paraneoplastic syndrome are of various kinds, one cannot assume that there will be standardized follow-up care. The way in which the symptoms subside also varies greatly from person to person and is linked to the underlying cancer and, accordingly, to the trigger for the paraneoplastic syndrome. It is not possible to say in general whether medication or other aids are required in aftercare. The oncologist can advise the patient individually. The follow-up appointments should be kept conscientiously.

You can do that yourself

Since paraneoplastic syndrome is often a parallel disease to a malignant tumor, this tumor must be discovered and treated - if it has not already been done. Otherwise, the paraneoplastic syndrome shows up with different symptoms, which should be addressed individually.

A major focus here is diet as patients can develop life-threatening cachexia. The cancer cells consume so many calories that the patient can no longer take in enough nutrients, even with a normal diet. There is a tumor-related weight loss. In this case, high-calorie, but healthy foods such as avocados, bananas or peanut butter are recommended as a spread.

Paraneoplastic syndrome also weakens the immune system. Patients who want to do something for themselves are therefore advised to do everything that strengthens the immune system: This includes adequate sleep as well as a healthy diet with fresh, high-fiber food, but little sugar and fat. Probiotics that enrich the intestinal flora are also recommended. Of course, the patients should not smoke or drink alcohol in order not to unnecessarily burden the organism. Studies show that exercise can be as important as medication. Even short walks in the fresh air activate the immune system, regulate the metabolism and thus ensure a better quality of life.