Neuroblastoma is a cancer and the second most common malignant growth, after tumors in the brain, in children. In Germany, about 150 children are affected by neuroblastoma each year, and the survival rate depends greatly on the stage of the tumor.
What is neuroblastoma?
Graphic illustration and infogram of a typical cancer cell. A neuroblastoma is a solid, malignant tumor that arises from degenerate cells of the sympathetic nervous system. This nervous system controls, among other things, the heart and circulation or intestinal activity. If the immature cells of this nervous system degenerate, a neuroblastoma forms. The degeneration of the immature cells begins prenatally. The tumor occurs most frequently in the adrenal medulla (about 50 percent of all cases) or on both sides of the spinal column in the so-called border cord. If this boundary cord is affected, the tumor can arise along the entire spine, i.e. in the abdominal, thoracic or cervical region. Neuroblastomas may be confined to the site of origin or may spread, i.e., metastasize. Metastases are not limited to single sites, and may appear in the lungs, kidneys, brain, or lymph nodes. Neuroblastomas have the peculiarity of regressing spontaneously in some cases, for which doctors have not been able to find a reason to date. In more than 50 percent of all cases, neuroblastomas occur within the first 15 months of life, with boys being affected more often than girls. With increasing age, the risk of developing neuroblastoma decreases. However, adolescents and adults can rarely be diagnosed with neuroblastoma.
Causes
Neuroblastoma is caused by degenerated cells of the sympathetic nervous system. Because the degeneration often occurs before birth, genetic mutations and spontaneous chromosomal changes may be the reason for the cell change. Genetic inheritance has not yet been proven, but there are families in which neuroblastoma occurs more frequently (about 1 percent of cases). Nutrition during pregnancy, stress, and environmental factors have also not yet been shown to be causes of cell degeneration.
Symptoms, complaints, and signs
- In the neck: a Horner syndrome in 15 to 20 percent.
- In the chest area: shortness of breath
- In the abdominal area: abdominal pain, back pain, urinary tract, intestinal problems.
- Pain, pallor, fever and weight loss.
- If necessary, high blood pressure or diarrhea
Diagnosis and course
In neuroblastoma, many initially many patients have no symptoms at all. These only appear later with advanced tumor growth or metastasis. When symptoms do occur, they usually depend on the location of the tumor. Thus, abdominal pain, fever, diarrhea, sore throat, fatigue and a general feeling of illness may occur. However, since these are all nonspecific symptoms, the physician must use various tools such as magnetic resonance imaging, X-rays, computer tomography and laboratory tests to make a diagnosis. If a tumor is discovered, further examinations can clarify whether individual organs are affected. A final confirmation of the diagnosis is obtained by a fine tissue examination of the tumor. To detect or rule out metastasis, an MIBG scintigraphy is performed, in which a radioactive substance is injected into the patient’s bloodstream.
Complications
Neuroblastoma leads to many different symptoms and complications. As a rule, the further course of this disease depends very much on its cause and the particular region of the body affected. For this reason, a general prediction about the further course is usually not possible. In most cases, those affected suffer from diarrhea and high blood pressure. Fever and weight loss may also occur. Not infrequently, neuroblastoma also leads to pain in the abdomen and back and can have a very negative effect on the patient’s digestion. Furthermore, the course of this complaint depends very much on the time of diagnosis, so that there is not always a positive course of the disease. In the most extreme stage, the affected person dies from the symptoms of neuroblastoma. Furthermore, it is not uncommon for the parents and relatives of the child to be affected by neuroblastoma and suffer from psychological symptoms. No particular complications occur during the treatment of this disease.However, a positive course cannot always be guaranteed. It is also possible that the patient’s life expectancy is significantly reduced by neuroblastoma.
When should you see a doctor?
If children show abnormalities and health changes, special care is needed. Because neuroblastoma is a tumor disease, a visit to the doctor is recommended at the first sign of an irregularity. The sooner a diagnosis is made possible, the better the prospects of cure. Otherwise, in severe cases, the child may die prematurely. If there are restrictions in breathing, a shortness of breath or interruptions in breathing activity, a doctor is needed. If the child complains of abdominal pain, a general malaise or back pain, a visit to the doctor is advised. If there are disturbances of the digestive tract, a decrease in play activities, or other behavioral problems, consultation with a physician is indicated. Diarrhea or refusal to eat are signs of health impairment. A pale appearance, increased body temperature, and change in weight are worrisome and must be presented to a physician. If abnormalities occur in the area of the face or neck, these should be examined. Restricted vision, changes in the retina or a pigment disorder are warning signs of the organism for an existing disease. A visit to the doctor should be initiated as soon as possible to investigate the cause. If existing symptoms persist for several days or increase in intensity, a doctor must be consulted immediately. An ambulance service should be called in the event of an acute health condition.
Treatment and therapy
After diagnosis of neuroblastoma, treatment planning should begin as soon as possible. Special consideration should be given to the stage of the tumor, the age of the patient, and possible metastatic disease. A distinction is made between the following stages:
1. the tumor is confined to the site of origin and is completely operable. 2. the tumor is operable, but residual tumor can still be detected. 3. the tumor is inoperable, but lymph nodes may already be affected. 4. metastases in brain, liver, bone marrow or other organs are present. 5. criteria according to 1. and 2. but the patient is under 18 months and there are few to no metastases. In this case, the tumor may regress spontaneously. Treatment of neuroblastoma usually consists of a combination of chemotherapy and surgery. Often, tumors can be surgically removed only after they have been reduced by radiation; in other cases, the neuroblastoma is first removed and then radiation treatment of any residual malignant tissue that may be present is begun. Other therapeutic procedures may also be used adjunctively to treat neuroblastoma. These include MIBG therapy, stem cell transplantation or treatment with retinoic acid. Each individual case requires individualized treatment, dictated primarily by the stage of the tumor. For example, in stage 1, it may be advisable not to use radiotherapy after surgical removal, but to wait and see. If the tumor has already spread and metastasized to other organs, high-risk therapy is usually used, consisting of surgery, radiation therapy and chemotherapy. It can last up to 2 years.
Outlook and prognosis
Basically, the disease course of neuroblastoma should be considered individual. In neuroblastoma, the age of the patient, in addition to the stage of development, proves to be decisive for the course of the disease and prognosis. The prognosis is better in young patients or those in the early stages of the disease. About 75 percent of patients diagnosed with neuroblastoma survive the following fifteen years. In low-risk patients, the survival rate is even higher – it is even over 95 percent here. The prognosis is considerably worse for high-risk patients. Here, the rate after five years is only 30 to 40 percent. Even if the tumor has been completely removed, some patients will still relapse after a certain period of time. The majority of recurrences occur in the first few years following therapy. Patients should therefore undergo regular check-ups, especially within ten years of the end of therapy.In addition to physical examinations, this also includes laboratory tests and examinations using imaging techniques. In this way, a possible recurrence of neuroblastoma can be identified at an early stage. Neuroblastoma cannot yet be specifically prevented. Since this disease occurs mainly in children and adolescents, parents have a special responsibility here. This includes creating a good, stable environment characterized by harmony for those affected.
Prevention
One cannot prevent neuroblamstomas according to current scientific knowledge. Neuroblastoma is usually discovered by chance, so physicians have been trying for years to find a reliable early detection method. In addition to marker tests, neuroblastoma screening has therefore been developed in Germany and Canada. However, it cannot yet be said whether this really improves early detection.
Follow-up
After intensive medical treatment of the tumor, rehabilitation and follow-up care of the patient begin. As part of tumor aftercare, regular checkups and patient consultations are performed. Specifically, follow-up care includes a thorough physical examination, laboratory tests, and imaging studies such as ultrasound and X-ray. If necessary, further imaging procedures can be used, for example, to examine specific regions of the body or to detect side effects and drug interactions. Close monitoring enables early detection of any recurrences. Possible concomitant diseases or consequences of the therapy are also identified and treated during follow-up care. In addition, aftercare includes therapeutic support for the patient and his or her relatives. Affected children in particular must receive comprehensive care while still in the hospital. Within the framework of aftercare, the persons involved are informed of the necessary contact points and measures in order to cope with the disease in the best possible way. The attending physician accompanies both the examinations and the care of the patient. The follow-up can last for several years. Provided that no complications occur, the intervals between examinations are gradually extended. In case of complications such as recurrences or concomitant diseases, a resumption of therapy is necessary. Follow-up is usually discontinued in the event of major complications.
What you can do yourself
Because children and adolescents are increasingly affected with this disease, parents have a special responsibility. It is extremely important to create a good, harmonious and stable environment for those affected. The children should be seen and included as full members of the family. However, it is important that prescribed treatments are regularly attended. Supportive homeopathic preparations or other natural products can also be taken in consultation with the doctor in order to alleviate the course of the disease or to relieve the body. A lifestyle that is almost “normal” will provide patients with an easier daily routine. This includes meeting friends, attending school, and doing lots of things that bring pleasure. Of course, this must be approved by the attending physician, taking into account the stage of the disease. Otherwise, attention should be paid to healthy, high-quality food, sufficient rest and sleep, and adequate exercise in the fresh air to provide the body with good things. Many sufferers find it pleasant to go to a self-help group. In this way, they can exchange information with other sufferers and encourage each other. Those who wish to take advantage of psychological support will also benefit. This helps sufferers to accept the disease and live with it.
