A rhabdomyosarcoma belongs to the group of soft tissue tumors; rhabdomyosarcomas arise from degenerated or not fully mature cells of the muscle or connective tissue. Almost exclusively children are affected by rhabdomyosarcoma; 87% of all patients are younger than 15 years. Boys are affected slightly more often than girls.
What is rhabdomyosarcoma?
A rhabdomyosarcoma develops from degenerate cells of muscle tissue. Rhabdomyosarcoma can occur virtually anywhere in the body, although a cluster of sites of origin has been noted in the sinuses, nasopharynx, orbits, bladder, and vagina. Rhabdomyosarcoma very rarely affects adults, usually occurring in children under 15 years of age. Boys are more likely to be affected than girls, with a 1 in 4 chance.
Causes
Causes of rhabdomyosarcoma have not been clearly established. However, there is a correlation between the occurrence of certain previous diseases and the occurrence of rhabdomyosarcoma. For example, prior radiation therapy or infection with HIV or EB virus have a negative effect on the likelihood of developing rhabdomyosarcoma. A genetic link is also suspected, particularly disease of the genetic Li-Fraumeni syndrome. Most likely, the tumor cells originate from mesenchymal cells – embryonic cells that later give rise to muscle and connective tissue.
Symptoms, complaints, and signs
Complaints of rhabdomyosarcoma depend on the extent and location. Most commonly, the head, genitourinary tract, and extremities are affected. Adults rarely suffer from the disease, children the more frequently. Most often, the tumors spread the disease to other nearby areas of the body. Metastases are formed. The brain and the pelvic organs are conspicuously often affected. There are hardly any limits to the individual severity of the symptoms. Typical for a rhabdomyosarcoma in the head and neck region is the protruding of the eyeballs. Sometimes swelling of the jaw is also present. Breathing through the nose is difficult. Some patients even report hearing disorders. If the urogenital tract is affected, patients complain of pain when urinating. A burning sensation accompanies urination. The excretion is not infrequently mixed with blood. Very often, abdominal pain also occurs, indicating the extension of the infestation. Depending on the sex, there are other signs. Male patients have swollen testicles, and female sufferers have vaginal bleeding. If the rhabdomyosarcoma occurs on the extremities, swellings develop there. These do not necessarily have to be painful. In addition, the affected body appendages can no longer be moved as usual.
Diagnosis and course
Rhabdomyosarcoma forms clearly palpable swellings and tumors. Less commonly, tumors may appear that are too small to palpate or are visible only under a microscope. The patient will also experience pain and, depending on the location and size of the tumor, may have significant limitations in movement. If a physician can palpate tumors or detect them after a sonography (examination with ultrasound), he will initiate further diagnostic steps. These essentially include biopsy. He will use a thin needle to remove tissue under local anesthesia and then have it pathologically examined. The procedure is performed on an outpatient basis and does not cause any pain. The laboratory physician can conclude a rhabdomyosarcoma based on the cell composition. After a positive finding, the physician will clarify the degree of the disease. To do this, he will use a computed tomography scan or a magnetic resonance imaging (MRI) scan to determine whether the tumor has already metastasized or whether only the primary tumor is present. He will also determine if it is a recurrence (cancer that has reappeared from a previous, same cancer). Depending on the grade of the disease, the physician will take further therapeutic steps.
Complications
Rhabdomyosarcoma causes death within a few weeks or months if left untreated because it spreads rapidly through the bloodstream and lymphatic system. Today, however, the prognosis has improved decisively because of intensive treatment with much more effective chemotherapeutic agents.Furthermore, the prognosis and the development of complications depend on which form of rhabdomyosarcoma is involved. Thus, alveolar rhabdomyosarcoma grows much more aggressively than the so-called embryonal rhabdomyosarcoma. The formation of metastases also takes place much faster in alveolar rhabdomyosarcoma. The same is true for the risk of recurrence. Although rhabdomyosarcomas can occur in almost all organs, they prefer the head and neck region, the limbs, and the urinary and genital organs. The symptoms that occur depend on the location of the particular rhabdomyosarcoma. Abdominal pain, pain during urination and blood in the urine are common. Metastases are first found in the adjacent lymph nodes, skeletal system and lungs. Complete cure of rhabdomyosarcoma is possible today if intensive cancer therapy begins before metastases appear. However, successful surgical removal of the tumor also depends on its location. After complete removal, there is still follow-up treatment with radiation therapy. Tumors that have not been completely removed can still recur years later. In addition, the risk of metastases forming is increased in this case.
When should you go to the doctor?
Since rhabdomyosarcoma is a tumor, it should always be treated by a doctor. There is no self-healing in this disease, and rhabdomyosarcoma cannot be treated by self-help measures. In the worst case, if left untreated, it will lead to the death of the affected person. An early diagnosis of the disease always has a very positive effect on the further course of the disease. As a rule, the doctor should be consulted in the case of rhabdomyosarcoma if the affected person suffers from severe swelling of the eyes or protruding eyeballs. In this case, the affected person’s breathing is also impeded, so that strenuous activities or sports activities cannot be carried out without further ado. In girls, rhabdomyosarcoma often leads to vaginal bleeding that occurs for no particular reason. Pain during urination may also be indicative of this disease and should be examined and treated by a physician. Rhabdomyosarcoma is usually diagnosed in a hospital. Further treatment is then given by a specialist depending on the symptoms. Under certain circumstances, the life expectancy of the affected person is also limited by the rhabdomyosarcoma.
Treatment and therapy
As a rule, surgical removal of the tumor is performed first. A computed tomography scan or magnetic resonance imaging scan obtained as part of the diagnostic workup facilitates surgical planning and delineates the area in which radiation is subsequently administered. In addition to radiation therapy, chemotherapy is attempted. Both are intended to prevent any remaining tumor cells from spreading or growing further. If the tumor is inoperable, chemotherapy is first used to try to shrink the tumor so that surgical removal is still possible afterwards. If the combined therapy of tumor removal, radiotherapy and chemotherapy is successful, the prognosis is good. The 5-year prognosis is 70%. If metastases have already formed, the prognosis is about 30%. If it is a recurrence, the prognosis is significantly lower.
Prevention
Rhabdomyosarcoma cannot be prevented. However, it is recommended that children suffering from unfavorable previous disease (EBV or HIV infection, previous radiation therapy, presence of Li-Fraumeni syndrome) be presented to a physician at close intervals. If a child repeatedly complains of pain in the throat, nose, eye sockets or vagina, he/she should also be presented to a doctor. After the rhabdomyosarcoma has been overcome or treated, the affected person should also be examined closely for a recurrence of the disease. Especially for children at risk, a healthy lifestyle – healthy diet, plenty of exercise in the fresh air – should be used to curb the likelihood of developing cancer.
Follow-up
The disease is very serious and it is imperative that doctors treat it. However, there are many therapies that sufferers can do at home. In this way, the discomfort can be alleviated. For sufficient regeneration, affected persons should take sufficient rest after treatment.As the immune system is extremely weakened after treatment, contact with sick people in the social environment should be avoided. Affected persons should have an intact sleep hygiene. Sufficient sleep is very important after treatment. A balanced diet rich in vitamins and sports should also be started after treatment. This strengthens the immune system considerably. Since the disease in most of the cases is a great burden for those affected, it is recommended to undergo permanent psychological counseling. Going to a self-help group can also help sufferers learn how to live with the disease. In the self-help group, sufferers can also exchange ideas with other sufferers and learn new ways of coping with the disease. As a result, many do not feel alone. Close relatives should also be sufficiently involved in life. They can also support and help those affected. It is imperative to avoid drugs containing nicotine and alcohol, as this can lead to unwanted complications.
What you can do yourself
Rhabdomyosarcoma is a serious disease that belongs in the treatment of experienced doctors. However, self-help in everyday life can support therapies or alleviate their consequences. Depending on whether surgery, radiotherapy or chemotherapy are or were used, the patient is advised to take it easy for optimal recovery. Due to the weakened immune system, it is better to avoid contact with people who are ill with an infection. A good night’s sleep is just as important as a healthy diet and adequate drinking. The thought of suffering from a malignant disease is often stressful. Here, self-help groups help people to come to terms with this finding. It is often children and adolescents who are affected by rhamboymyosarcoma. They benefit from psychologists or psychooncologists who specialize in adolescents. Parents can then be involved in these therapies in a goal-oriented way. This also applies to teachers, so that children can continue to learn in a well-dosed manner during the illness and do not lose touch with the class. Exercise is a factor that can also be integrated into self-help. Here, the patient’s invidual strengths must be taken into account. A walk in the fresh air is just as helpful as some fitness after surviving therapies. Adult patients better refrain from nicotine and alcohol, since vascular toxins are naturally not beneficial in cancer.
