Rhabdomyosarcoma is a tumor that occurs mainly in children under 5 years old and adolescents. It is a disease in which malignant cells form in muscle tissue. This tumor can occur in any part of the body.
The symptoms depend mainly on the initial location of the tumor as rhabdomyosarcoma often manifests as a lump that you can see or feel.
The most common areas of the body to be affected are the head or neck and the urinary tract.
Orbital tumors cause the eye to bulge, which leads to asymmetry in the position of the eyes, strabismus or pushes the eye forward. The child may complain of headaches or strange vision as their two eyes are not providing the same information.
Nasopharyngeal tumors (nose and pharynx) gradually lead to a long-term blocked nose and nasal voice. They can also erode the bones at the base of the skull and produce symptoms of cranial nerve involvement which can be identified by the general practitioner.
Tumors of the urinary tract develop from the bladder or prostate. They give rise to urinary problems ranging from repeated infection and bladder control problems (leaking, urgency, etc.) to difficulties emptying the bladder (pain in the lower stomach, major urinary retention).
If it occurs elsewhere, the tumor presents as an isolated mass, developing most commonly in children of good general health. In rare cases, the disease is widespread from the outset. Patients typically present a more pronounced change in general condition (tiredness, fever, loss of appetite, etc.)
A biopsy of the tumor is performed early in order to obtain further information about the type of rhabdomyosarcoma. There are effectively two types of disease, embryonal or alveolar, for which the treatment differs in intensity.
It is important to find out exactly where the tumor started which is typically identified by performing an MRI scan of the area in question. This is used to accurately determine whether or not the mass has spread to surrounding tissues.
A PET Scan and aspiration (bone marrow biopsy) may also be performed to identify any remote lesions.
Other important examinations are also requested to ensure that the patient is able to tolerate the treatment at the doses usually administered:
- Echocardiogram, ECG
- Assessment of the kidney function
Treatment generally starts with chemotherapy which aims to reduce the volume of the initial mass in order to facilitate its excision.
Surgery is then carried out to remove as much of the lesion as possible. The extent of the procedure is discussed in a multidisciplinary team meeting to ensure maximum excision while preserving the functionality of the adjacent organs as far as possible.
Chemotherapy is then resumed.
Radiotherapy can be added to this part of the treatment if the medical team think that it could enhance the surgical treatment. Local control of the disease is critical as, in most cases, relapses occur in this area. Radiotherapy can be given in different forms in order to combine maximum efficacy with minimum side effects (conventional radiotherapy, brachytherapy, proton therapy).