Rhabdomyosarcoma is one of the most malignant soft tissue tumors of childhood, which can be localized in any part of the body and consists of primitive muscle cells. The favorite localization of rhabdomyosarcoma is head and neck region (30% of all such tumors), with up to 7% of cases affecting the orbit.
There are two histological types of rhabdomyosarcoma:
- Embryonic – 60% of cases.
- Alveolar – 40% of cases.
Rhabdomyosarcoma is extremely rare among adults. 87% of cases are children under 15 years of age. There are 2 age peaks in incidence of rhabdomyosarcoma: children 2-6 years old, who are most often diagnosed with rhabdomyosarcoma of head and neck, as well as damage to urogenital tract, and adolescents 14-18 years old, who are diagnosed with damage to extremities, trunk and paratesticular localization of tumor.
Specialists at Pediatric Oncology Department at Tel Aviv Medical Clinic have unique experience in treating orbital rhabdomyosarcoma in children of all ages. To treat this tumor, only modern and safe methods are used, organ-sparing surgeries, modern chemotherapy drugs and immunobiological drugs of the latest generation are used. TAMC Oncology Ophthalmologists not only save children lives with orbital rhabdomyosarcoma, but also preserve their vision.
Despite the fact that rhabdomyosarcoma is an aggressive and fast-growing tumor, TAMC specialists, if they seek medical help in a timely manner, help to save life and vision of 90% of children with this diagnosis.
Causes of orbital rhabdomyosarcoma
To date, the exact cause of rhabdomyosarcoma development is unknown. But researchers were able to identify several factors that increase the risk of developing this type of malignant tumor:
- Age. The younger the child, the higher his chances of developing rhabdomyosarcoma.
- Children under 10 years of age are a special risk group.
- Gender. Boys get sick more often than girls.
- Ethnicity. European children have a higher risk of rhabdomyosarcoma.
- The child has certain hereditary genetic syndromes or diseases: Li-Fraumeni syndrome, neurofibromatosis type 1, Costello syndrome, Noonan syndrome, Beckwith-Wiedemann syndrome.
Symptoms of orbital rhabdomyosarcoma in children
Orbital rhabdomyosarcoma is characterized by rapid onset of symptoms, since the tumor itself grows and progresses very quickly. Main symptoms:
- Swelling and redness of eyelid that does not go away for a long time and does not respond to standard symptomatic medications.
- Protrusion of one eye from orbit (exophthalmos). Displacement of eyeball to one side (depending on tumor location), impaired mobility.
- Development of persistent ptosis (drooping of eyelid).
- Inability to completely close the eye due to its protrusion.
- Watery eyes, pain in the eyes.
Modern diagnostic methods
The peculiarity of diagnostics in TAMC is its complexity, speed and accuracy. All necessary studies can be completed in one place within 3-5 working days. Pediatric Oncology Department is equipped with only the best expert-class diagnostic equipment, so diagnostic process becomes not only effective, but also safe.
Diagnostic program for rhabdomyosarcoma includes:
- Studying child’s medical history, identifying specific risk factors for a given tumor type development, blood tests and other examinations to determine child’s general health.
- Methods of modern medical imaging – CT, MSCT, MRI, PET-CT, PET-MRI, SPECT, bone scintigraphy. These techniques make it possible to visualize the tumor in detail, determine its size and location, the degree of spread to surrounding tissues, and distant metastases presence. Based on obtained data, doctor sets the stage of rhabdomyosarcoma, according to which a set of therapeutic measures is planned.
- Tumor biopsy and subsequent pathohistological examination of tissue samples. This allows us to confirm the diagnosis, determine rhabdomyosarcoma histological variant, and also makes it possible to conduct modern molecular genetic studies to select the most effective modern therapy, for example, targeted drugs.
Prognosis for orbital rhabdomyosarcoma
The prognosis for children with orbital rhabdomyosarcoma depends on several factors:
- Tumor localization.
- Possibility of complete tumor conglomerate surgical removal.
- Stage of the disease.
- Histological variant of rhabdomyosarcoma and its molecular genetic features.
- Child’s age.
Based on combination of these factors, specialist identifies a risk group for orbital rhabdomyosarcoma, according to which a treatment protocol is planned.
Overall survival varies from 90% in low-risk children to 50% in high-risk patients.
Modern treatment of orbital rhabdomyosarcoma in children
Rhabdomyosarcoma requires complex treatment, which includes tumor surgical removal, polychemotherapy and radiation; in some situations, as part of clinical trials, patients may be prescribed immunobiological therapy, as well as high-dose chemotherapy followed by autologous hematopoietic stem cell transplantation.
The question about possibility of radical surgery for orbital rhabdomyosarcoma is decided individually for each patient and depends on tumor size, its location and spread degree to surrounding tissues. With a small tumor that does not grow into neighboring anatomical structures, it can be completely removed during one surgery. Our ophthalmological surgeons skillfully perform such interventions using minimally invasive techniques. In some cases, several interventions are required to remove the entire tumor conglomerate. Subsequently, TAMC specialists perform unique reconstructive surgeries on such patients to avoid deformation of child’s face and maintain a natural appearance.
Radiation therapy is carried out both before and after surgery. In some cases where surgery is not indicated, radiation may be primary treatment for rhabdomyosarcoma. Radiation therapy for orbital rhabdomyosarcoma can be remote with application of the latest generation linear accelerators, and contact radiation therapy (brachytherapy) is also used.
The main types of radiation therapy for orbital rhabdomyosarcoma:
- intensively modulated radiation therapy (IMRT);
- fractionated stereotactic radiosurgery;
- brachytherapy (introducing a radiation source directly into the tumor or surrounding healthy tissue).
The type of radiation therapy, the total dose of radiation, the duration and frequency of sessions in each case is determined individually by an experienced radiation therapist at Pediatric Oncology Department.
Chemotherapy can be used before surgery to improve the results of surgery, and after surgery to prevent recurrence. Chemotherapy can also be used independently if patient is not indicated for surgery. To treat patients with this diagnosis, the following drugs are used in the protocols: vincristine, actinomycin D, irinotecan, cyclophosphamide.
Many children are offered treatment as part of a clinical trial if basic treatment protocols have failed. Today, some immunobiological drugs, for example, targeted drugs, are being tested. They have a targeted effect on malignant cells and block their growth and reproduction. Monoclonal antibodies and kinase inhibitors are being studied for rhabdomyosarcoma treatment.
Soft tissue sarcomas, including orbital rhabdomyosarcoma, can recur even years after recovery. Therefore, after treatment completion, patients are advised to undergo regular examinations for relapse. Specialists will give specific recommendations to each child on necessary examinations and frequency of medical examinations.
