الفهرس 
Aetiology and geneticsOnly 14 pages are availabe for public view
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Abstract
Rb is the most common primary intraocular malignancy in childhood. There have been many recent advances in the diagnosis and treatment of children with Rb.
The management of Rb is difficult, and the ophthalmologist often must make difficult decisions regarding the use of various treatment modalities. The physician must be able to recognize residual tumor or recurrent tumor and select the most appropriate supplement treatment method doing so generally requires considerable training and experiences.
Rb has become a less life-threatening malignancy with the use of appropriate treatment modalities such as enucleation, EBR, local therapies, and systemic chemotherapy. The clinical use of neo-adjuvant systemic chemotherapy has dramatically changed the treatment strategies for Rb, avoiding EBR because of the risk of second malignancies and facial deformities. However, about half of the eyes with advanced Rb still have to be enucleated, and EBR is still one of the most important regimens of treatment.
New treatment modalities are expected to be employed clinically not only for eye preservation but also for a good visual prognosis.
The urgent demand of early and accurate diagnostic work up to confirm and solidify the diagnosis of Rb as well as to confirm that the tumor is confined to the eye, has already been noted after a discussion of the different clinical manifestations of the disease with its differential diagnosis.
The variety of growth patterns of Rb has been exhibited and its characteristics histopathologic features have been described.
New or more effective treatment alternatives with their advantages, indication, and possible complications have been presented and followed by new modalities under development in several laboratories, which may be available in the next decade.
Recent reports have documented a decreasing frequency of enucleation and an increasing tendency to use eye-preserving methods of management. However, enuclearion is still recommended in situations where eyes contain large tumors, long standing retinal detachment, neovascular glaucoma, pars plana tumor seeding, anterior chamber involvement, and if there is suspicion of invasion of optic nerve or choroid, or extrascleral extension. Most of these eyes either have or expected to have no useful vision.
After initial treatment, a follow up plan should be applied for each patient, depending on the clinical and pathological findings. The child should be examined under course of irradiation. The frequency of subsequent examinations depends on the response to previous treatment, and varies from patient to patient. A long term follow up of patients is important to detect and manage treatment complications, development of second primary tumors, tumor metastases, and pinealoblastoma.
In recent years, there have been a number of new developments related to the genetics and genetic counseling for patients with Rb.
The aim of this work was to report trends in management of Rb in ocular oncology clinic, ophthalmology department, Ain Shams University in the period from 2000-2010.
Medical records of Rb children were reviewed with extraction of patient’s age, sex, complaint, laterality, staging, modality of treatment used and outcome of therapy. All data was studied statistically.
It was found that 229.0 patients with Rb presented to our clinic. The mean age at the diagnosis was 26.5months in unilateral cases and 17.7 months in bilateral cases. No sex predilection of the disease. Girls affected in 49.3% of patients and boy affected in 50.7% of patients. Family history was positive in 8% of cases. Bilateral involvement was in 47.6% and unilateral affection was in 52.4%. leucocoria was the most common presenting symptoms 77.7% followed by strabismus in 11.4%.
Reese Ellsworth classification in bilateral cases was as follows; 7.5% of eyes were in group I, 25% were in group II, 8.5% were in group III, none was in group IV, 59.2% were in group V, extraocular disease was in 2.5% of eyes.
In unilateral cases, 26.1% were in group I, 20.6% were in group II, 6.4% were in group III, 5% were in group IV, 30.7% were in group V, extraocular disease was in 11% of eyes.
Chemoreduction ± focal therapy was used as a primary treatment modality in 52.3% of eyes in bilateral disease and 35% of eyes in unilateral disease.
EBR was used as a primary treatment modality in 21.5% of eyes with bilateral disease and in 20% of eyes in unilateral disease.
Enucleation was used as a primary treatment modality in 25.7% of eyes and 45.8% of eyes in bilateral and unilateral cases respectively.
69.7% of eyes in bilateral Rb didn’t receive secondary treatments compared to 57.5%of eyes in unilateral Rb.
62.4% of eyes in bilateral disease were preserved versus 34.2% of eyes in unilateral disease. The overall ocular preservation rate over the period of the study was 52.3% of eyes following different modalities of treatment.
So enucleation was the most common treatment modality in unilateral cases and the most common treatment modality in bilateral cases was chemoreduction ± focal therapy.
The 10 year survival rate in the present study was 98.3% which was similar to the results in developed countries.