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Stages of Retinoblastoma
In order to properly diagnose retinoblastoma, there must be guidelines to follow to properly classify the risk of the tumor. The Reese Ellsworth Classification System, by Dr. Algernon Reese and Dr. Robert Ellsworth, is universally used to determine the size, location, and multi-focality of the tumor. The system was originally used to decide the best treatment result by using external beam radiotherapy, as well as, the likeliness of salvaging the globe of the eye. Due to chemotherapy not being part of the Reese Ellsworth Classification System, there needed to be an updated classification system to foresee the treatment outcomes of chemotherapy. The International Classification for Intraocular Retinoblastoma is now the current system being used, and it was created by Murphree and associates. According to Reese and Ellsworth, there were different groups that had various features in order to classify the globe salvage as very favorable to the category of very unfavorable. In order to salvage the affected eye, the disc diameter had to be around four and behind the equator to have higher favorability. If the tumor was around ten in disc diameter and involved roughly 50% of the retina, it was considered unfavorable to salvage the globe which could result in enucleation. According to Murphree, the different groups were classified from very low risk to very high risk which was determined by features of the given tumor. Very low risk means that the tumor has to be less than 3mm and there must be no seeding of the vitreous or sub-retinal area. When a patient is very high risk, the tumor presents itself with multiple features and is going to have to be treated with conservative treatment modalities or enucleation. International Classification for Intraocular Retinoblastoma

Signs and Symptoms
Retinoblastoma is universally known as the most intrusive intraocular cancer among children. The chance of survival and preservation of the eye depends fully on the severity. Retinoblastoma is extremely rare as there are only about 200 to 300 cases every year in the United States. Looking at retinoblastoma globally, only 1 in about 15,000 children have this malignancy but these numbers continuously increase. Intraocular malignancies are more curable rather than extraocular malignancies due to early diagnosis and an early treatment prognosis. Leucocoria is the primary indication of retinoblastoma and is when the cancer is still intraocular, meaning inside the eye. When light is reflected by the dangerous white tumor, the view of the red retina is blocked. Retinoblastoma can be curable after the initial sign and up to six months, if the tumor is intraocular. If you do not visit an ophthalmologist with signs of leucocoria within a reasonable amount of time, the delay in the diagnosis could lead to a more severe prognosis. Due to a delay in the diagnosis, it could result in proptosis which is then considered extraocular, the most severe.

Conclusion
Retinoblastoma is a very rare intraocular malignant tumor that needs to have more attention when exploring prevention. During infant screenings, if they incorporate an eye screening like they do a hearing screening, we may be able to detect it at an earlier age, therefore, preventing its spread. Regardless if retinoblastoma is unilateral, bilateral, sporadic, or hereditary, there are a variety of treatments available to eradicate or reduce its severity. There are some support groups available for those patients and families with retinoblastoma; however more ongoing support is necessary. Emotional support plays a key factor in improving one’s outcome and quality of life. There are worldwide networks that are dedicated to those affected by retinoblastoma; one suggestion might be to develop a retinoblastoma registry that provides patient and familial support.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5998397/