OBJECTIVE: To collect and present updated evidence about epidemiological aspects of retinoblastoma (Rb) in the world.
METHODS: A comprehensive search without the time and language restrictions was conducted in international databases, including MEDLINE, Scopus, Web of Science, and PubMed. The search keywords were \"retinoblastoma\" OR \"retinal Neuroblastoma\" OR \"retinal glioma\" OR \"retinoblastoma eye cancer\" OR \"retinal glioblastoma\".
RESULTS: The worldwide incidence of Rb is 1 in 16 000-28 000 live births, but was higher in developing compared to developed countries. Several attempts for improving early detection and treatment had increased the Rb survival rate from 5% to 90% in developed countries over the past decade, but its survival was lower in developing countries (about 40% in low-income countries) and the majority of mortalities occurred in developing countries. The etiology of Rb could be viewed as genetics in the heritable form and environmental and lifestyle factors in the sporadic form. Some environmental risk factors such as in vitro fertilization; insect sprays; father\'s occupational exposure to oil mists in metal working, and poor living conditions might play a role in the occurrence of the disease. Although ethnicity might affect Rb incidence, sex has no documented effect and the best treatment approaches were now ophthalmic artery chemosurgery and intravitreal chemotherapy.
CONCLUSIONS: Determining the role of genetics and environmental factors helps to accurately predict the prognosis and identify the mechanism of the disease, which can reduce the risk of tumor development.

UNASSIGNED: Retinoblastoma is the most common primary intraocular malignancy of childhood. The present study was undertaken to overcome the scarcity of data regarding the epidemiology, demographic and clinical profile, and nature of the first health-care professional consulted.
UNASSIGNED: A retrospective analysis of medical records was performed for all cases of retinoblastoma who presented to the department of ophthalmology and oncology between 2010 and 2017. A total of 54 cases were reviewed.
UNASSIGNED: Unilateral disease was seen in 85.2% while 14.8% presented with bilateral disease with a median age of 3 years and 2 years, respectively, at diagnosis. The male: female ratio was 2:1. The most common presenting symptom was leukocoria which was noticed in 42 patients (77.7%), followed by red eye (33.3%) and proptosis (20.3%). The most common stage of presentation was Stage I (44.4%), followed by Stage IV (20.4%), Stage III (9.3%), and Stage II (5.6%). The median time to diagnosis was 8.7 months (range, 0.5-98.7 months), and the median time to treatment was 37.4 days (range, 0-645 days). Majority of the patients were referred by local ophthalmologists (48%), followed by general practitioners (20%), nurses (19%), and quacks (13%). Enucleation was the most preferred treatment modality (48.1%), followed by chemotherapy (33.3%), radiotherapy (13%), photocoagulation (3.7%), and exenteration (1.9%). The overall remission rate was 79.6%, while 12.3% had relapse and 7.4% died.
UNASSIGNED: The study showed a dire need for timely detection and treatment of retinoblastoma which is possible with improved awareness and better accessibility to health-care facilities. It also revealed a decrease in histopathological risk factors with chemoreduction.

Retinoblastoma (Rb), the most frequent malignant intraocular tumor in childhood, is caused by mutations in the retinoblastoma gene (RB1) situated on chromosome 13q14.2. The incidence of retinoblastoma is approximately 1 in 17,000 live births with approximately 8,000 new cases diagnosed each year worldwide. Rb is the prototypical hereditary cancer in humans. Autosomal dominant inheritance is seen in 30-40% of cases whereas the non-inherited sporadic type accounts for the remaining 60-70%. Rb arises due to inactivation of both alleles of the Rb tumor suppressor gene, which results in a defective Rb protein (pRB) with subsequent cell cycle impairment and uncontrolled cell proliferation. Patients with Rb have survival rates higher than 95-98% in industrialized countries but mortality remains high in developing countries. For example, the mortality rate in Africa is 70%. In all cases of intraocular and extraocular retinoblastoma, there is a need for new therapies that are more effective and carry less risk of toxicity. The Bruckner test is a practical and easy test for the detection of Rb, this test consists of assessing the fundus reflex through the pupil (red reflex) in both eyes simultaneously with a bright coaxial light produced with the direct ophthalmoscope. Rb can be detected by the Bruckner test showing a pupil that shines white or \"Leukocoria\". Although the diagnosis of Rb remains essentially clinical, the newly identified biomarkers could contribute to early molecular detection, timely detection of micrometastases and establish new therapeutic options for Rb.

Coats disease may cause diagnostic dilemma because of its variable clinical presentation that can be suspicious of retinoblastoma. Late sequelae of the disease are blinding with possible enucleation. We demonstrate the main histopathological findings of Coats enucleated eyes with literature review.
This was a retrospective study of all enucleated globes diagnosed as Coats disease over 30 years and were reviewed by two pathologists. The corresponding demographic data, clinical presentation, pre-operative clinical impression, and indication for enucleation were collected. Descriptive analysis of our own series data was performed. Our findings were then correlated to published data that were collected from 1983 to 2021 from the PUBMED database in English-written language. Shields classification was used as an inclusion criterion for the published reports to be analyzed.
We had seven enucleated globes with Coats disease. Mean age at presentation was 3.2 years (range 3 months to 9 years). Male predominance was observed in 6 and all cases were unilateral. Strabismus was the most common initial presentation (57%, n = 4), followed by leukocoria (43%, n = 3). Indication for enucleation was mostly suspected retinoblastoma (57%, n = 4). Four eyes were classified as stage 4, and 2 had advanced stage 5 changes. Histopathologically, subretinal fluid with lipid-laden macrophages was seen in all cases, the anterior chamber was shallow in 5/7 with angle neovascularization in 2/7. Telangiectatic vessels were clearly observed in 4/7.
Coats disease is a potentially visually disabling disease that is mostly unilateral in 95%, has male predominance of 81%, and wide age range with a mean of 17 years. In Saudi Arabia, the disease seems to present at younger age, tends to be more advanced, and may be indistinguishable from retinoblastoma at the time of diagnosis. Shields staging of Coats is highly recommended to be followed clinically to unify the pathways for treatment and to correlate the concluded outcomes.

Bilateral persistent hyperplastic primary vitreous (PHPV) is a rare ocular disorder. Its clinical manifestations include bilateral corneal haziness, microphthalmia, and cataract. It is the second most common cause of leukocoria after retinoblastoma. Most cases of PHPV are unilateral. The typical imaging features of PHPV comprise bilateral echogenic masses and a fibrous cord extending from the posterior surface of the lens to the optic disc. In this report, we present a case of bilateral PHPV in an infant who presented with bilateral corneal haziness and watery discharge. A detailed ocular examination and knowledge about its features on imaging can lead to a timely and accurate diagnosis of the condition.

We present a case of a 9 years old boy, followed up for 4 years, with bilateral combined pigmented epithelial and retinal hamartoma, complicated with recurrent vitreous hemorrhages in one eye and neovascular glaucoma and cataract in the other eye, treated with repeated intravitreal injections of Bevacizumab. A review of the literature suggested that such lesions may be symptomatic because of decreased vision, macular pucker, strabismus and vitreous hemorrhages. This particular compressive, bilateral form of hamartoma of the optic nerve has not previously been reported as a cause for such an ischemic syndrome, complicated with neovascular glaucoma and cataract.

OBJECTIVE: To analyze the demography, presenting clinical features, spread of the disease of retinoblastoma in patients who were treated in two tertiary hospitals in Malaysia.
METHODS: In this retrospective study, information of gender, age, race, presenting clinical features, findings of CT scan orbits and brain, lumbar puncture and bone marrow aspiration results were collected from the medical records of retinoblastoma patients diagnosed in Hospital of Universiti Sains Malaysia and General Hospital of Kuala Lumpur over a period of ten years. The data were collected in the same type of proforma from both hospitals.
RESULTS: A total of 64 patients were treated in both hospitals together, of whom boys and girls were almost equally affected. The mean age of children at presentation was 24.2 (range 3-84) months, 53 (82.8%) children were under 36 months old. The disease was unilateral in 39 (60.9%) patients. The most common presenting signs were leukocoria in 46 (71.8%), followed by proptosis in 21 (32.8%) patients. Routine screening of the siblings of affected children revealed retinoblastoma in 2 patients. There was intracranial extension in 8 (12.5%) and metastasis in bone marrow in 6 (9.3%) patients.
CONCLUSIONS: Leukocoria is the most common presentation of retinoblastoma in Malaysia. However, the disease was intraocular in 40 (62.5%) and extraocular (orbital involvement, intracranial and distant metastasis) in 24 (37.5%) patients.