A change roadmap towards research paradigm in low-resource countries: retinoblastoma model in Egypt.

Research on childhood diseases represents a great global challenge. This challenge is maximized in both childhood cancer disciplines and developing world. In this paper, we aim at describing our institution experience in starting a structured childhood cancer research program in one of the developing countries in a short time based on philanthropic efforts. We used retinoblastoma as an example for what was conducted in this program. Starting in 2008, this program included improving clinical practice and its related supporting services besides developing new research services that both complement the clinical activities and pave the way towards creating a research foundation in the country. Results included developing hospital standard treatment protocols, developing national clinical trials, joining international consortia for childhood cancers clinical trials, developing data collection tools and real-time analytics, establishing a biobanking facility, and developing highly qualified team for conducting clinical, epidemiologic, and translational research studies. Moreover, this effort resulted in improving both clinical practice and patients' awareness nationally. This model can be used for other startup facilities that aim at finding answers for their national health problems in low-resource setting.

Phthisis bulbi: clinical and pathologic findings in retinoblastoma.

PURPOSE: Phthisis bulbi represents an ocular end-stage disease characterized by shrinkage and disorganization of the eye. We aim at identifying the pathologic changes of phthisis bulbi associated with retinoblastoma. DESIGN: Retrospective observational case series study. METHODS: 16 enucleated eyes were enrolled retrospectively between 2007 and 2012. Pathologic gross and microscopic findings were assessed. RESULTS: Cases showed grossly shrunken eyes with a mean volume of 4.3 cc. Sclera was markedly thickened in the majority of cases with mean of 2272.8 µ. Choroid showed an average thickness of 1029 µ. Necrosis, dystrophic calcification, ossification, gliosis, residual viable tumor was identified in many cases. Pathologic high risk factors were seen in three specimens. CONCLUSIONS: We conclude that retinoblastoma must be considered in the differential diagnosis of phthisis bulbi in the pediatric patients and active tumor was present in half of the patients.

Clinical presentation of intraocular retinoblastoma; 5-year hospital-based registry in Egypt.

PURPOSE: To study the presenting signs of Retinoblastoma in Egypt at Egypt's main pediatric oncology referral center. METHODS: This is a prospective descriptive study (hospital-based registry) conducted at Children's Cancer Hospital Egypt between July 2007 and December 2012. RESULTS: Out of 262 patients diagnosed with retinoblastoma, 244 were suffering from intra-ocular disease at presentation. One hundred thirty-nine (57%) patients presented with unilateral disease, while 105 (43%) suffered bilateral disease. The mean age at presentation was 20.6 ± 17 months, averaging 18.87 ± 11.76 months for bilateral and 25.72 ± 18.78 months for unilateral disease. The most common clinical presentation was leukocoria in 180 (73.8%) patients, strabismus in 32 (13.1%) patients and decreased visual acuity in 12 (4.9%) patients. Group D and E disease represented 62% of all affected eyes. Patients with advanced disease (Group C-E) had longer duration of symptoms. CONCLUSION: In Egypt, retinoblastoma patients present more frequently with advanced disease. There is an ever-increasing need to develop a national team dedicated to studying disease significance and formulating a national awareness program.