High Risk Retinoblastoma: Prevalence and Success of Treatment in Developing Countries

Elzomor, Hossam; Taha, Hala; Aleieldin, Adel; Nour, Radwa; Zaghloul, Mohamed S.; Fawzi, Mary C. Smith; Kamel, Amr; Alfaar, Ahmad Samir

doi:10.3109/13816810.2015.1016241

Cited by 12 publications

(7 citation statements)

References 29 publications

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“…Guided by the definitions of the Children’s Oncology Group (COG), high-risk features were defined as massive invasions of the choroid or of the optic nerve beyond the lamina cribrosa or any degree of combined optic nerve and choroid invasion [ 6 ]. Vincristine–etoposide–carboplatin (VEC) were used as both neoadjuvant and adjuvant regimens of chemotherapy [ 7 ]. Resistance to chemotherapy, radiation and progression of the disease was an indication for enucleation.…”

Section: Methodsmentioning

confidence: 99%

Unilateral retinoblastoma; natural history and an age-based protocol in 248 patients

Zomor

Nour

Saad

et al. 2020

Eye

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Objectives We aimed to study the clinical state and prognosis of patients with unilateral retinoblastoma who were being treated at a paediatric comprehensive cancer centre in a limited-resource country, to assess the different phases of treatment and the success of different, more complex real-life models. Subjects In this retrospective study, we created a snapshot of our retinoblastoma database for the period between 2007 and 2015. Patients whose data were included in the study were followed up until 2016. Out of a total of 744 screened patients, we included data of 248 patients who had been diagnosed with unilateral retinoblastoma. Results As classified as per the International Retinoblastoma Classification, 1 patient presented with group A, 21 with group B, 39 with group C, 104 with group D and 83 with group E retinoblastoma. Chemotherapy was the initial line of treatment in 115 patients and enucleation in 133 others. Later, 141 patients (56.9%) required further management. Patients had a mean ocular survival time of 20.8 months. Nine patients developed extraocular disease at a later stage of management: five after upfront enucleation and four after neoadjuvant chemotherapy. Mean overall survival time stood at 90.2 months. Four and three deaths were recorded in groups D and E, respectively. A single patient died in the initial chemotherapy arm, while six passed away in the initial enucleation arm. Conclusion Our study highlights the importance of initial chemotherapy and close follow-up after enucleation of classes D and E affected eyes even in absence of germline mutations.

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Section: Methodsmentioning

confidence: 99%

Unilateral retinoblastoma; natural history and an age-based protocol in 248 patients

Zomor

Nour

Saad

et al. 2020

Eye

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“…13 There have also been efforts to demonstrate an association between high-risk histopathologic features of enucleated eyes and orbital relapse, although a consensus is lacking. 19–21 In a study of 1674 consecutive patients who underwent enucleation for RB, orbital tumor recurrence was noted in 71 cases (4%). Of these 71 cases, all except 11 subjects had evidence of high-risk histopathology features.…”

Section: Discussionmentioning

confidence: 99%

Orbital relapse of retinoblastoma in patients with high-risk histopathology features

Cicinelli

Kaliki

2019

Ophthalmol Eye Dis

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Aim: To describe the clinical picture of orbital relapse of retinoblastoma following uncomplicated enucleation. Methods: Case series of two patients with group E retinoblastoma with high-risk features on histopathology, namely, post-laminar optic nerve extension in one patient, and massive choroidal tumor infiltration in the other. Neither of them received adjuvant chemotherapy post enucleation. Results: Both patients had orbital relapse of tumor within 4 months from enucleation, manifesting as implant migration and unstable conformer. Systemic chemotherapy and external beam radiotherapy to the orbit resulted in complete tumor regression. Both patients were tumor free at the last follow up. Conclusion: Implant migration post enucleation should raise the suspicion for orbital relapse of retinoblastoma. High-risk histopathology features should increase the alert in an otherwise uncomplicated enucleation for retinoblastoma.

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“…Retinoblastoma is a rare form of retina malignancy but is the most common cancer of the eye in children, which is around 1 in 20 000 in live births,22 and it is almost exclusively found in young children. Therefore, instead of simple operations such as eye salvage, elucidating the underlying genetic mechanisms of retinoblastoma and its progression is of utmost importance.…”

Section: The Role Of Lncrnas In Various Types Of Visual Impairmentmentioning

confidence: 99%

Precise long non-coding RNA modulation in visual maintenance and impairment

Wan

Zhuo

2016

J Med Genet

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Long non-coding RNAs (lncRNAs) are remarkably powerful, flexible and pervasive cellular regulators. With the help of cheaper RNA-seq, high-throughput screening of lncRNAs has become widely applied and has identified large numbers of specific lncRNAs in various physiological or pathological processes. Vision is known to be a complex and vital perception that comprises 80% of the sensory information we receive. A consensus has been reached that normal visual maintenance and impairment are primarily driven by gene regulation. Recently, it has become understood that lncRNAs are key regulators in most biological processes, including cell proliferation, apoptosis, differentiation, immune responses, oxidative stress and inflammation. Our review is intended to provide insight towards a comprehensive view of the precise modulation of lncRNAs in visual maintenance and impairment. We also highlight the challenges and future directions in conducting lncRNA studies, particularly in patients whose lncRNAs may hold expanded promise for diagnostic, prognostic and therapeutic applications.

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High Risk Retinoblastoma: Prevalence and Success of Treatment in Developing Countries

Cited by 12 publications

References 29 publications

Unilateral retinoblastoma; natural history and an age-based protocol in 248 patients

Unilateral retinoblastoma; natural history and an age-based protocol in 248 patients

Orbital relapse of retinoblastoma in patients with high-risk histopathology features

Precise long non-coding RNA modulation in visual maintenance and impairment

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