Retinoblastoma Presentation and Survival: A four-year analysis from a tertiary care hospital

Abstract

Objective: To study the clinical presentation, treatment, and outcome of Retinoblastoma (Rb) in a tertiary care hospital of Pakistan.
 Methods: A retrospective study was conducted in the Department of Pediatric Hematology Oncology, The Indus Hospital (TIH), Karachi from 1st June 2013 to 30th June 2017. Data including patients’ demography, clinical symptoms and duration, laterality, extent of the tumor, type of treatment, relapse, and final outcome were extracted and evaluated with respect to progression and survival.
 Results: A total of 93 patients were included; 34.4% were boys. The median age at presentation was 30 months. Leukocoria was the commonest symptom (61.3%), followed by proptosis (37.6%). Unilateral disease was seen in 59.1%, extraocular tumors in 43.5% and metastasis in 28.1%. Enucleation was performed on 46.2%, chemotherapy given to 80.6% and external beam radiation therapy to 29.3% patients.
 Conclusion: Delayed presentation, recurrent disease, extraocular disease and metastasis on presentation were factors affecting outcome in our cohort. Awareness about the early warning signs and symptoms in both public and health professionals for early recognition and timely management are mandatory to decrease morbidity and mortality.
 doi: https://doi.org/10.12669/pjms.36.ICON-Suppl.1720
 How to cite this:Zia N, Hamid A, Iftikhar S, Qadri MH, Jangda A, Khan MR. Retinoblastoma Presentation and Survival: A four-year analysis from a tertiary care hospital. Pak J Med Sci. Special Supplement ICON 2020. 2020;36(1):S61-S66. doi: https://doi.org/10.12669/pjms.36.ICON-Suppl.1720
 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Retinoblastoma in South Asia: A Scoping Review

“…Detection of mutation in the RB1 gene among Pakistanis was cited as a promising tool for early detection [5]. Although leukocoria remained the most common presenting symptom, proptosis which is indicative of advanced disease was also common [7,9]. I intraocular Group E, extraocular extension or metastatic disease was common at initial presentation and was associated with delay in consultation [7,9].…”

“…Although leukocoria remained the most common presenting symptom, proptosis which is indicative of advanced disease was also common [7,9]. I intraocular Group E, extraocular extension or metastatic disease was common at initial presentation and was associated with delay in consultation [7,9]. Delay in presentation had been attributed to lack of knowledge on the severity of the disease and lack of access to proper and affordable eye care [7].…”

“…The mean age at consult ranged from 24 to 42 months. Median delay in consultation from a series was 4 months [9]. Both genders were equally affected.…”

“…Only four articles had data on treatment and outcomes [3,[7][8][9]. Parents of 45 patients refused treatment.…”

microRNA-144 functions as a diagnostic and prognostic marker for retinoblastoma

“…According to previous studies, RB shows rapid growth, a high metastatic rate, and poor prognosis (1)(2)(3). With the development of modern technology, the survival rate of RB has increased to over 50% (4,5). However, long-term prognosis of RB patients is still unfavorable, and among children who survive, some lose their vision (1).…”