Correlation between clinical presentations, radiological findings and high risk histopathological features of primary enucleated eyes with advanced retinoblastoma at Queen Sirikit National Institute of Child Health: 5 years result

Vayani

et al. 2023

Preprint

Purpose To retrospectively analyse globe salvage rates in group D retinoblastoma (RB) with currently available treatment options in Pakistan over a period of nine years. Methods A retrospective, cross-sectional analysis including all patients who presented with Group D RB according to the International Classification of Retinoblastoma (ICRB), from April 2013 to December 2022 at Patel Hospital, Karachi, Pakistan. Indirect ophthalmoscopy done under anaesthesia was used to visualise and evaluate tumour characteristics and clinical response to the treatment offered. Globe salvage was attempted with systemic chemotherapy, local consolidative therapy along with intravitreal melphalan. Survival analysis was performed using Kaplan-Meier curves on SPSS version 26. Results The mean age at diagnosis was 17.2 months.14 (93%) patients had bilateral disease and one (7%) had unilateral disease. Globe salvage was achieved in 11 out of 15 eyes (73.33%) in a mean follow up time of 57.5 weeks (range 7–263 weeks). All eyes received laser therapy, 5 required additional cryotherapy and 1 eye was given adjuvant intravitreal melphalan. 4 eyes underwent secondary enucleation. All eyes amongst this group received systemic chemotherapy, laser, cryotherapy and 3 eyes received intravitreal melphalan (4 cycles). Kaplan-Meier survival analysis showed an overall globe salvage rate of 93%, 76%, and 65% at 1, 2 and 3 years, respectively. No metastasis or deaths were reported. Conclusion Intravenous chemotherapy (IVC) coupled with local therapy remains an effective mode of treatment in select patients with Group D RB despite advancements in treatment modalities like intra-arterial chemotherapy. This is especially promising for developing countries with limited resources and socioeconomic barriers.

Section: Discussionmentioning

confidence: 89%

Clinical outcomes of Group D Retinoblastoma at a tertiary care hospital in Pakistan

Vayani

et al. 2023

Preprint

“…The formation of retinoblastoma begins from a photoreceptor cell of the retina that is related to the mutation of the RB1 gene on chromosome 13q14; all people receive a copy of this gene from the mother and father (8,9). This pathology is considered rare, since for every 16,000 to 18,000 newborns worldwide, one will get retinoblastoma, with a prevalence of 15% of pediatric cancers (10,11); on the other hand, it is estimated that more than half of retinoblastoma cases come from Asia-Pacific and almost a quarter from Africa (12,13). In Korea they report an incidence of 11.2 cases per 100,000 inhabitants, in the United States and Europe they have similar records (14).…”

Section: Ntroductionmentioning

confidence: 99%

Retinoblastoma management: a systematic review

Polo¹,

Anaya²,

Ortiz³

2023

Braz. J. Hea. Rev.

Retinoblastoma is a rare cancer that occurs in children under 5 years of age from which leukocoria and strabismus are the characteristic signs of this disease. For every 16,000 to 18,000 newborns worldwide, one will get retinoblastoma, while at the Latin American context there are no reported data due to scant research. Therefore, the reason for this research is to describe the management of retinoblastoma. Trauma, human papillomavirus (HPV), and genetic alterations in the RB1 gene are possible causative factors for retinoblastoma. The diagnosis is based on the clinic, genetic tests, imaging tests and in cases of metastasis, a cerebrospinal fluid (CSF) aspiration is performed through lumbar puncture. There is another method such as non-invasive prenatal diagnosis (NIPD), here the main diagnosis is indirect ophthalmoscopy. The treatment is directly related to the evolution of the disease using different methods such as enucleation, cryotherapy, chemotherapy and radiotherapy that can be used individually or in combination to improve the quality of life of the patient; however, enucleation followed by chemotherapy remain as the main method of treatment.

“…One of the advantages of MRI compared to ultrasound and fundoscopy is that the complete orbit can be evaluated. This allows for a more accurate screening for extrascleral extension, optic nerve invasion and inflammatory processes [11,15,[27][28][29][30][82][83][84][85][86][87][88][89][90][91][92][93][94][95]. MRI generally outperforms ultrasound in screening for extrascleral extension (Figure 3C) [27][28][29][30].…”

Section: Anatomical Evaluationmentioning

confidence: 99%

Magnetic Resonance Imaging in the Clinical Care for Uveal Melanoma Patients—A Systematic Review from an Ophthalmic Perspective

Jaarsma-Coes

Klaassen

Marinković

et al. 2023

Cancers

Conversely to most tumour types, magnetic resonance imaging (MRI) was rarely used for eye tumours. As recent technical advances have increased ocular MRI’s diagnostic value, various clinical applications have been proposed. This systematic review provides an overview of the current status of MRI in the clinical care of uveal melanoma (UM) patients, the most common eye tumour in adults. In total, 158 articles were included. Two- and three-dimensional anatomical scans and functional scans, which assess the tumour micro-biology, can be obtained in routine clinical setting. The radiological characteristics of the most common intra-ocular masses have been described extensively, enabling MRI to contribute to diagnoses. Additionally, MRI’s ability to non-invasively probe the tissue’s biological properties enables early detection of therapy response and potentially differentiates between high- and low-risk UM. MRI-based tumour dimensions are generally in agreement with conventional ultrasound (median absolute difference 0.5 mm), but MRI is considered more accurate in a subgroup of anteriorly located tumours. Although multiple studies propose that MRI’s 3D tumour visualisation can improve therapy planning, an evaluation of its clinical benefit is lacking. In conclusion, MRI is a complementary imaging modality for UM of which the clinical benefit has been shown by multiple studies.