Bilateral Retinoblastoma: Management and Impact on the Lives of Survivors

Introduction: Retinoblastoma is a malignant tumor of neuroepithelial origin occurring in infants and young children. Despite its rarity, it is the most common intraocular malignancy in children. It is unilateral in 2/3 of cases and bilateral in 1/3, and may represent a genetic predisposition to cancer. RB survivors are at risk of disease- and treatment-related adverse effects, including loss of vision or one eye, second cancer, genetic predisposition to cancer and reduced quality of life. The aim of our study is to investigate the management Bilateral Retinoblastoma and its impact on the lives of survivors. Method: We report in this retrospective study of 15 cases collected at the Department of Hematology and Pediatric Oncology at CHU Mohammed VI in Marrakech over a 12-year period, from January 2012 to June 2O24. Result: The mean age was 1 year and 5 months with a slight male predominance (60%). No patients had a family history of retinoblastoma. Consanguinity was present in 26%. The most frequent reasons for consultation were: leukocoria (86.6%), strabismus (33.3%) and exophthalmos (13.33%). The majority of patients (80%) were seen within 6 to 14 months, with an average delay of 9 months. Diagnosis was based on ophthalmoscopic examination, ultrasound and CT scan data, while histological examination of the surgical specimen enabled confirmation of the diagnosis and prognostic classification. Optic nerve invasion was found in 3 cases, and choroidal involvement in 3 patients. Therapeutically, enucleation was required in most cases, given the advanced stage of the tumours (86.66%): enucleation of the right eye in 4 patients, enucleation of the left eye in 2 patients, and bilateral enucleation in 8 patients, given the advanced stage of the tumours. Neoadjuvant chemotherapy was indicated in 46.6% of cases, to facilitate enucleation. Eleven 11 patients (73.3%) received post-operative chemotherapy, 3 patients received unilateral conservative treatment (thermotherapy, cryotherapy and intravitreal chemotherapy) and 1 patient received bilateral conservative treatment (radiotherapy). In our series, 11 patients achieved complete remission, two died of sev

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Journal
Journal of Diagnosis & case Reports
Published
2024-12-31
DOI
https://doi.org/10.47363/jdcrs/2024(5)152
Primary Topic
Ocular Oncology and Treatments
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article
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article

Bilateral Retinoblastoma: Management and Impact on the Lives of Survivors

Banane NK, F Saadi, J Hanafi, El Houdzi
Journal of Diagnosis & case Reports
Ocular Oncology and Treatments
article

Bilateral Retinoblastoma: Management and Impact on the Lives of Survivors

Banane NK, F Saadi, J Hanafi, El Houdzi
article en

Abstract

Introduction: Retinoblastoma is a malignant tumor of neuroepithelial origin occurring in infants and young children. Despite its rarity, it is the most common intraocular malignancy in children. It is unilateral in 2/3 of cases and bilateral in 1/3, and may represent a genetic predisposition to cancer. RB survivors are at risk of disease- and treatment-related adverse effects, including loss of vision or one eye, second cancer, genetic predisposition to cancer and reduced quality of life. The aim of our study is to investigate the management Bilateral Retinoblastoma and its impact on the lives of survivors. Method: We report in this retrospective study of 15 cases collected at the Department of Hematology and Pediatric Oncology at CHU Mohammed VI in Marrakech over a 12-year period, from January 2012 to June 2O24. Result: The mean age was 1 year and 5 months with a slight male predominance (60%). No patients had a family history of retinoblastoma. Consanguinity was present in 26%. The most frequent reasons for consultation were: leukocoria (86.6%), strabismus (33.3%) and exophthalmos (13.33%). The majority of patients (80%) were seen within 6 to 14 months, with an average delay of 9 months. Diagnosis was based on ophthalmoscopic examination, ultrasound and CT scan data, while histological examination of the surgical specimen enabled confirmation of the diagnosis and prognostic classification. Optic nerve invasion was found in 3 cases, and choroidal involvement in 3 patients. Therapeutically, enucleation was required in most cases, given the advanced stage of the tumours (86.66%): enucleation of the right eye in 4 patients, enucleation of the left eye in 2 patients, and bilateral enucleation in 8 patients, given the advanced stage of the tumours. Neoadjuvant chemotherapy was indicated in 46.6% of cases, to facilitate enucleation. Eleven 11 patients (73.3%) received post-operative chemotherapy, 3 patients received unilateral conservative treatment (thermotherapy, cryotherapy and intravitreal chemotherapy) and 1 patient received bilateral conservative treatment (radiotherapy). In our series, 11 patients achieved complete remission, two died of sev

Journal of Diagnosis & case Reports
Centre Hospitalier Universitaire Mohammed VI (MA)
Openalex Percentile: Top 60%
Ocular Oncology and Treatments
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