Impact of Systemic Chemotherapy and Delayed Enucleation on Survival of Children with Advanced Intraocular Retinoblastoma (original) (raw)
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Chemotherapy in Retinoblastoma
International Journal of Therapies and Rehabilitation Research, 2016
Retinoblastoma is an uncommon intraocular tumor of childhood.The survival of retinoblastoma patients has improved in the developed world but is still a major concern in the developing countries.The goal of retinoblastoma treatment is to preserve vision without compromising long-term survival and minimizing side effects. Retinoblastoma is curable when diagnosed early and treated. Several treatment options are available. Chemotherapy has become a promising method to cure not only the recurrence but also primary tumors. The aim of this article is to review the literature on different chemotherapeutic modalities available for the treatment of retinoblastoma and present an update. The review included the published research studies on this paediatric malignancy to date in indexed journals.This review has tried to give information regarding one of the many treatment modalities in retinoblastoma,its benefits and adverse effects with the aim of keeping the physician/ophthalmologist updated about this malignancy which still poses a great danger in the developing worlds.
Chemotherapy in Retinoblastoma: Current Approaches
Türk Oftalmoloji Dergisi, 2015
Retinoblastoma (RB) is the most common childhood malignant intraocular tumor. Although enucleation and external beam radiotherapy have been historically used, today the most commonly used eye-sparing approach is chemotherapy. Chemotherapy can be used in both intraocular and extraocular RB cases. Chemotherapeutic agents may be applied in different ways, including systemic, subconjunctival, intra-arterial and intravitreal routes. The main purposes of application of systemic therapy are to reduce the tumor size for local treatment (chemoreduction), or to reduce the risk of metastasis after enucleation surgery (adjuvant therapy). Intra-arterial chemotherapy with or as a secondary method in tumor recurrence. The most important advantage of intra-arterial therapy is the prevention of systemic chemotherapy complications. Intravitreal chemotherapy is administered in the presence of persistent or recurrent vitreous seeding. The term "extraocular RB" includes orbital invasion and metastatic disease. Current treatment for orbital invasion is neoadjuvant chemotherapy followed by surgical enucleation and adjuvant chemotherapy and radiotherapy after surgery. In metastatic disease, regional lymph node involvement, distant metastases, and/or central nervous system (CNS) involvement may occur. Among them, CNS involvement has the worst prognosis, remaining at almost 100% mortality. In metastatic disease, high-dose salvage chemotherapy and autologous hematopoietic stem cell rescue therapy are the possible treatment options; radiotherapy could also be added to the protocol according to the side of involvement.
Retinoblastoma : Recognise the Disease early and save a child's life
Retinoblastoma is the most common malignant intraocular tumor of childhood, with an incidence of 1 in 16,000 to 18,000 live births. The most common symptoms of retinoblastoma are leukocoria and strabismus. The diagnosis of retinoblastoma is delayed in the developing countries when compared to that of developed countries. The management of retinoblastoma has dramatically changed over the years from previous radiotherapy methods to current chemotherapy strategies. The treatment of choice in less advanced cases is chemotherapy and in more advanced cases is enucleation. With the improved treatment strategies, patient survival, globe salvage, and vision salvage in patients with retinoblastoma has drastically improved over the years. The aim of this article is to discuss the presenting features, treatment strategies, and prognosis of retinoblastoma.
Pediatric blood & cancer, 2016
To evaluate a chemoreduction regimen using systemic vincristine and carboplatin (VC) and local ophthalmic therapies to avoid external-beam radiotherapy (EBRT) or enucleation in patients with Group B intraocular retinoblastoma. Twenty-one patients (25 eyes) were treated with six cycles of VC, accompanied by local ophthalmic therapies after cycle 1. The primary study objective was to determine the 2-year event-free survival (EFS) where an event was defined as the use of systemic chemotherapy in addition to vincristine or carboplatin, EBRT, and/or enucleation. All patients had tumor regression after the first cycle of VC and only two patients had progression during therapy. There were seven treatment failures within 2 years of study enrollment, resulting in 2-year EFS of 65% and early study closure in accordance with the statistical design. The 2-year cumulative incidence of enucleation was 15%; for external beam radiation therapy, it was 10%; and for chemotherapy to control progressiv...
South Asian Journal of Cancer, 2022
Background Retinoblastoma (RB) is the most common primary intraocular malignancy in children. We sought to provide a comprehensive assessment of epidemiological profile and treatment outcomes of children with RB. Methods In this retrospective study, we analyzed 189 children diagnosed with RB at our center between 2004 and 2017. Survival was analyzed with the Kaplan–Meier method and log-rank test. Results Median age at presentation was 14 months with male: female ratio 1.2:1. Mean duration between onset of symptoms and presentation was 49 days (standard deviation ± 79). Most common presenting symptom was white pupillary reflex in 60% of children. Family history of RB and other cancers was found in one (0.5%) and seven (4%) children, respectively. Primary mode of diagnosis and staging was ocular ultrasonography (bone scan) in 87% of patients. Computed tomographic scan and magnetic resonance imaging were done in 124 (66%) and 30 (16%) patients, respectively. International staging syste...
Intravitreal chemotherapy in the management of retinoblastoma in a resource-limited setting
African Vision and Eye Health, 2022
Intravitreal chemotherapy (IVitC) in the management of retinoblastoma has increased the rate of globe salvage, specifically in patients with recurrent disease and associated vitreous seeds. A significant number of children with retinoblastoma in developing countries present late, resulting in higher intraocular tumour-stage at presentation. Treatment requirements for such tumours usually include intravenous chemotherapy (IVC) and/or intra-arterial chemotherapy (IAC). While IVC has a long usage track record and a good efficacy, it has been reported to be associated with higher recurrence rates in a significant number of patients. Intra-arterial chemotherapy has the advantage of lower recurrence rates but requires personnel with advanced interventional radiology skills and has limited efficacy in treating intravitreal seeds. Intravitreal chemotherapy has gained popularity recently, largely because of its superior efficacy in the management of vitreous seeds, subretinal seeds and recur...
Journal of Clinical Oncology, 2011
Initial response of intraocular retinoblastoma to chemotherapy has encouraged primary chemotherapy instead of primary enucleation for eyes with clinical features suggesting high risk of extraocular extension or metastasis. Upfront enucleation of such high-risk eyes allows pathologic evaluation of extraocular extension, key to management with appropriate surveillance and adjuvant therapy. Does chemotherapy before enucleation mask histologic features of extraocular extension, potentially endangering the child's life by subsequent undertreatment? Methods We performed retrospective analysis of 100 eyes with advanced retinoblastoma enucleated with, or without, primary chemotherapy, in Beijing Tongren Hospital, retrospectively, from October 31, 2008. The extent of retinoblastoma invasion into optic nerve, uvea, and anterior chamber on histopathology was staged by pTNM classification. The treatment groups were compared for pathologic stage (Cochran-Armitage trend test) and disease-specific mortality (competing risks methods). Results Children who received chemotherapy before enucleation had lower pTNM stage than primarily enucleated children (P ϭ .01). Five patients who received pre-enucleation chemotherapy died as a result of extension into brain or metastasis. No patients who had primary enucleation died. For children with group E eyes, disease-specific survival (DSS) was lower with pre-enucleation chemotherapy (n ϭ 45) than with primary enucleation (n ϭ 37; P ϭ .01). Enucleation longer than 3 months after diagnosis was also associated with lower DSS (P Ͻ .001). Conclusion Chemotherapy before enucleation of group E eyes with advanced retinoblastoma downstaged pathologic evidence of extraocular extension, and increased the risk of metastatic death from reduced surveillance and inappropriate management of high-risk disease, if enucleation was performed longer than 3 months after diagnosis.
Asia-Pacific Journal of Ophthalmology, 2021
Purpose: To evaluate retinoblastoma control after intravenous chemotherapy (chemoreduction) by patient age at presentation. Design: Retrospective case series. Methods: This study included 964 eyes of 554 patients treated with chemoreduction at Ocular Oncology Service at Wills Eye Hospital. Patients received 6 monthly cycles of standard chemoreduction. Additional therapies for tumor control were performed as needed. Results: Of 964 eyes, a comparison by age group (<6 months vs. 6-12 months vs. 13-24 months vs. >24 months) revealed more advanced age group with higher frequency of group E tumor (15% vs. 25% vs. 32% vs. 39%, P < 0.001). By treatment outcomes, complete tumor control was achieved with chemoreduction alone more often in less advanced age group (46% vs. 30% vs. 17% vs. 8%, P < 0.001). Additional treatment after chemoreduction was needed more often in more advanced age group with external beam radiotherapy (EBRT; 9% vs. 16% vs. 20% vs. 15%, P ¼ 0.006) or enucleation (12% vs. 18% vs. 26% vs. 37%, P < 0.001).