Retinoblastoma Survival Following Primary Enucleation by AJCC Staging

Zhao, Junyang; Feng, Zhaoxun; Leung, Gareth; Gallie, Brenda L.

doi:10.3390/cancers13246240

Cited by 7 publications

(5 citation statements)

References 34 publications

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“…Primary enucleation followed by adjuvant chemotherapy will improve survival, reducing the occurrence of metastasis in children with locally advanced retinoblastoma and histopathologic high-risk characteristics. [29][30][31][32][33] This patient will be given adjuvant chemotherapy with a regimen of vincristine, etoposide, and carboplatin by a pediatric oncologist. Systemic chemotherapy for retinoblastoma is chemo reduction of large tumors (neoadjuvant chemotherapy) or reduces the risk of relapse, metastasis, or recurrence after surgical enucleation (adjuvant chemotherapy).…”

Section: Resultsmentioning

confidence: 99%

Enucleation for retinoblastoma in a 22-month-old boy

Ningtyas,

Irawan

2023

J. Teknol. Laboratorium

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Retinoblastoma is the commonest intraocular tumor in childhood. Management of retinoblastoma is quite complex depending on the stage, visual and globe saving, psychosocial factors, modality of treatment, and health infrastructure. We report a case of retinoblastoma in a child in a limited health facility without a pediatric oncologist or ophthalmology oncologist. A 22-month old boy came with complaints of leukocoria which his parents had known since the previous week. Ultrasonography of the eye shows calcification on the retina that supports Group E retinoblastoma. Bone marrow aspiration and MRI cannot be performed due to limited resources. Even though the parents only noticed the symptoms for a week and immediately brought to the ophthalmologist, the retinoblastoma stage was already in Group E. Enucleation and globe salvage were unavoidable. Enucleation was performed on the patient with histopathology finding the presence of Flexner-Wintersteiner rosette cells. After enucleation, he was referred to the oncologist for adjuvant chemotherapy. Early retinoblastoma detection is crucial in increasing the cure rate while maintaining children's vision.

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Section: Resultsmentioning

confidence: 99%

Enucleation for retinoblastoma in a 22-month-old boy

Ningtyas,

Irawan

2023

J. Teknol. Laboratorium

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“…In our study, the median lag time was longer (12mo) when compared to previous studies which were 7 and 9mo [33][34] . Longer lag time and advanced stage have also been mentioned as predictors for poor prognosis in retinoblastoma [35][36][37] . Thus, the longer lag time might be attributable to the distinct result between this study and previous study.…”

Section: Discussionmentioning

confidence: 99%

Lag time, high-risk histopathological features, metastasis, and survival interrelation in retinoblastoma: a perspective from lower-middle income country

Utomo¹,

Ardianto²,

Rinonce³

et al. 2022

Int J Ophthalmol

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AIM: To investigate the impact of lag time to metastasis and survival rates among patients with retinoblastoma. METHODS: This retrospective study was conducted with 52 patients from the Department of Ophthalmology and the Department of Pediatrics of Dr. Sardjito General Hospital, between 1st January 2014 and 31st December 2020. Lag time was defined as the time delay between the first sign of retinoblastoma to the diagnosis of retinoblastoma. The subjects with lag time > one year were included in the case group, while the subjects with lag time < one year were included in the control group. RESULTS: The lag time was significantly correlated with American Joint Committee on Cancer and Intraocular Classification of Retinoblastoma staging of retinoblastoma (P=0.005 and P=0.006, respectively). The lag time was also significantly correlated with both metastasis event [odds ratio (OR): 5.06, 95%Cl: 1.56-16.44, P=0.006] and mortality (OR: 4.54, 95%Cl: 1.37-15.07, P=0.011). The follow-up was continued for 32 subjects for 3y after initial diagnoses. Survival analysis revealed a significant difference among these two groups (P=0.021). Furthermore, lag time was significantly correlated with survival of retinoblastoma (r=-0.53, P=0.046). CONCLUSION: The study highlights the importance of lag time between the onset of first symptoms and the time of retinoblastoma diagnosis which significantly contribute to metastasis and mortality of patients with retinoblastoma. Examinations for the early detection of retinoblastoma should be performed for individuals at-risk to minimize lag time and improve the outcomes.

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“…Primary enucleation was recommended in some unilateral IIRC group D eyes, and for all IIRC group E eyes [11]. In cases of parents' refusal, systemic chemotherapy followed by focal treatment was proposed as an alternative treatment.…”

Section: Treatment Modalitiesmentioning

confidence: 99%

The Impact of Tumor Laterality (Unilateral vs. Bilateral) on Presentation and Management Outcome in Patients with Retinoblastoma

Mohammad,

Mehyar,

Halalsheh

et al. 2024

JCM

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Background: This study compares the outcomes of managing retinoblastoma between patients with unilateral and bilateral presentations. Methods: The study, conducted at the King Hussein Cancer Center in Amman, Jordan, retrospectively analyzed cases of retinoblastoma treated between March 2003 and December 2019. Evaluation criteria included clinical features, disease stage, treatment methods, and overall management outcomes. Results: The study comprised 697 eyes from 478 patients with retinoblastoma, with 52% being males. Bilateral disease was observed in 70% of patients, and a family history of retinoblastoma was more prevalent in cases with bilateral disease (20%) compared to those with unilateral disease (4%). Unilateral cases had a median age at diagnosis of 28 months, whereas bilateral cases were diagnosed at a median age of 6 months. Extra-ocular retinoblastoma was detected in 1% of eyes. According to the International Intraocular Retinoblastoma Classification (IIRC), 88% of unilateral cases presented with advanced disease (IIRC group D/E), compared to 46% in bilateral cases. Primary enucleation was performed in 29% of unilateral cases and 16% of bilateral cases (p-value 0.0007). Eye salvage rates were 31% in unilateral cases and 68% in bilateral cases (p-value < 0.0001). At 120 months of follow-up, 5% of patients died from secondary neoplasms or metastases, 81% were alive, and 14% were lost to follow-up. There was no significant difference in metastasis, secondary neoplasms, or mortality between patients with unilateral and bilateral retinoblastoma. Conclusions: This study highlights the nuanced differences in clinical characteristics and outcomes between unilateral and bilateral retinoblastoma, emphasizing the necessity of customized management and early detection strategies. It demonstrates that while bilateral retinoblastoma benefits from earlier detection and has a higher rate of eye salvage, there is no significant difference in metastasis or mortality rates when compared to unilateral cases. The critical roles of primary enucleation in advanced cases, along with effective communication and patient education, are also underscored to improve treatment adherence. Overall, these findings point to the importance of tailored approaches in optimizing outcomes for the diverse patient population affected by retinoblastoma.

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Retinoblastoma Survival Following Primary Enucleation by AJCC Staging

Cited by 7 publications

References 34 publications

Enucleation for retinoblastoma in a 22-month-old boy

Enucleation for retinoblastoma in a 22-month-old boy

Lag time, high-risk histopathological features, metastasis, and survival interrelation in retinoblastoma: a perspective from lower-middle income country

The Impact of Tumor Laterality (Unilateral vs. Bilateral) on Presentation and Management Outcome in Patients with Retinoblastoma

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