Retinoblastoma in older patients: A retrospective comparative analysis of 100 consecutive patients based on age

Abstract

PurposeTo describe comparative clinical features, treatment, and outcomes of retinoblastoma in patients initially diagnosed at age 4 or older.MethodsRetrospective case series.ResultsThere were 101 eyes in 100 consecutive patients age ≥4 years diagnosed with retinoblastoma. Mean patient age at diagnosis was 6.6 years (median 5.3, range 4.0–41.0 years). Tumors were predominantly classified (International Classification of Retinoblastoma) as group D (31%) or E (65%). Patients were divided by age into 3 groups: young (4–6 years [65%]), middle (>6–8 years [23%]), and older (>8 years [12%]). Comparing by age group (young vs. middle vs. older), mean tumor basal diameter (19.9 vs. 17.3 vs. 17.0 mm, p = 0.05) and mean tumor thickness (11.0 vs. 9.4 vs. 7.0 mm, p < 0.01) were greatest in the youngest group. Distance to the optic nerve (1.5 vs. 1.7 vs. 5.0 mm, p = 0.01) and foveola (1.9 vs. 1.8 vs. 6.0 mm, p < 0.01) were greatest in the oldest age group. Objective findings of leukocoria and strabismus were more common in younger patients, while older patients complained of subjective findings, like decreased vision (19% vs. 30% vs. 60%, p < 0.01) and floaters (3% vs. 4% vs. 17%, p = 0.05). Primary treatment included enucleation (76%) and other modalities (24%). Globe salvage rate was 13%, with no significant difference by age. Comparison of globe salvage by revealed significant improvement between 1974–2008 (6%) and 2009–2017 (38%, p < 0.01).ConclusionRetinoblastoma in older patients (>8 years) tends to be smaller and more peripherally located, with more subjective presenting symptoms.

Retinoblastoma in an Older Child: A Case Report

“…8 The pathophysiology for late onset retinoblastoma is postulated to be the malignant transformation of benign retinoma (retinocytoma) or RB1 mutation in persistent embryonic retinal cells. 9 The presence of clinical features unusual to that of younger children and delay in suspicion and referral due to its rare occurrence in that particular age group further leads to delay in diagnosis and treatment. 2 Various studies have shown atypical clinical features including decreased vision, floaters, masquerade syndrome, vitreous hemorrhage as more common in older retinoblastoma cases compared to the stereotype description of leucocoria and strabismus in younger children.…”

Late onset retinoblastoma in a 12-year-old girl: a case report

“…also presented two patients with RB who were initially misdiagnosed as OT and underwent a series of unnecessary procedures. 17 As RB in older children has been reported to occur between 3.5-8.5%, RB should be considered in the differential diagnosis of a fundal mass in any age group. 17-21 Shields et al reviewed 400 cases of RB and found that 8.5% of patients were older than 5 years old and 0.8% of patients were older than 15 years old at the time of the diagnosis.…”

“…Moreover, it is located more peripherally from the optic disc and foveola and it has smaller diameter than RB of younger patients. 17 It is of grater staging and symptoms appear to be more subjective (e.g. reduced visual acuity, floaters) rather than objective (e.g.…”

Intra-Arterial Chemotherapy: New Hope for Adult Retinoblastoma Treatment—A Case Report and Brief Review

“…Moreover, RB in older patients mostly presents with unusual symptoms and signs (e.g., floater or decreased vision). As a result, the unusual appearance triggers a late diagnosis and these cases are usually diagnosed clinically in advanced stages [10].…”