Risk of metastasis and orbital recurrence in advanced retinoblastoma eyes treated with systemic chemoreduction versus primary enucleation

Berry, Jesse L.; Kogachi, Kaitlin; Aziz, Hassan A.; McGovern, Kathleen R.; Zolfaghari, Emily; Murphree, A. Linn; Jubran, Rima; Kim, Jonathan W.

doi:10.1002/pbc.26270

Cited by 36 publications

(38 citation statements)

References 35 publications

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“…The relatively higher but nonsignificant rates of death and metastasis in the group receiving chemoreduction are largely due to the fact that these patients had advanced disease to begin with, which per se increases the risk of metastasis and death. The results of our study are in accordance with the results of Berry et al [35] that globe salvage therapy with systemic chemoreduction and subsequent enucleation for poor response does not increase the risk of metastatic disease or orbital recurrence. Larger future prospective trials will shed more light on this issue before guidelines are laid down.…”

Section: Discussionsupporting

confidence: 83%

Retinoblastoma: A Sixteen-Year Review of the Presentation, Treatment, and Outcome from a Tertiary Care Institute in Northern India

Singh¹,

Katoch²,

Kaur³

et al. 2017

Ocul Oncol Pathol

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Purpose: To study epidemiology, demographic profile, clinical characteristics, and outcome in pediatric patients with retinoblastoma. Methods: This was a retrospective review of retinoblastoma patients of a tertiary institute from January 1st 1998 to December 31st 2014. Results: The study included 467 patients (618 eyes) with a mean age of 34.7 ± 24.6 months (median = 30; 15 days to 144 months). Retinoblastoma was bilateral in 151 (32.3%) and there were 61.7% males. Intraocular disease was seen in 301 patients (451 eyes [72.9%]) and extraocular in 166 patients (167 eyes; 27.0%). Out of the 347 (74.3%) who received treatment, primary treatment was chemoreduction in 228 (65.7%) and enucleation in 117 (33.7%), while 25.6% of patients refused treatment and 151 (43.5%) defaulted therapy. Local recurrence was seen in 20 (4.3%), metastasis in 2 (0.4%), and deaths in 13 (2.8%) (average follow-up 28.5 ± 44.4 months). Histopathological high risk features were significantly less in the eyes that received chemoreduction (5.0%) versus primary enucleation (20.8%) (p < 0.0004), but there was no difference in the rate of metastasis, recurrence, and death between the two. Conclusions: The majority of retinoblastoma patients in our study had advanced disease, and nearly a third had extraocular extension. There were a significant number of therapy refusals and dropouts. Chemoreduction led to a significant decrease in the histopathological risk factors without affecting the outcomes.

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Section: Discussionsupporting

confidence: 83%

Retinoblastoma: A Sixteen-Year Review of the Presentation, Treatment, and Outcome from a Tertiary Care Institute in Northern India

Singh¹,

Katoch²,

Kaur³

et al. 2017

Ocul Oncol Pathol

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“…The literature remains very controversial about histopathological risk factors in retinoblastoma and the benefit of neoadjuvant chemotherapy before enucleation . However, extrascleral and ON resection margin involvement are widely accepted high‐risk factors.…”

Section: Discussionmentioning

confidence: 99%

“…The literature remains very controversial about histopathological risk factors in retinoblastoma and the benefit of neoadjuvant chemotherapy before enucleation. 20,[22][23][24][25][26][27][28][29][30][31][32] However, extrascleral and ON resection margin involvement are widely accepted high-risk factors. ON resection margin involvement is associated with a mortality rate between 50% and 81%.…”

Section: Discussionmentioning

confidence: 99%

Management of advanced uni‐ or bilateral retinoblastoma with macroscopic optic nerve invasion

Choucair

Brisse

Fréneaux

et al. 2019

Pediatric Blood & Cancer

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Background Retinoblastoma with macroscopic optic nerve (ON) invasion depicted by imaging at diagnosis remains a major problem and carries a poor prognosis. We sought to describe the treatment and outcome of these high‐risk patients. Methods Retrospective mono‐institutional clinical, radiological, and histological review of patients with uni‐ or bilateral retinoblastoma with obvious ON invasion, defined by radiological optic nerve enlargement (RONE) depicted by computed tomography scan or magnetic resonance imaging (MRI), was performed. Results Between 1997 and 2014, among the 936 patients with retinoblastoma treated at Institut Curie, 11 had detectable RONE. Retinoblastoma was unilateral in 10 and bilateral in one. Median age at diagnosis was 28 months (range, 11‐96). ON enlargement extended to the orbital portion in three patients, to the optic canal in five, to the prechiasmatic portion in two, and to the optic chiasm in one. Nine patients received neoadjuvant chemotherapy and partial response was obtained in all. Enucleation was performed in 10/11 patients—by an anterior approach in three and by anterior and subfrontal approaches in seven. Three patients had a positive ON resection margin (2/3 after primary enucleation). All enucleated patients received adjuvant treatment (conventional chemotherapy: 10, high‐dose chemotherapy: seven, radiotherapy: five). Leptomeningeal progression occurred in four patients. Seven are in first complete remission (median follow up: 8 years [3.5‐19.4]). Conclusion Neoadjuvant chemotherapy and microscopic complete resection have a pivotal role in the management of retinoblastoma with RONE. MRI is recommended for initial and pre‐operative accurate staging. Surgery should be performed by neurosurgeons in case of posterior nerve invasion. Radiotherapy is required in case of incomplete resection.

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“…However, the validity of loss of fundus view as an indication for enucleation has not been previously analyzed. Rates of high‐risk histopathologic features after secondary enucleation range from 0 to 21% and indicate either persistent or progressive disease, with the latter category indicating a lack of sufficient monitoring in some cases. Both the rate of active disease and high‐risk histopathology in eyes enucleated with loss of fundus view would encourage a short period of monitoring before enucleation.…”

Section: Introductionmentioning

confidence: 99%

Loss of fundus view as an indication for secondary enucleation in retinoblastoma

Berry

Kogachi

Jubran

et al. 2017

Pediatric Blood & Cancer

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In this retrospective study, we evaluated loss of fundus view as an indication for secondary enucleation and associated histopathologic findings. Of 64 secondarily enucleated eyes, 24 were enucleated for loss of fundus view. Average time from loss of fundus view to enucleation was 4.7 months. Of these eyes, 22 had viable tumor cells on histopathology, but none had high-risk features. Loss of fundus view was a common indication for secondary enucleation after chemoreduction. Given the high prevalence of viable histopathologic tumor cells, enucleation for loss of fundus view should not be significantly delayed to decrease risk of high-risk tumor progression.

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Risk of metastasis and orbital recurrence in advanced retinoblastoma eyes treated with systemic chemoreduction versus primary enucleation

Cited by 36 publications

References 35 publications

Retinoblastoma: A Sixteen-Year Review of the Presentation, Treatment, and Outcome from a Tertiary Care Institute in Northern India

Retinoblastoma: A Sixteen-Year Review of the Presentation, Treatment, and Outcome from a Tertiary Care Institute in Northern India

Management of advanced uni‐ or bilateral retinoblastoma with macroscopic optic nerve invasion

Loss of fundus view as an indication for secondary enucleation in retinoblastoma

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