Risk Factors for Metastases in Patents with Retinoblastoma

doi:10.1016/S0161-6420(91)32325-X

Ophthalmology

Volume 98, Issue 2, February 1991, Pages 136-141

https://doi.org/10.1016/S0161-6420(91)32325-X Get rights and content

Abstract

The study is based upon a review of data from 583 consecutive patients with retinoblastoma over the years 1956 to 1986. Mean follow-up was 8 years, and median was 5.5 years. In 41 patients, metastases developed within 5 years. The influence of clinical and histopathologic risk factors on the occurrence of metastases was first analyzed by univariate tests. Significant variables were then reevaluated using the Cox proportional hazards method. Four factors were found to be independently associated with the development of metastases: optic nerve invasion with and without involvement of the resection line, choroidal invasion and enucleation of an affected eye more than 120 days after initial diagnosis. The 5-year metastatic risks associated with these factors were 67%, 13%, 8%, and 4%, respectively. The relative risk estimate, calculated from the Cox model, was used for a score classification with groups of low, medium, and high metastatic risk. The 5-year incidence of metastases was 4%, 43%, and 68%, respectively.

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A retrospective nationwide analysis of retinoblastoma cases diagnosed between 2004-2016 using the Surveillance, Epidemiology, and End Results database was conducted. Overall survival, cause-specific survival, and all-cause mortality risk were assessed as primary outcomes.
A total of 393 retinoblastoma patients were included, of whom 268 (68.2%) had no choroidal invasion, 91 (23.2%) had focal choroidal invasion, and 34 (8.7%) had massive choroidal invasion on enucleation. A total of 6 deaths occurred throughout an average follow-up period of 72.2 ± 47.1 months: 4 deaths were cancer related. Adjusted Cox regression demonstrated higher all-cause mortality in patients with massive choroidal invasion (HR, 41.29; 95% CI, 4.05-420.49; P = 0.002) relative to those without choroidal invasion; however, those with focal choroidal invasion (HR, 2.69; 95% CI, 0.17-43.09; P = 0.484) demonstrated no difference in all-cause mortality. On further stratification by level of optic nerve invasion (ONI), all cancer-related deaths (4/4) were found to have occurred in patients with massive choroidal invasion and concomitant postlaminar ONI (PLONI). Patients with massive choroidal invasion without PLONI demonstrated 5-year overall and cause-specific survival of 100%, whereas patients with massive choroidal invasion and PLONI demonstrated 5-year overall and cause-specific survival of 80.2%.
All retinoblastoma-related deaths occurred in patients with both massive choroidal invasion and PLONI. These findings could not establish that massive choroidal invasion is an independent risk factor for poor outcome.
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To compare the efficacy of 2 chemotherapeutic drug combinations as part of multimodal therapy for orbital retinoblastoma.
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Demographic and clinical features were recorded at presentation. Treatment consisted of a multimodal protocol with neoadjuvant chemotherapy, enucleation, orbital external-beam radiotherapy, and adjuvant chemotherapy. For chemotherapy, patients were randomized into 2 groups: group A patients were treated with vincristine, etoposide, and carboplatin (VEC) and group B patients were treated with carboplatin and etoposide, alternating with cyclophosphamide, idarubicin, and vincristine. Treatment outcomes and adverse effects were recorded. Efficacy parameters were compared between the groups.
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A total of 54 children were recruited (27 in each group). The mean ± SD follow-up was 21.3±11.34 months. The overall Kaplan-Meier survival probability was 80% (95% confidence interval [CI], 0.67–0.89) and 42% (95% CI, 0.24–0.59) at 1 year and 4 years, respectively. There were 9 deaths in group A and 15 deaths in group B. The Kaplan-Meier survival probability at 1 year was similar between the groups: 81% (95% CI, 0.60–0.91) and 79% (95% CI, 0.58–0.9) for groups A and B, respectively. At 4 years, the survival probability for group A was higher (63% [95% CI, 0.41–0.79] vs. 25% [95% CI, 0.08–0.46] for groups A and B, respectively), with a strong trend of better survival in group A over time (P = 0.05). The major cause of death was central nervous system relapse (8 patients in group A and 7 patients in group B). Two patients in group B died of sepsis after febrile neutropenia. Grade 3 and grade 4 hematologic toxicities were more common in group B, with a significant difference in grade 4 neutropenia (P = 0.002).
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High-resolution magnetic resonance imaging can reliably detect orbital tumor recurrence after enucleation in children with retinoblastoma
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Citation Excerpt :
Altogether, we showed in this study that high-resolution MRI is a reliable tool to detect and exclude orbital tumor recurrence in children with retinoblastoma after enucleation. The presence of risk factors for metastases/local tumor recurrence, such as postlaminar optic nerve infiltration, massive choroidal invasion, and scleral invasion, in the histopathologic evaluation after enucleation increases the risk of recurrent or metastatic disease.11–15 In our patient group, at least 1 risk factor was present in all children with orbital tumor recurrence.
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High-resolution MRI using orbital surface coils was performed on 1.5 Tesla MRI systems to assess abnormal orbital findings.
Five European experts in retinoblastoma imaging evaluated the MRI examinations regarding the presence of abnormal orbital gadolinium enhancement and judged them as “definitive tumor,” “suspicious of tumor,” “postsurgical condition/scar formation,” or “without pathologic findings.” The findings were correlated to histopathology (if available), MRI, and clinical follow-up.
Abnormal orbital enhancement was a common finding after enucleation (100% in the first 3 months after enucleation, 64.3% >3 years after enucleation). All histopathologically confirmed tumor recurrences (3 of 55 orbits, 5.5%) were correctly judged as “definitive tumor” in MRI. Two orbits from 2 children rated as “suspicious of tumor” received intravenous chemotherapy without histopathologic confirmation; further follow-up (67 and 47 months) revealed no sign of tumor recurrence. In 90.2%, no tumor was suspected on MRI, which was clinically confirmed during follow-up (median follow-up after enucleation, 45 months; range, 8–126).
High-resolution MRI with orbital surface coils may reliably distinguish between common postsurgical contrast enhancement and orbital tumor recurrence, and therefore may be a useful tool to evaluate orbital tumor recurrence after enucleation in children with retinoblastoma. We recommend high-resolution MRI as a potential screening tool for the orbit in children with retinoblastoma to exclude tumor recurrence, especially in high-risk patients within the critical first 2 years after enucleation.
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^b: Drs. Messmer and Heinrich are currently affiliated with the University of Zürich, Switzerland.

^c: Dr. De Sutter is currently in private, practice in Kortrijk, Belgium.

View full text

Risk Factors for Metastases in Patents with Retinoblastoma

Abstract

Ophthalmology

Ophthalmology

Am J Ophthalmol

Ophthalmology

Ophthalmology

Am J Ophthalmol

Retinoblastoma, including a report of illustrative cases

Med Ann DC

Letale Verlaufe bei Retinoblastom. Retrospektive Studie uber 22 Patienten

Helv Paediatr Acta

Die Therapie des Retinoblastoms

Ber Dtsch Ophthalmol Ges

The prognosis of retinoblastoma in terms of survival: a computer assisted study. Part II

Ophthalmic Paediatr Genet

Intraocular retinoblastoma group V: an analysis of prognostic factors

J Clin Oncol

Prognosis in retinoblastoma: influence of histopathologic characteristics

J Pediatr Ophthalmol Strabismus

Prognostic importance of choroidal invasion in retinoblastoma

Arch Ophthalmol

Retinoblastoma: correlation of invasion of the optic nerve and choroid with prognosis and metastases

Br J Ophthalmol

Retinoblastoma: a study of natural history and prognosis of 268 cases

Br Med J

Investigation of retinoblastoma with special reference to histology and prognosis

Br J Ophthalmol

A histopathological study on the prognosis and radiosensitivity of retinoblastoma

Arch Ophthalmol.