title: High-Risk Histopathology Features in Primary and Secondary Enucleated International Intraocular Retinoblastoma Classification Group D Eyes.
abstract: To evaluate the rate and identify the risk factors for high-risk histopathologic features in group D retinoblastoma eyes enucleated as primary or secondary treatment.
  
  
  
  Retrospective analysis.
  
  
  
  A total of 64 enucleated group D eyes (62 patients), of which 40 (40 patients) were primary and 24 (22 patients) were secondary to other treatments.
  
  
  
  Clinicopathologic correlation of consecutive group D eyes enucleated from 2002 to 2014. High-risk histopathologic features were defined as the presence of anterior chamber seeds, iris infiltration, ciliary body/muscle infiltration, massive (≥3 mm) choroidal invasion, retrolaminar optic nerve invasion, or combined non-massive choroidal and prelaminar/laminar optic nerve invasion.
  
  
  
  High-risk histopathologic features, metastasis, and death.
  
  
  
  Of the 64 group D eyes, 37 (58%) were classified as cT2bN0M0H0, 24 (38%) were classified as cT2bN0M0H1, and 3 (5%) were classified as cT2aN0M0H1, according to the 8th edition cTNMH Retinoblastoma Staging. High-risk histopathologic features were detected in 10 eyes (16%) in the entire cohort, 5 eyes (13%) of the primary enucleated group (pT3aNxM0, n = 2 and pT3bNxM0, n = 3, 8th edition pTNM), and 5 eyes (21%) of the secondary enucleated group (pT2bNxM0, n = 2, pT3aNxM0, n = 2 and pT3cNxM0, n = 1). Absence of vitreous seeds at presentation was the only predictive factor found for high-risk histopathologic features in the primary enucleation group (P = 0.042), whereas none were found in the secondary group (P ≥ 0.179). Invasion of the anterior structures (anterior chamber, iris, ciliary body/muscle) was detected significantly more after secondary enucleation (P = 0.048). All patients with high-risk histopathologic features were treated with adjuvant chemotherapy, and no metastases were recorded in a median follow-up time of 73.2 months (mean, 71.5; range, 13.7-153.0).
  
  
  
  The choice of primary treatment for group D retinoblastoma should be carefully weighed, because according to this study, 13% of eyes harbor high-risk histopathologic features at presentation, with the absence of vitreous seeds being a potential risk factor. It is of special importance in group D eyes being considered for nonsystemic treatment, such as primary intraophthalmic artery chemotherapy. Secondary enucleated group D eyes with high-risk histopathologic features more commonly involved anterior structures, warranting meticulous clinical and histologic examinations for this subset of patients.
authors.affiliation: Ocular Oncology Service, Moorfields Eye Hospital, London, United Kingdom; Retinoblastoma Service, Royal London Hospital, London, United Kingdom. Electronic address: didifabian@gmail.com.
authors.forename: Ido D
authors.initials: ID
authors.lastname: Fabian
authors.affiliation: Ocular Oncology Service, Moorfields Eye Hospital, London, United Kingdom; Retinoblastoma Service, Royal London Hospital, London, United Kingdom; Department of Ophthalmology, University of Washington, Seattle, Washington.
authors.forename: Andrew W
authors.initials: AW
authors.lastname: Stacey
authors.affiliation: Retinoblastoma Service, Royal London Hospital, London, United Kingdom; Paediatric Oncology Department, Great Ormond Street Hospital, London, United Kingdom.
authors.forename: Tanzina
authors.initials: T
authors.lastname: Chowdhury
authors.affiliation: Retinoblastoma Service, Royal London Hospital, London, United Kingdom; Paediatric Oncology Department, Great Ormond Street Hospital, London, United Kingdom.
authors.forename: Catriona
authors.initials: C
authors.lastname: Duncan
authors.affiliation: Department of Cellular Pathology, Barts Health NHS Trust, London, United Kingdom.
authors.forename: Esin K
authors.initials: EK
authors.lastname: Karaa
authors.affiliation: Department of Cellular Pathology, Barts Health NHS Trust, London, United Kingdom.
authors.forename: Irene
authors.initials: I
authors.lastname: Scheimberg
authors.affiliation: Retinoblastoma Service, Royal London Hospital, London, United Kingdom.
authors.forename: M Ashwin
authors.initials: MA
authors.lastname: Reddy
authors.affiliation: Ocular Oncology Service, Moorfields Eye Hospital, London, United Kingdom; Retinoblastoma Service, Royal London Hospital, London, United Kingdom; National Institute for Health Research Biomedical Research Centre at Moorfields Eye Hospital NHS Foundation Trust and University College London, Institute of Ophthalmology, London, United Kingdom.
authors.forename: Mandeep S
authors.initials: MS
authors.lastname: Sagoo
mesh_terms: D002648:Child; D002675:Child, Preschool; D015353:Eye Enucleation; D005260:Female; D006801:Humans; D007223:Infant; D038801:International Classification of Diseases; D008297:Male; D019572:Retinal Neoplasms / Q000145:classification* / Q000473:pathology* / Q000601:surgery; D012175:Retinoblastoma / Q000145:classification* / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D012307:Risk Factors