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Histopathologic Features and Prognostic Factors

  • Patricia Chévez-BarriosEmail author
  • Ralph C. Eagle
  • Eduardo F. Marback
Chapter

Abstract

Retinoblastoma is a tumor that arises from the neuroblastic cells that comprise the nuclear layers of the retina. Grossly, the tumor is classified by its pattern of growth into endophytic, exophytic, mixed, diffuse infiltrative, and necrotic variants. The characteristic histopathologic findings in retinoblastoma include a tumor that replaces the retina with medium-sized cells that have a high nuclear/cytoplasmic ratio, marked apoptotic and mitotic activity, and foci of necrosis with calcification. If left untreated, retinoblastoma usually fills the eye and completely destroys the internal architecture of the globe. The tumor tends to spread locally by invading the optic nerve and choroid, and then hematogenously, and by lymphatics once it reaches the extraocular structures such as the conjunctiva and eyelids. Multivariate statistical analysis has suggested the correlation of histopathologic findings such as the presence of tumor in the optic nerve posterior to the lamina cribrosa, tumor at the site of surgical transection, and extrascleral extension of tumor into the orbit with risk of metastasis. Other factors associated with probable risk for metastatic behavior, especially in conjunction with the major factors cited above, are massive choroidal invasion, tumor invasion into the anterior chamber, large tumor size with vitreous seeding, neovascularization of the iris, and glaucoma. It is important to analyze the eye appropriately to evaluate the possible high-risk features. Guidelines for handling the eye have been published as a result of a consensus from the International Retinoblastoma Staging Working Group that also includes criteria used in the prospective clinical trial from the Children’s Oncology Group (ARET0332 A Study of Unilateral Retinoblastoma With and Without Histopathologic High-Risk Features and the Role of Adjuvant Chemotherapy: A Groupwide Phase III Study). Animal models, and histopathological and collaborative clinical trials, will certainly facilitate the understanding of these factors and ultimately allow the use of targeted therapies to prevent metastasis and death from retinoblastoma.​

Keywords

High-risk features Histopathology Optic nerve invasion Choroidal invasion Prognostic Factors Vitreous seeds 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Patricia Chévez-Barrios
    • 1
    • 2
    Email author
  • Ralph C. Eagle
    • 3
  • Eduardo F. Marback
    • 4
  1. 1.Departments of Pathology and Genomic Medicine and OphthalmologyWeill Cornell Medical CollegeNew YorkUSA
  2. 2.Houston Methodist HospitalHoustonUSA
  3. 3.Department of PathologyWills Eye Hospital Thomas Jefferson UniversityPhiladelphiaUSA
  4. 4.Department of OphthalmologyFederal University of BahiaRio Vermelho, SalvadorBrazil

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