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Chapter-29 Pathology of High-RiskRetinoblastoma

BOOK TITLE: Retinoblastoma

Author
1. Barrios Patricia Chévez
ISBN
9789350257845
DOI
10.5005/jp/books/11629_29
Edition
1/e
Publishing Year
2012
Pages
8
Author Affiliations
1. University of Texas MD Anderson Cancer Center, 6565 Fannin St, M227, Houston, Texas, USA
Chapter keywords

Abstract

Children with retinoblastoma with tumors contained to the intraocular portion of the eye show more than 95% cure rate after enucleation and other treatment modalities. The probabilities of metastasis increase proportional to the amount of tumor as well as the closeness of the tumor to the extraocular orbital tissues and the intracranial portion of the optic nerve. The incidence of high-risk histopathologic features varies from 7-9% invasion into the anterior segment, 12-42% invasion found in the choroid, 8-12% tumor invasion into sclera, 2-20% invasion into the extrascleral tissues, 6-28% tumor invasion in the postlaminar optic nerve and 1-38% for involvement of the optic nerve to surgical margin, with the higher numbers representing the recently published study from India. Adequate evaluation of histopathologic features is essential in the treatment of patients with retinoblastoma. The successful evaluation of histopathologic features starts with the adequate handling of the eye immediately after enucleation, after fixation and at the time of histologic sectioning. The entire eye can be submitted for evaluation in four blocks: central PO section, two calottes in segments and the cross section of margin of optic nerve. Correct interpretation of histopathologic findings and objective measurement of tumor invading the choroid and optic nerve allow for comparison among different studies and in multicenter trials. The leading high-risk features in children with retinoblastoma are extrascleral extension, postlaminar optic nerve invasion at the level of the cut margin and massive choroidal invasion of more than 3 mm.

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