Clinical spectrum of diffuse infiltrating retinoblastoma in 34 consecutive eyes.

Abstract:

PURPOSE:To evaluate the clinical features and management of diffuse infiltrating retinoblastoma. DESIGN:Retrospective case series. PARTICIPANTS:Thirty-four eyes in 32 patients. METHODS:The patients' records were reviewed for patient and tumor features, ocular management, histopathologic findings, and patient survival. MAIN OUTCOME MEASURES:Clinical features, tumor management, and patient survival. RESULTS:Of 1507 patients with retinoblastoma, only 32 (2%) were classified with diffuse infiltrating retinoblastoma. The mean age at diagnosis was 4 years (range 1.5-16 years). The referring diagnoses included retinoblastoma (26 eyes, 76%), uveitis (3, 9%), Coats disease (1, 3%), trauma (1, 3%), and unspecified retinal problem (3, 9%). The anterior segment displayed tumor seeds on the corneal endothelium (8, 24%), cornea stromal edema (3, 9%), pseudohypopyon (11, 32%), hyphema (3, 9%), iris neovascularization (17, 50%), and iris tumor nodules (6, 18%). The posterior segment revealed extensive ill-defined retinoblastoma infiltrating the retina for a mean basal diameter of 20 mm and overall flat growth, sometimes with undulating retinal thickening. Overlying extensive vitreous tumor seeds (31, 91%) and vitreous hemorrhage (8, 24%) were noted. Calcification was present on ultrasonography (27/34, 79%) and computed tomography (8/9, 89%). Enucleation was performed for all 34 eyes, and there were no cases of metastases at 47 months follow-up. CONCLUSIONS:Diffuse infiltrating retinoblastoma can masquerade as uveitis or unexplained hyphema or keratic precipitates. Suspicion for retinoblastoma is important. FINANCIAL DISCLOSURE(S):The authors have no proprietary or commercial interest in any materials discussed in this article.

journal_name

Ophthalmology

journal_title

Ophthalmology

authors

Shields CL,Ghassemi F,Tuncer S,Thangappan A,Shields JA

doi

10.1016/j.ophtha.2008.07.003

subject

Has Abstract

pub_date

2008-12-01 00:00:00

pages

2253-8

issue

12

eissn

0161-6420

issn

1549-4713

pii

S0161-6420(08)00700-8

journal_volume

115

pub_type

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