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. 2017 Mar-Apr;1(2):165-172.
doi: 10.1016/j.oret.2016.09.009.

Proliferative Vitreoretinopathy in Treated Retinoblastoma

Cindy S Hwang  1 , Pia R Mendoza  1 , Jill R Wells  1 , Hans E Grossniklaus  1 , G Baker Hubbard 3rd  1
Affiliations

Proliferative Vitreoretinopathy in Treated Retinoblastoma

Cindy S Hwang et al. Ophthalmol Retina. 2017 Mar-Apr.

Abstract

Objective: To evaluate the clinical and histopathologic characteristics of patients who develop proliferative vitreoretinopathy after retinoblastoma treatment.

Design: Retrospective review of three cases of proliferative vitreoretinopathy (PVR) that developed after successful treatment of retinoblastoma from 2003 to 2015.

Subjects: Three patients with treated retinoblastoma who developed severe PVR and required enucleation.

Methods: Review of clinical charts, fundus drawings, Ret-Cam 3 images, and histopathology specimens.

Main outcome measures: Clinical and histopathologic characterization of PVR in treated retinoblastoma.

Results: Three patients developed severe PVR after sequential thermal laser combined with systemic chemotherapy for retinoblastoma. At presentation patients were 6, 7, and 9 months of age, and all had bilateral retinoblastoma. Time to development of proliferative tissue was 9, 12, and 20 months after initial treatment. Proliferation was characterized by progressive growth of white vascularized tissue with associated traction on the retina and sometimes hemorrhage. All patients underwent enucleation. Histopathologic evaluation revealed treated retinoblastoma tumor with a Type 3 regression pattern, pre- and subretinal fibrovascular tissue consistent with PVR, and reactive changes in the retinal pigment epithelium. None of the patients developed recurrence of retinoblastoma or systemic metastasis.

Conclusion: PVR uncommonly develops after successful treatment of retinoblastoma and may result in traction or rhegmatogenous retinal detachment along with vitreous hemorrhage. Early stages of proliferation may be difficult to distinguish from recurrent tumor. Enucleation may be required due to poor vision and inability to adequately monitor for tumor recurrence.

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Figures

Figure 1
Figure 1
Color fundus photo depicting progression of proliferative vitreoretinopathy and retinal detachment in patient 1
Figure 2
Figure 2
A. Gross examination of the enucleated eye shows a calcified mass (asterisk) with overlying white fibrous membrane (arrow); B. Histologic section demonstrating regressed retinoblastoma (asterisk), detached retina (arrow), and PVR (arrowhead); C. The PAS stain highlights the blood vessels within the fibrovascular PVR membrane; D. Higher magnification of the area of regressed tumor shows calcification and non-viable tumor cells consistent with Type 3 regression pattern. (Hematoxylin & eosin: B. 10×; Periodic acid Schiff: C. 25×; Hematoxylin & eosin: D. 25×)
Figure 3
Figure 3
Color fundus photos depicting progression of proliferative vitreoretinopathy over a four-year period in patient 2. This patient also developed a combined tractional-rhegmatogenous retinal detachment as a result of the fibrovascular proliferation.
Figure 4
Figure 4
A. Gross examination of the enucleated eye shows white fibrous membranes (arrow) overlying a mass with a combination of “cottage cheese-like” (asterisk) and translucent “fish flesh-like” (arrowhead) areas; B. Histologic section demonstrating regressed retinoblastoma (asterisk), detached retina (arrow), and PVR (arrowhead); (Hematoxylin & eosin: B. 10×)
Figure 5
Figure 5
Color fundus photos depicting progression of proliferative vitreoretinopathy over a five-month period in patient 3
Figure 6
Figure 6
A. Histologic section demonstrating regressed retinoblastoma (asterisk), detached retina (arrow), and PVR (arrowhead) extending to lens (L); B. Fibroglial tissue (arrowhead) adjacent to lens (L) and incorporating peripheral retina (arrow) (Hematoxylin & eosin: A. 5×; Periodic acid Schiff: B. 25×)
Figure 7
Figure 7
Fellow eyes in patient 1 (A), patient 2 (B), and patient 3 (C). Arrows indicate fibrosis in these eyes, even in cases of small tumor burden.
See this image and copyright information in PMC

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