Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2011 Apr;25(2):159-67.
doi: 10.1016/j.sjopt.2010.10.010. Epub 2010 Oct 13.

Orbital retinoblastoma: Present status and future challenges - A review

Mohammad J Ali  1 , Santosh G Honavar, Vijay A P Reddy
Affiliations

Orbital retinoblastoma: Present status and future challenges - A review

Mohammad J Ali et al. Saudi J Ophthalmol. 2011 Apr.

Abstract

Orbital retinoblastoma is a catastrophic event traditionally carrying a dismal prognosis. Although its incidence is less in the developed countries it continues to be one of the major diagnosis at presentation in the developing world. Orbital retinoblastoma encompasses a wide range of distinct clinical entities with varying tumor load. There are no standard treatment protocols as of now but the current preferred management is multimodal with a combination of initial high-dose chemotherapy, surgery, external beam radiotherapy and prolonged chemotherapy for twelve cycles. In spite of progress on all fronts including surgical, medical, diagnostic, genetic and rehabilitative with improving survival rates, however, lack of access to medical facilities, lack of education about the need for early medical attention and cultural resistance to enucleation continue to contribute to an epidemic of extra ocular disease at diagnosis in the developing world. This review introduces the various terminologies used in the spectrum of orbital retinoblastoma, discusses in details the clinical aspects and management protocols, current status and the future directions.

Keywords: Chemotherapy; Extra ocular; Orbit; Proptosis; Radiotherapy; Retinoblastoma.

PubMed Disclaimer

Figures

Figure 1
Figure 1
(a and b). Bilateral advance retinoblastoma. Left eye gross proptosis and right eye anterior segment seeding can be appreciated. (a) CT Scan axial cuts of the same patient confirmed orbital mass and gross optic nerve extension (b).
Figure 2
Figure 2
Primary orbital retinoblastoma. The right eye shows proptosis with extra scleral extension in the form of nodular masses.
Figure 3
Figure 3
Fungating orbital retinoblastoma.
Figure 4
Figure 4
Histopathology specimen of an enucleated eye demonstrating extra scleral extension of the tumor.
Figure 5
Figure 5
(a and b). CT Scan axial cuts show gross left optic nerve thickening of the tumor measured radiologically (a) CT Scan axial cuts show gross left optic nerve extension along with intracranial extension (b).
Figure 6
Figure 6
(a and b). Bilateral phthisical eyes following chemotherapy for advance retinoblastoma (a). CT Scans with saggital reconstruction of the same patient (b).
See this image and copyright information in PMC

References

    1. Abramson D.H., Niksarli K., Ellsworth R.M. Changing trends in the management of retinoblastoma 1951–1965 vs 1966–1980. J. Pediatr. Ophthalmol. Strabismus. 1994;31:32–37. - PubMed
    1. Abramson D.H., Beaverson K., Sangani P. Screening for retinoblastoma: presenting signs as prognosticators of patient and ocular survival. Paediatrics. 2003;112:1248–1255. - PubMed
    1. Acquaviva A., Barberi L., Bernardini C. Medical therapy of retinoblastoma in children. J. Neurosurg. Sci. 1982;26:49–52. - PubMed
    1. Ajaiyeoba I.A., Akang E.E., Campbell O.B. Retinoblastomas in Ibadan: treatment and prognosis. West Africa J. Med. 1993;12:223–227. - PubMed
    1. Ali, M.J., Gupta, R., Vemuganti, G.K., Honavar, S.G., 2009. Histopathology of retinoblastoma after primary chemotherapy. In: Proceedings of the XIV International Congress of Ocular Oncology, Cambridge, p. 296.

LinkOut - more resources