Late-onset retinoblastoma in a well-functioning fellow eye.
OBJECTIVE
To describe the documented growth, clinical course, and histopathology of retinoblastomas in an untreated and otherwise normal right eye of a 27-year-old white male with a g.153211T>A (p.Tyr606X) mutation in the retinoblastoma 1 gene, whose left eye was enucleated at age 2 years for 2 retinoblastomas.


METHODS
Retrospective interventional case report.


METHODS
Over the years, the right eye was irradiated twice and underwent trans-pars plana vitrectomy, transscleral cryocoagulation, argon laser photocoagulation of tumors and their feeder vessels, extracapsular cataract extraction with posterior chamber lens implantation, and neodymium:yttrium-aluminum-garnet laser treatment of after-cataract in the form of Elschnig's pearls. Finally, the patient received combination chemotherapy with etoposide, methotrexate, actinomycin D, cisplatin, and vincristine.


RESULTS
The eye finally had to be removed 12 years later due to tumor recurrences and seeding, pseudohypopyon, and elevated intraocular pressure. Histopathology showed microcellular retinoblastoma cells in the anterior chamber angle and trabecular meshwork without subconjunctival extension and in the nasal ciliary body, pars plana, internal limiting membrane, and optic nerve head anterior to the cribriform plate. The patient is without local or systemic recurrences at age 50, 11 years after the last eye was enucleated.


CONCLUSIONS
This report shows that retinoblastoma patients may have tumor growth in their fellow eye 25 years after the first eye and also that Elschnig's after-cataract pearls still can arise after irradiation of a lens with 45 Gy.
de Jong
Paulus T V M
PT
Netherlands Institute for Neurosciences, Royal Netherlands Academy of Arts and Sciences, Amsterdam, The Netherlands. p.dejong@ioi.knaw.nl
Mooy
Cornelia M
CM
Stoter
Gerrit
G
Eijkenboom
Wilhelmina M H
WM
Luyten
Gregorius P M
GP
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002387:Cataract Extraction; D017679:Cryotherapy; D015353:Eye Enucleation; D007839:Functional Laterality; D006801:Humans; D017075:Laser Coagulation; D008297:Male; D017354:Point Mutation; D019572:Retinal Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D012175:Retinoblastoma / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D016160:Retinoblastoma Protein / Q000235:genetics; D012189:Retrospective Studies; D014443:Tyrosine / Q000235:genetics; D014821:Vitrectomy
D016160:Retinoblastoma Protein; D014443:Tyrosine