High-Risk Histopathological Features of Retinoblastoma following Primary Enucleation: A Global Study of 1426 Patients from 5 Continents.

PURPOSE: To evaluate high-risk histopathological features (HRHF) following primary enucleation of eyes with retinoblastoma (RB) and assess the patient outcomes across continents. METHODS: Retrospective study of 1426 primarily enucleated RB eyes from five continents. RESULTS: Of all, 923 (65%) were from Asia (AS), 27 (2%) from Australia (AUS), 120 (8%) from Europe (EUR), 162 (11%) from North America (NA), and 194 (14%) from South America (SA). Based on the continent (AS vs. AUS vs. EUR vs. NA vs. SA), the histopathology features included massive choroidal invasion (31% vs. 7% vs. 13% vs. 19% vs. 27%, p=0.001), post-laminar optic nerve invasion (27% vs. 0% vs. 16% vs. 21% vs. 19%, p=0.0006), scleral infiltration (5% vs. 0% vs. 4% vs. 2% vs. 7%, p=0.13), and microscopic extrascleral infiltration (4% vs. 0% vs. <1% vs. <1% vs. 4%, p=0.68). Adjuvant chemotherapy with/without orbital radiotherapy was given in 761 (53%) patients. Based on Kaplan-Meier estimates in different continents (AS vs. AUS vs. EUR vs. NA vs. SA), the 6-year risk of orbital tumor recurrence was 5% vs. 2% vs. 0% vs. 0% vs. 12% (p<0.001), systemic metastasis was reported in 8% vs. 5% vs. 2% vs. 0% vs. 13% (p=0.001), and death in 10% vs. 3% vs. 2% vs. 0% vs. 11% (p<0.001) patients. CONCLUSION: There is a wide variation in the infiltrative histopathology features of RB across continents, resulting in variable outcomes. SA and AS had a higher risk of orbital tumor recurrence, systemic metastasis, and death compared to AUS, EUR, and NA.

Trifocal choroidal melanoma in an eye with oculodermal melanocytosis: A case report.

We report a case of trifocal choroidal melanoma (three separate tumors) in a 48-year-old Caucasian female who had been followed for oculodermal melanocytosis since childhood. At presentation, no tumor was present and annual examination was advised. Seventeen years later, three choroidal melanocytic lesions were detected in the right eye. Growth of each was documented, enucleation was performed, and histopathology revealed three independent choroidal melanomas. The patient developed extensive liver and bone metastases and subsequently died. Oculodermal melanocytosis is a risk factor for the development of uveal melanoma and a potential marker for worse prognosis. Careful long-term follow-up is required.

Advanced Midzonal Iris Pigment Epithelial Cyst Managed by Aspiration Deflation.

BACKGROUND: There are several cysts that can occur in the iris. The midzonal (retroiridic) iris pigment epithelial (IPE) cyst is important because it can simulate melanoma of the ciliary body. However, it is frequently asymptomatic and requires no treatment. METHODS: A 37-year-old asymptomatic woman with visual acuity of 20/20 in each eye was found to have an elevated mass behind the left iris, presumed to be melanoma, and was referred for management. On our examination, there was a typical midzonal IPE cyst in the left eye (OS), requiring observation without intervention. After 4 years, she developed progressive visual loss to 20/40 OS and cyst enlargement was noted, obstructing most of the visual axis and causing partial lens subluxation. Translimbal fine needle aspiration deflation with a 30-G needle was performed. RESULTS: Immediately at aspiration, the cyst demonstrated collapse, disappearing behind the iris stroma, and the lens subluxation resolved. Visual acuity returned to 20/20 OS and has remained stable without recurrence for 3 years. CONCLUSION: Although most midzonal IPE cysts are asymptomatic, this case showed progressive enlargement with visual loss and was managed with translimbal aspiration. Symptomatic midzonal IPE cysts can be deflated with translimbal aspiration rather than more aggressive surgical excision.