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.

Efficacy of topical 5-Fluorouracil in the management of ocular surface squamous neoplasia: a study of 101 eyes.

OBJECTIVE: To study the efficacy and side-effect profile of topical 5-Fluorouracil (5-FU) in the treatment of ocular surface squamous neoplasia (OSSN). METHODS: Retrospective study of 101 eyes of 100 patients treated with 5-FU with one week on and 3 weeks off regimen. RESULTS: Of the 100 patients (101 eyes), the mean age at diagnosis of OSSN was 49 (median, 52 years; range, 11-87 years). History of prior intervention was noted in 6 (6%) eyes. Tumor epicenter included bulbar conjunctiva (n = 54; 53%), limbus (n = 27; 27%), and cornea (n = 20;20%). Mean number of cycles of topical 5-FU administered was 3 (median, 3; range, 1-8). Complete tumor regression was achieved with topical 5-FU in 89 (88%) eyes with a mean number of 2 cycles (median, 2; range, 1-6) of 5-FU. The remaining 12 (12%) lesions underwent additional treatment including excisional biopsy (n = 7), extended enucleation (n = 3), and topical Interferon alpha 2b (n = 2) for complete tumor control. Over a mean follow-up period of 6 months (median, 5 months; range, 1-36 months) following treatment, tumor recurrence was noted in 2 (2%) patients, and side-effects were noted in 7 (7%) eyes including conjunctival hyperemia (n = 1), punctal stenosis (n = 1), sterile keratitis (n = 4), and limbal stem cell deficiency (n = 1). CONCLUSION: Topical 5-FU is an effective non-invasive therapy for OSSN with a minimal side-effect profile.

Ocular surface squamous neoplasia in India: a study of 438 patients.

PURPOSE: To describe the clinical features, histopathology, treatment, and outcomes of patients with ocular surface squamous neoplasia (OSSN) presenting to a referral centre in India. METHODS: Retrospective interventional study. RESULTS: Of 438 patients, the mean age at presentation was 49 years. Human immunodeficiency virus infection was noted in 72 (16%), xeroderma pigmentosum in 22 (5%), hepatitis B virus infection in 14 (3%), and systemic cancer in 8 (2%) patients. Tumor pigmentation was noted in 243 (54%) tumors with a mean percentage of tumor pigmentation of 44% (median, 40%; range, 1 to 100%). Intraocular tumor extension was noted in 12 (3%), and orbital tumor extension in 16 (4%) eyes. Of the 381 treated lesions, excisional biopsy (n = 247; 65%) was the most common treatment modality. Of the 311 lesions with histopathology diagnosis of OSSN, invasive squamous cell carcinoma (n = 92; 30%) was the most common. Over a mean follow-up period of 11 months (median, 5 months; range, 1 to 108 months) in 368 patients, tumor recurrence was noted in 16 (4%) eyes, globe salvage was achieved in 341 (90%) eyes, vision salvage in 338 (89%) eyes, regional lymph node metastasis occurred in 9 (2%), and metastasis-related death in 9 (2%) patients. CONCLUSION: Pigmented OSSN is common in Asian Indian population. Appropriate management of OSSN is associated with good vision, globe, and life salvage rates in India.

Periocular Lobular Capillary Hemangioma in Adults: A Clinicopathological Study.

PURPOSE: To describe a case series of periocular lobular capillary hemangiomas in adults, outlining characteristic clinical and histopathological patterns. MATERIALS AND METHODS: This was a retrospective case series of 16 patients with review of clinical and histopathological features. RESULTS: Eleven male and five female patients were diagnosed with periocular lobular capillary hemangioma at a median age of 38 years (mean, 41 years; range, 21-71 years). The median tumor basal diameter was 6 mm (mean, 7 mm; range, 3-14 mm) and all were well circumscribed. They arose over the course of weeks to months and developed most commonly in the eyelid region (n = 10), followed by the conjunctiva (n = 6). Excisional biopsy of the lesion was done in all cases. On histopathology, the tumors were composed of repeating units of capillary-sized lobules lined by plump endothelial cells. Lesion recurrence was noted in one case. CONCLUSION: Lobular capillary hemangiomas are common benign vascular tumors of periocular region in adults. Clinicohistopathological features and clinical presentation of these lesions are distinctive. Excisional biopsy was curative with recurrence noted rarely.

Interferon Alfa 2b for Ocular Surface Squamous Neoplasia: Factors Influencing the Treatment Response.

PURPOSE: To study the factors influencing the response to treatment with interferon alfa 2b (IFN) in ocular surface squamous neoplasia (OSSN)Methods: Retrospective study of 91 patientsResults: The mean age at presentation of patients with OSSN was 58 years (median, 60 years; range, 21 to 83 years). The mean number of clock hours of conjunctiva/cornea/limbus involvement by the tumor was 6 (median, 6; range, 1 to 12). The mean duration of topical IFN was 3 months (median, 3 months; range, 1 to 6 months) and the mean number of subconjunctival injections of IFN was 2 (median, 2; range, 0 to 6), till complete tumor regression or initiation of alternate treatment. Of 91 OSSN cases treated with IFN, 72 (79%) patients showed complete response to treatment, while 19 (21%) showed partial response displaying mean % tumor reduction of 34% (median, 20%; range, 5% to 90%). Patient demographics, immune status, disease chronicity, tumor location, or morphological pattern were not predictive of tumor response to IFN. The only factor predictive of incomplete response of OSSN to IFN was more than 6 clock hour involvement of ocular surface by OSSN (p = .04). Of 31 (34%) cases with OSSN >6 clock hours, 23 (74%) patients showed complete tumor regression with IFN alone, while 8 (26%) patients displayed incomplete response; and of 60 (66%) cases with OSSN ≤6 clock hours, 49 (82%) patients showed complete tumor regression with IFN alone, while 11 (18%) patients displayed incomplete response. Tumor recurrence was noted in 3% cases and one case had corneal perforation secondary to infective keratitis over a mean follow-up period of 14 months (median, 8 months; range, 3 to 58 months). CONCLUSION: Clock hour involvement of ocular surface by OSSN determines the response to IFN. Interferon alfa 2b is an effective immunotherapy agent for tumors ≤6 clock hours of ocular surface in 82% cases and serves as an immunoreducing agent for larger tumors involving >6 clock hours of ocular surface in 26% cases.

Enucleation in Asian Indian patients: a histopathological review of 2009 cases.

OBJECTIVE: To review the indications of enucleation in Asian Indian patients and study the trend over the 22-year period. METHODS: Retrospective study of 2009 patients who underwent enucleation. RESULTS: The mean age at presentation of patients who underwent enucleation was 155 months The histopathology diagnosis included a benign tumor (n = 22, 1%), malignant tumor (n = 1472, 73%), acute trauma (n = 93, 5%), retinal vascular disease (n = 50, 3%), inflammatory/infective pathology (n = 33, 2%), or other miscellaneous/non-specific diagnosis (n = 460, 23%). There was a good correlation between the clinical and histopathology diagnoses at 96%. The most common indication for enucleation in young patients ( ≤ 20 years) was retinoblastoma (n = 1257, 82%; p < 0.001), atrophic bulbi or phthisis bulbi (n = 163, 39%; p < 0.001) in middle-age adults, and uveal melanoma (n = 25, 42%; p < 0.001) in older adults. Over the years, there was a decreasing trend of enucleations for atrophic bulbi/phthisis bulbi/painful blind eye (33% from the years 1996 through 2000 to 7% from 2010 to 2018; p < 0.001) and acute trauma (3% from the years 1996 through 2000 to < 1% from 2010 to 2018; p < 0.001) and an increasing trend for intraocular tumors including retinoblastoma (56% from the years 1996 through 2000 to 73% from 2010 to 2018; p = 0.01) and uveal melanoma (3% from the years 1996 through 2000 to 11% from 2010 to 2018; p < 0.006). CONCLUSION: In Asian Indian population, malignant tumors remain the most common indication for enucleation in young and older patients, while desire for better cosmesis with customized ocular prosthesis is the main indication for enucleation in middle-age adults.

Malignant Eyelid Tumors in India: A Study of 536 Asian Indian Patients.

PURPOSE: To study the proportion of eyelid malignant tumors in an Asian Indian population and to review their clinical features and outcomes. METHODS: This is a retrospective study of 536 patients. RESULTS: The mean age at presentation with eyelid malignancy was 58 years. Histopathology-proven diagnoses of these patients included sebaceous gland carcinoma (SGC) (n = 285, 53%), basal cell carcinoma (BCC) (n = 128, 24%), squamous cell carcinoma (SCC) (n = 99, 18%), and miscellaneous tumors (n = 24, 4%). The statistically significant differences between eyelid malignant tumors included age at presentation, tumor location, and tumor extent. The clinicopathological correlation of SGC, BCC, SCC, and miscellaneous tumors was 91, 86, 46, and 38% (p = 0.001), respectively. Comparing SGC with BCC, SCC, and miscellaneous tumors, SGC was more commonly associated with tumor recurrence (21 vs. 3, 8, and 13%; p = 0.001), systemic metastasis (13 vs. 0, 4, and 13%; p = 0.001), and death (9 vs. 0, 4, and 0%; p = 0.004). Compared to SGC, BCC, and SCC, locoregional lymph node metastasis was more common with miscellaneous tumors (26 vs. 16, < 1, and 8%; p = 0.001) over a mean follow-up period of 19 months. CONCLUSION: In Asian Indians, SGC is twice as common as BCC and 3 times more common than SCC. SGC is associated with poorer prognosis compared to other eyelid malignant tumors.