Comparative evaluation of clinical characteristics of biopsy-proven conjunctival intraepithelial neoplasia and invasive squamous cell carcinoma using image processing software programs.

PURPOSE: To identify morphological parameters aiding clinical differentiation of conjunctival intraepithelial neoplasia (CIN) and invasive squamous cell carcinoma (iSCC) and to demonstrate the utility of image processing software to objectively assess ocular surface squamous neoplasia (OSSN). METHODS: This retrospective case series included all biopsy-proven cases of OSSN presenting as an ocular surface nodule. Based on histopathology, lesions were classified as CIN and iSCC. Clinical image analysis utilized 'Contour' and 'ImageJ' software. The effect of predictors demography, seropositivity, lesion dimensions, keratin, pigmentation, corneal involvement, vascularity and feeder vessels on the final histopathologic grade were assessed. RESULTS: A total of 108 OSSN lesions (74 CIN and 33 iSCC) were included. Mean age was 46.1 ± 17.2 years in CIN and 47.2 ± 13.9 years in iSCC. By univariate logistic regression analysis, significant predictors of iSCC were HIV seropositivity (p < 0.0001), maximum diameter (p = 0.003), perpendicular to maximum diameter (p = 0.003), height (p = 0.003), nodular morphology (p = 0.006) and feeder vessels (p = 0.03), whereas gelatinous morphology (p = 0.02) was predictor of CIN. By multiple logistic regression, seropositivity was the predictor of iSCC (p < 0.0001, OR 13.33 ± 8.35, 95% CI 3.90-45.53). CONCLUSION: HIV seropositivity is an important predictor of iSCC. Large, thick, nodular lesions with feeder vessels may favor the diagnosis of iSCC, whereas gelatinous, small, flatter lesions without feeder vessels may favor CIN. In a first of its kind study, simple and objective analysis of OSSN with image processing software was demonstrated.

Posterior uveal melanoma in 321 Asian Indian patients: analysis based on the 8th edition of American Joint Committee Cancer classification.

PURPOSE: To evaluate the presenting features, treatment, and outcomes of posterior uveal melanoma (PUM) in Asian Indians based on the 8th edition of American Joint Committee on Cancer (AJCC) classification. METHODS: Retrospective interventional case series of 321 Asian Indian patients with PUM. RESULTS: Based on AJCC, PUM was classified into categories T1 (n = 36; 11%), T2 (n = 74; 23%), T3 (n = 126; 39%), and T4 (n = 85; 27%). Regarding tumor features, T4 was more likely to have pre-equatorial epicenter (vs T1 and T2; p ≤ 0.011), iris abnormalities (vs T2 and T3; p ≤ 0.002), and extraocular tumor extension (vs T3; p = 0.001), whereas T1 was more likely to have macular epicenter (vs T2, T3, T4; p ≤ 0.013), lipofuscin deposits (vs T3 and T4; p ≤ 0.008), and amelanotic tumors (vs. T4; p = 0.003). On multivariate analysis, factors predictive of systemic metastasis were increasing tumor thickness (p = 0.002) and extraocular tumor extension (p = 0.009). The 5-, 10-, and 15-year melanoma-related metastases rates were 0%, 0%, and 0% in T1, 0%, 60%, and 60% in T2, 7%, 40%, and 70% in T3 and 13%, 36%, and 76% in T4, respectively. Risk for metastasis was 1.23 times more for every 1-mm increase in tumor thickness and 9 times more with extraocular tumor extension. CONCLUSION: The AJCC 8th edition provides prognostic classification for PUM in Asian Indian patients. The significant risk factors for metastasis were increasing tumor thickness and extraocular tumor extension.

Sebaceous gland carcinoma: analysis based on the 8th edition of American Joint Cancer Committee classification.

PURPOSE: To assess the prognostic value of T category of the 8th edition of American Joint Committee on Cancer (AJCC) classification in periocular sebaceous gland carcinoma (SGC). METHODS: Retrospective interventional case series of 119 cases. RESULTS: Based on the T category of 8th edition of AJCC classification, 119 periocular SGCs were classified into T1 (n = 33, 28%), T2 (n = 37, 31%) T3 (n = 17, 14%) and T4 (n = 32, 27%). There were no statistically significant differences in the rate of tumour recurrence based on T category. The outcome measures that showed significant increase with increase in T category included regional lymph node metastasis (3% for T1, 3% for T2, 12% for T3, and 44% for T4; p < 0.0001), systemic metastasis (0% for T1, 0% for T2, 12% for T3, and 25% for T4; p = 0.002) and death due to metastasis (0% for T1, 0% for T2, 12% for T3, and 22% for T4; p = 0.005). The 5-year Kaplan-Meier estimate rate for regional lymph node metastasis, systemic metastasis and metastasis-related death were all higher for the T4 category tumours (42%, p = 0.005; 34%, p = 0.0002; and 43%, p = 0.0001 respectively) compared to T1 (9%, 0%, and 0%), T2 (5%, 0%, and 0%) and T3 (10%, 17 and 8%) tumours. CONCLUSION: Primary tumour (T) category of the 8th edition AJCC classification predicts the prognosis of patients with periocular SGC. The rates of lymph node metastasis, systemic metastasis, and death is much higher in T4 tumours compared to T1, T2, and T3 tumours. There was no association between T category and tumour recurrence.

Ocular surface squamous neoplasia with intraocular tumour extension: a study of 23 patients.

PURPOSE: To describe the clinical features, histopathology and treatment of ocular surface squamous neoplasia (OSSN) with intraocular tumour extension. METHODS: Retrospective study of 23 patients. RESULTS: The mean age at presentation of OSSN with intraocular tumour extension was 48 years. Mass (52%) and pain with redness (30%) were the most common presenting complaints. The mean duration of symptoms was 9 months. History of human immunodeficiency virus (HIV) infection was present in eight (35%) patients and one (4%) had xeroderma pigmentosum. History of prior tumour excision was noted in 16 (70%) patients. The mean basal diameter was 17 mm and the mean tumour thickness was 4 mm. Fifteen (65%) tumours had a nodulo-ulcerative tumour pattern at the time of detection of intraocular extension of OSSN. Anterior chamber cells and flare was noted in five (24%) cases and two (9%) patients had secondary glaucoma. Ultrasound biomicroscopy (UBM) (n = 11) revealed blunting of anterior chamber in three (27%) cases and uveal thickening in seven (67%) cases. Over the course of follow-up, extended enucleation (n = 6; 26%) or orbital exenteration (n = 17; 74%) was required for tumour control. At a mean follow-up period of 18 months, locoregional lymph node metastasis was seen in two (9%) patients, and one patient died with systemic metastasis. On histopathology, ciliary body was involved in all (100%) cases. CONCLUSION: Multiple tumour recurrences with history of prior tumour excision and nodulo-ulcerative tumour pattern are commonly associated with intraocular tumour extension of OSSN. UBM is a useful tool to detect intraocular extension of OSSN.

Neonatal retinoblastoma: A study of five cases.

PURPOSE: The purpose was to study the clinical features, treatment, and outcome of retinoblastoma (RB) in neonates. METHODS: This was a retrospective study of five patients with RB detected in the 1st month of life. RESULTS: The mean age at diagnosis of RB was 22 days (median, 24 days; range, 14-28 days). There were one female and four males with neonatal RB. Two patients had a known family history of RB, and the tumor was detected by routine fundus screening in these children. Two patients were brought in with complaints of leukocoria and one patient with complaints of red-eye. All patients had an intraocular tumor at presentation. Four patients had bilateral involvement, whereas only one had unilateral involvement. Based on the International Classification of Intraocular Retinoblastoma, the tumors were classified as Group A (n = 2), Group B (n = 3), Group C (n = 1), Group D (n = 1), and Group E (n = 2). Macular involvement was noted in 6 (67%) eyes. The primary treatment included systemic chemotherapy with/without focal treatment in all patients. One patient subsequently underwent secondary enucleation as the globe became phthisical. One child died while on treatment due to pneumonia secondary to chemotherapy-induced neutropenia. Of the four patients who completed treatment, globe salvage was achieved in 6 (86%) eyes over a mean follow-up period of 89 months (median, 92 months; range, 29-144 months). CONCLUSION: Neonatal RB though rare, if detected early, has a favorable outcome of ocular and life salvage. Sporadic RB can occur in neonates, and a family history may not always be elicited.

Ocular and Periocular Tumors in Xeroderma Pigmentosum: A Study of 120 Asian Indian Patients.

PURPOSE: We studied the incidence, treatment, and outcome of ocular and periocular tumors in patients with xeroderma pigmentosum (XP). DESIGN: Retrospective case series. METHODS: This single-institution study included 120 patients with XP who underwent intervention with excisional biopsy, enucleation, or orbital exenteration. The primary outcome measures were the occurrence of eyelid or ocular surface tumor, globe salvage, locoregional and systemic metastasis, and death. RESULTS: The mean age at presentation was 19 years. A family history of XP was present in 32 (27%) patients. Over a mean follow-up of 61 months, 34 (28%) patients developed no ocular/adnexal tumor, 86 (72%) developed ocular surface malignancy, 15 (13%) developed eyelid malignancy, and 22 (18%) developed other head and neck malignancies. Of the 86 patients with ocular surface malignancy, 48 (56%) had unilateral tumor and 38 (44%) had bilateral tumors. Invasive squamous cell carcinoma (n = 51, 41%) was the most common ocular surface tumor. Of the 15 patients with eyelid tumors, 14 (93%) had unilateral tumor and 1 (7%) had bilateral involvement. Basal cell carcinoma (n = 8, 50%) was the most common eyelid tumor. There were events of ocular surface tumor recurrence (n = 55 eyes, 44%), eyelid tumor recurrence (n = 5 eyes, 31%), locoregional lymph node metastasis (n = 3, 2%), systemic metastasis (n = 1, 1%), and death (n = 1, 1%). Overall, globe salvage was achieved in 119 (99%) patients (both eyes were salvaged in 92 [76%] patients and at least 1 eye was salvaged in 27 [23%] patients). CONCLUSION: XP is frequently associated with ocular surface, eyelid, and other head and neck malignancies. Lifelong follow-up is mandatory in these patients.

Choroidal schwannoma presenting with neovascular glaucoma: A report of two cases.

Schwannoma is a rare benign tumor, which is more commonly found elsewhere in the body along myelin-producing peripheral nerves and is rarely reported within the eye. In this report, we describe two adult patients presenting with a choroidal mass lesion and neovascular glaucoma who underwent enucleation with clinical differential diagnoses of choroidal melanoma and choroidal hemangioma. Histopathology confirmed the diagnosis of choroidal schwannoma. This case series highlights the variable presentation of intraocular schwannoma and how they masquerade as other choroidal tumors.

Bilateral advanced (group D or E) intraocular retinoblastoma: outcomes in 72 Asian Indian patients.

PURPOSE: To study the clinical presentation, treatment, and outcomes of patients with bilateral advanced intraocular retinoblastoma. METHODS: Retrospective case series of 72 patients. RESULTS: The mean age at presentation was 19 months. Leukocoria (n = 49, 68%) was the most common presenting complaint. The tumors were classified as groups D (n = 60, 42%) or E (n = 84, 58%) based on the Philadelphia version of International Classification of Retinoblastoma (ICRB); groups D (n = 84, 58%) or E (n = 60, 42%) based on Children's Hospital Los Angeles version of International Classification of Intraocular Retinoblastoma (ICIoR); T2 (n = 116, 81%) or T3 (n = 28, 19%) based on 8th edition American Joint Committee Classification (AJCC). Systemic chemotherapy (n = 138, 96%) was the most common primary treatment modality. The chance of globe salvage was higher for group D based on ICRB (83%; odds ratio (OR) 7.73; 95% confidence interval (CI) 3.45-17.33) or ICIoR (81%; OR 12.75; 95% CI 5.74-28.34) and T2b (73%; OR 5.19; 95% CI 2.51-10.73) based on AJCC. Over a mean follow-up period of 59 months, tumor recurrence was noted in 42 (29%) eyes and globe salvage was achieved in 83 (58%) eyes. Of the 50 eyes where vision was recorded, vision of 20/200 or better was achieved in 24 (48%) eyes. There were events of leukemia (n = 1, 1%), pinealoblastoma (n = 1, 1%), systemic metastasis (n = 3, 4%), and death (n = 4, 6%) during the follow-up period. CONCLUSION: Multimodality treatment allows globe salvage (58%) and vision salvage (48%) in eyes with advanced group D and E intraocular retinoblastoma.

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.