Intravitreal Topotecan for Vitreous Seeds in Retinoblastoma: A Long-term Review of 91 Eyes.

Purpose: To study the long-term efficacy of intravitreal topotecan (IVT) for vitreous seeds in eyes with retinoblastoma and risk factors for their recurrence.

Design: Retrospective, non-comparative, interventional study.

Participants: Ninety-one eyes of 90 patients with retinoblastoma treated between January 2013 and April 2019.

Metastatic retinoblastoma at presentation: Clinical presentation, treatment, and outcomes.

The aim of this study was to retrospectively determine clinical features, treatment outcomes, and overall survival in four patients with metastatic retinoblastoma at presentation. The mean age at diagnosis was 63 months (range: 24-108 months). Three patients had overt orbital disease of at least one eye and one patient had microscopic orbital disease with scleral infiltration on histopathology.

Plaque brachytherapy - Radiating positivity.

Background: Plaque brachytherapy is an evolving, yet effective globe and vision-sparing modality for the treatment of intraocular tumors by transscleral irradiation of the tumor base with a radioactive implant. The American Brachytherapy Society (ABS) along with the collaboration of the international multicenter Ophthalmic Oncology Task Force (OOTF) was assembled to reach a consensus regarding establishing practice guidelines and setting standards of care for intraocular tumors. The advent of plaque brachytherapy has revolutionized the outcomes of intraocular tumors, thus ensuring globe salvage, reducing morbidity and mortality, and avoiding cosmetic disfigurement.

Does Multimodal Treatment Improve Eye and Life Salvage in Adenoid Cystic Carcinoma of the Lacrimal Gland?

Purpose: To evaluate the efficacy of multimodal treatment in adenoid cystic carcinoma (ACC) of the lacrimal gland.

Methods: A retrospective comparative case series of 40 consecutive patients with ACC of the lacrimal gland without systemic metastasis at the initial presentation and primarily managed by one of the 3 treatment protocols-surgery + external beam radiotherapy (EBRT) (group 1), surgery + EBRT + adjuvant chemotherapy (group 2), and neoadjuvant chemotherapy + surgery + EBRT + adjuvant chemotherapy (multimodal treatment) (group 3) at a tertiary care ocular oncology center. Local tumor control, eye salvage, and systemic metastasis were the primary outcome measures.

Primary orbital low-grade fibromyxoid sarcoma - A case report.

An 18-year-old female presented with rapidly progressive proptosis of the left eye for one month and grade II relative afferent pupillary defect. Orbital imaging showed a well-defined homogenous extraconal mass in close relation to the lateral rectus muscle and extending up to the superior orbital fissure, associated with bony erosion. An incisional biopsy was performed, with the histopathology demonstrating stellate to spindle-shaped tumor cells (fibroblasts) embedded in a richly myxoid matrix.

Eye salvage in diffuse anterior retinoblastoma using systemic chemotherapy with periocular and intravitreal topotecan.

Diffuse anterior retinoblastoma is an infrequent presentation in which the tumor is confined to the anterior segment, and there is no retinal mass. An 18-month-old Asian Indian boy presented with white spots in the left eye. There were extensive anterior segment seeds without a retinal tumor.

Intravitreal topotecan in the management of refractory and recurrent vitreous seeds in retinoblastoma.

Background/aim: To evaluate the efficacy of intravitreal topotecan for refractory or recurrent vitreous seeds in retinoblastoma.

Methods: Intravitreal injection of topotecan hydrochloride (30 µg/0.15 mL) was provided every 3 weeks by the safety enhanced technique.

Aqueous Deficient Dry Eye Syndrome Post Orbital Radiotherapy: A 10-Year Retrospective Study.

Purpose: Despite advances in orbital radiotherapy (XRT), a significant proportion of patients develop ophthalmic complication like dry eye syndrome (DES). The study evaluates the prevalence of aqueous deficient DES (ADDE) and lacrimal gland (LG) changes through histologic evaluation and ex-vivo expansion potential postorbital XRT.

Methods: With the approval of the institutional review board, medical records of patients who underwent orbital XRT as management protocol were reviewed for evidence of ADDE using DEWS (Dry Eye Workshop) 2007 criteria ( = 51).

Orbital retinoblastoma: An update.

Orbital extension is a major cause of death in children with retinoblastoma in the developing countries. Delayed detection and inappropriate management contribute to poor outcome. Conventional treatment including primary orbital exenteration or chemotherapy or radiotherapy alone result in mortality as high as 70%.

Ocular Surface Squamous Neoplasia in Papillon-Lefèvre Syndrome: Outcome at Long-Term Follow-Up of 12 Years.

Purpose: To discuss the association between ocular surface squamous neoplasia (OSSN) and Papillon-Lefèvre syndrome (PLS) and present the long-term outcome in a patient with these diseases.

Methods: Case report.

Results: A 14-year-old boy presented with a raised pigmented mass lesion at the limbus in the right eye, which was clinically suggestive of OSSN.

Choroidal Infiltration by Retinoblastoma: Predictive Clinical Features and Outcome.

Purpose: To identify the clinical features predictive of choroidal infiltration by retinoblastoma on histopathology and to report the outcome in these patients.

Methods: Retrospective study.

Results: Of the 403 patients who underwent primary enucleation for retinoblastoma, 113 patients had choroidal tumor infiltration and 290 patients had no choroidal tumor infiltration.

OPTIC NERVE INFILTRATION BY RETINOBLASTOMA: Predictive Clinical Features and Outcome.

Purpose: To identify the clinical features predictive of any optic nerve infiltration and postlaminar optic nerve infiltration by retinoblastoma on histopathology and to report the outcome (metastasis and death) in these patients.

Methods: Retrospective study.

Results: Of the 403 patients who underwent primary enucleation for retinoblastoma, 196 patients had optic nerve tumor infiltration (Group 1) and 207 patients had no evidence of optic nerve tumor infiltration (Group 2).

Neoadjuvant Systemic Chemotherapy in the Management of Extensive Eyelid Sebaceous Gland Carcinoma: A study of 10 Cases.

Purpose: To report the efficacy of neoadjuvant systemic chemotherapy in the management of eyelid sebaceous gland carcinoma (SGC).

Methods: Retrospective study of 10 patients that received neoadjuvant systemic chemotherapy (Cisplatin/Carboplatin and 5-Fluorouracil) for eyelid SGC.

Results: The mean age at presentation of eyelid SGC was 58 years (median, 55 years; range, 45 to 72 years).

Orbital alveolar soft-part sarcoma: clinico-pathological profiles, management and outcomes.

Aim: To study the clinico-radiological and clinic-pathological features of orbital alveolar soft-part sarcomas (ASPS).

Materials And Methods: We retrieved 9 cases of orbital ASPS. Histopathological and immunohistochemical features and clinico-radiological findings were evaluated.

Management and outcome of retinoblastoma with vitreous seeds.

Purpose: To report the treatment response of retinoblastoma with vitreous seeds to high-dose chemotherapy coupled with periocular carboplatin.

Design: Retrospective, interventional case series.

Participants: Consecutive patients with retinoblastoma with vitreous seeds managed over 10 years at a comprehensive ocular oncology center and followed up for at least 12 months after the completion of treatment were included in this study.

Malignant teratoid ciliary body medulloepithelioma in a neonate.

Ciliary body medulloepithelioma is an intraocular tumor manifesting in early childhood, rarely at birth. A unique case of intraocular malignant teratoid ciliary body medulloepithelioma in a neonate presenting as buphthalmos at birth is reported. There was rapid progression with extraocular extension within 2 months and developed regional lymph node metastasis despite enucleation.

Adenoid cystic carcinoma of the lacrimal gland: role of nuclear survivin (BIRC5) as a prognostic marker.

Aims: This study aimed to evaluate the expression of nuclear survivin in adenoid cystic carcinoma (ACC) of the lacrimal gland and to determine if this expression is associated with histopathological features, markers of apoptosis and proliferation or clinical outcomes.

Methods And Results: Immunohistochemical staining for survivin, p53, Ki-67 and Bcl-2 was analyzed in 55 cases of ACC of lacrimal gland. Thirty-one cases (56.

Optic nerve meningeal hemangiopericytoma: a clinicopathologic case report.

A 36-year-old woman presented with progressive loss of vision in the left eye for 3 years, and rapid progression and painful protrusion of the eye for one month. Clinical evaluation revealed no light perception, severe proptosis and hypoglobus, optic atrophy, and optociliary shunt vessels. Orbital imaging showed a well-defined heterogeneous intraconal mass partially encasing the optic nerve.

Establishing human lacrimal gland cultures with secretory function.

Purpose: Dry eye syndrome is a multifactorial chronic disabling disease mainly caused by the functional disruptions in the lacrimal gland. The treatment involves palliation like ocular surface lubrication and rehydration. Cell therapy involving replacement of the gland is a promising alternative for providing long-term relief to patients.

Orbital retinoblastoma: where do we go from here?

Diagnosis of orbital retinoblastoma traditionally carries a dismal prognosis. Although its incidence is less in the developed countries, it continues to contribute to an epidemic of extraocular disease at diagnosis in the developing world. Orbital retinoblastoma encompasses a wide range of distinct clinical entities with varying tumor load.

RB1 gene mutations in retinoblastoma and its clinical correlation.

Purpose: To find correlation between the type of mutations observed and the severity of the disease using multiple techniques like polymerase chain reactions (PCR), quantitative multiplex PCR, sequencing and RNA analysis.

Methods: Prospective, observational study. Patients who had been screened for mutations in the RB1 gene were included in the study.

Histopathologic risk factors in retinoblastoma in India.

Context: The presence of histopathologic risk factors is associated with development of metastasis in a patient with retinoblastoma. Adjuvant chemotherapy administered to such patients decreases the risk of metastasis.

Objective: To analyze the incidence of histopathologic risk factors in our patient population and the clinical predictors of such risk factors.