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The outbreak of rapidly spreading COVID-19 pandemic in December 2019 has witnessed a major transformation in the health care system worldwide. This has led to the re-organization of the specialty services for the effective utilization of available resources and ensuring the safety of patients and healthcare workers. Suspension of oncology services will have major implications on cancer care due to delayed diagnosis and treatment leading to irreversible adverse consequences. Therefore various oncology organizations have called for a continuation of cancer care during this crisis with diligence. The COVID-19 pandemic has forced the clinicians to transform the components of care from screening to outpatient care and primary management. The purpose of this article is to establish guidelines and recommendations for ocular oncology in the management of ocular tumors set by a multidisciplinary team of experts including ocular, medical and radiation oncologists, and pathologists. As the pandemic is evolving fast, it will require constant updates and reformation of health strategies and guidelines for safe and quality health care.

Dermoid cyst, a developmental benign choristoma, is the most common orbital tumor of childhood, arising from ectodermal sequestration along the lines of embryonic fusion of mesodermal processes, lined by keratinized stratified squamous epithelium and expanding slowly due to constant desquamation and dermal glandular elements. Approximately 80% are found in the head and neck region and comprise 3-9% all orbital masses.It is mandatory to know about the variable presentations of orbital dermoids and the surgical techniques that can be adopted based on the site, extent, age and aesthetic needs, presence of inflammation and possibility of intraoperative rupture.Orbital dermoids can be classified as juxta-sutural, sutural or soft tissue cysts; superficial or deep; intraosseous or extraosseous, and intraorbital or extraorbital. These smooth, painless, mobile or partially mobile lesions mostly present at the fronto-zygomatic suture with proptosis, displacement, ptosis or diplopia, depending on depth and extent. Therefore, it is important to understand the various presentations and the appropriate surgical techniques.We describe the embryological origin, types and clinical features of dermoids in this video and demonstrate the surgical and minimally invasive techniques for their management.https://youtu.be/-q3xD2igjcQ.

Adenoid cystic carcinoma (ACC) is a rarely seen malignant epithelial tumor of the eyelids. We present a rare case of primary ACC arising from the tarsal plate of the lower lid that clinically resembled a chalazion. A 66-year-old female presented with a recurring nodule in her left lower lid. She gave history of surgery for chalazion removal from the same site twice earlier. An initial diagnosis of a recurrent chalazion was made, and incision and curettage was done. Light microscopy showed a solid tumor composed predominantly of sheet-like and nested pattern of basaloid to low-columnar cells with intervening fibrovascular septa and lacking an obvious cribriform or tubular architecture. The tumor cells were positive for pan-cytokeratin and CD117 and negative for adipophilin, HMB45, and BerEP4. A diagnosis of solid variant of ACC of the eyelid was made. Wide excision was performed and eyelid defect was reconstructed.

A 51-year-old Asian Indian male with right oculodermal melanocytosis presented with a blurred visual acuity of 20/40. Upon fundus examination, he was discovered to have two independent pigmented choroidal melanomas in the temporal juxtapapillary region and inferiorly, in the region of trace clinical sectoral choroidal melanocytosis. The patient underwent enucleation, and on histopathology the two tumors were found to be in the area of choroidal melanocytosis, in continuity with each other. Multifocal choroidal melanoma is an exceedingly rare diagnosis. Ocular melanocytosis is a known predisposing factor for both unifocal and multifocal melanomas. Lifelong monitoring for uveal melanomas must be done in all patients with ocular melanocytosis.

Ocular amylodosis, although a rare entity, is known to affect the conjunctiva, extraocular muscles, orbit, lacrimal gland, and skin around the eyes. Intraocular deposition of amyloid mainly confines to the vitreous and cornea. In this report, we describe two cases of intraocular amyloidosis presenting as multiple iris and anterior chamber cysts. Histopathological examination with special stain like Congo Red and Transmission Electron Microscopy confirmed the diagnosis of amyloidosis. Systemic investigations ruled out systemic association confirming the diagnosis of primary ocular amyloidosis.

Purpose: The novel coronavirus SARS-CoV-2 (COVID-19) and the resultant nationwide lockdown and travel restrictions led to difficulty in providing timely and regular treatment to patients with childhood cancers such as retinoblastoma. This study is aimed at assessing the demography, clinical presentation, treatment strategies, and outcome of treatment defaulters due to the lockdown. Methods: Cross-sectional, observational study of retinoblastoma patients at a tertiary care ocular oncology center during the first wave of COVID-19 and the resulting nationwide lockdown. Results: Of the 476 eyes of 326 patients undergoing active management with a median age of 57 months (range: 4–214 months), 205 (63%) patients returned for follow-up after a mean delay of 45.8 ± 24.3 weeks (range: 8–80 weeks) and 121 (37%) were defaulters according to the data analyzed till June 30, 2021. Distance of residence was ≥1000 km for 148 patients (46%). In terms of need for active treatment, the number of emergent cases was 2 (

Uveal melanoma is a rare malignancy originating from melanocytes within the uveal tract of the eye. True multifocal uveal melanomas (>2melanomas in 1 eye) occurring in the same eye are very rare. We report a clinically and histologically well-documented case of a patient who presented with 3 uveal melanoma lesions in the same eye over a span of 2.5 years. The eye had to be enucleated finally and diagnosis confirmed histologically. This case report highlights the need for a close follow-up, even after successful treatment of the presenting lesion in uveal melanomas.

Low-grade myofibroblastic sarcoma is a relatively recently-described neoplasm of the myofibroblasts having a predilection for the head and neck region. Ophthalmic involvement is extremely rare. Limbal involvement has not yet been documented in the literature. We describe one such case involving the limbus of a 48-year-old Asian male.

A 42-year-old Asian Indian male with a history of conjunctival melanoma in the left eye presented with a recurrent tumor in the upper tarsal conjunctiva. The tumor was completely excised under margin control, followed by two-staged eyelid reconstruction. During the second stage of the eyelid reconstruction, a brown-colored discharge was noted at the punctum, which on cytology was confirmatory of melanoma. Left dacryocystectomy with en bloc nasolacrimal duct (NLD) excision was performed. Histopathology demonstrated infiltration of the NLD by the tumor with no presence of melanoma in the lacrimal sac. Lacrimal oncorrhea is a term used to describe tumor spread by free-floating cells in the tear film. All conjunctival tumors carry a risk of tumor spread by oncorrhea.

Sweat gland neoplasms are rare adnexal tumors that pose a diagnostic challenge for both, ophthalmologists and pathologists. Endocrine, mucin producing sweat gland carcinoma (EMPSGC), considered to be analogous to the solid papillary mammary carcinoma is one such tumor. It usually affects elderly, is more frequent in women and has a predilection for skin of the eyelid. Although it has an indolent clinical course, EMPSGC is believed to be a precursor of the invasive mucinous carcinoma and has a potential for local recurrence. We report a series of 10 biopsy-proven EMPSGCs with their immunohistochemical features and review the literature.

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. A diagnosis of low-grade fibromyxoid sarcoma (LGFS) was made. The patient was treated by stereotactic external beam radiotherapy. Here, we report a case of LGFS which, to the best of our knowledge, is the first at an orbital location.

Isolated enophthalmos is a rarely observed entity in neurofibromatosis (NF). A 12-year-old male presented with right lower eyelid fat prolapse and enophthalmos for the past 7 years. There was no history of antecedent trauma/surgery. Computed tomography of orbit revealed an ill-defined intraconal hyperdense lesion located between lateral and inferior rectus along with an enlarged inferior orbital fissure (IOF). Superior orbital fissure was minimally widened without prolapse of any intracranial contents. Excision biopsy along with repair of widened IOF was performed through inferior transconjunctival route. Histopathology was suggestive of plexiform neurofibroma with positivity for S-100 and epithelial membrane antigen. No associated cutaneous lesions were present. Nonpulsatile enophthalmos with eyelid fat prolapse can be a presenting sign of NF.

Primary vitreoretinal lymphoma (PVRL) is an uncommon, but potentially fatal intraocular malignancy, which may occur with or without primary central nervous system lymphoma (PCNSL). Considered to be a subset of PCNSL, it is mostly of diffuse large B-cell type. The diagnosis of PVRL poses a challenge not only to the clinician, but also to the pathologist. Despite aggressive treatment with chemotherapy and/or radiotherapy, relapses or CNS involvement are common.