Unusual conjunctival melanocytic proliferations. Report of five cases and review of the literature.

Indeterminate melanocytic proliferations of the conjunctiva have both benign and malignant features that previously made these lesions nearly impossible to categorize in existing classification schemes. With the evolution of immunohistochemistry and molecular genetics, however, subclassifications have emerged that allow for a more tailored diagnosis and management. These conjunctival melanocytic proliferations include deep penetrating nevus, granular cell nevus, and nevoid melanoma. There remains a small subset of conjunctival melanocytic proliferations that defy precise characterization as nevi, primary acquired melanosis, or melanomas despite currently available ancillary diagnostic modalities and remain indeterminate. We highlight these unusual types of nevi and melanomas, with an update on their morphologic, immunohistochemical, and molecular genetic characteristics.

IODINE-125 PLAQUE BRACHYTHERAPY FOR DIFFUSE CHOROIDAL HEMANGIOMA.

PURPOSE: To report 6 cases of diffuse choroidal hemangioma in children treated with iodine-125 plaque brachytherapy at a single tertiary care center. METHODS: Retrospective case series. RESULTS: Six pediatric patients diagnosed with diffuse choroidal hemangioma were included in the study. Preplaque visual acuity ranged from 20/150 to no light perception. All patients had extensive serous retinal detachment at presentation. An iodine-125 radioactive plaque was placed on the affected eye to administer a dose of 34.2-42.1 Gy to the tumor apex over a median of 4 days. Tumor regression and subretinal fluid resolution were observed in all eyes within 17 months of treatment. Visual acuity improved in two patients. Radiation-induced cataract and subretinal fibrosis were documented in one case, and one patient developed radiation retinopathy. No patients developed neovascular glaucoma within the follow-up time of 12-65 months. CONCLUSION: Iodine-125 plaque radiotherapy is an effective option for diffuse choroidal hemangioma, although there is a risk for radiation-induced complications.

CHOROIDAL OSTEOMA IN AN INFANT.

PURPOSE: To describe a case of choroidal osteoma with macular involvement in an infant that was treated with transpupillary thermotherapy. METHODS: A case report. RESULTS: An 11-month-old infant presented for retinopathy of prematurity follow up examination and was found to have a three disc-diameter round, orange, subretinal, slightly elevated lesion with well-defined margins in the superior macula of the left eye. B-scan ultrasonography showed a slightly elevated, highly reflective choroidal mass with acoustic shadowing. A diagnosis of choroidal osteoma was made. Six months later, repeat examination under anesthesia (EUA) showed the lesion to be increasing in size and encroaching on the fovea. Given demonstrated growth and the risk of central vision loss, the patient was treated with transpupillary thermotherapy (TTT). Subsequent EUAs, over a period of four months, have shown complete decalcification with no subsequent growth. CONCLUSION: Choroidal osteomas are benign lesions that can be sight threatening if located in the macula. TTT in this case resulted in complete regression of a sight threatening extrafoveal choroidal osteoma at four month follow up.

Infusion port site tumor seeding after Pars Plana Vitrectomy-assisted Biopsy of Choroidal Melanoma.

PURPOSE: To report a rare case of infusion port site tumor seeding of choroidal melanoma after pars plana vitrectomy-assisted biopsy and successful treatment with second plaque brachytherapy. METHODS: Observational case report with clinical data and diagnostic images. RESULTS: A 55-year-old Caucasian woman diagnosed with choroidal melanoma of the left eye was found to have a second intraocular tumor thirty months after plaque brachytherapy and pars plana vitrectomy-assisted tumor biopsy. The second melanoma developed at the 5 o'clock vitrectomy infusion port site. The tumor regressed after plaque brachytherapy and the patient has not developed systemic metastasis five years after diagnosis of intraocular seeding. CONCLUSION: Intraocular seeding into the infusion port site, although rare, may be encountered after pars plana vitrectomy-assisted biopsy of choroidal melanoma.

Foreign body giant cell reaction to silicone oil presenting as a salmon-patch conjunctival lesion.

Purpose: To describe a case of foreign body giant cell reaction to silicone oil that presented as a salmon-patch conjunctival lesion. Observations: An elderly female with prior retinal surgery and oil tamponade was referred for a salmon-patch lesion in the conjunctiva. Biopsy revealed multiple vacuolations and foreign body giant cells in the substantia propria, consistent with a foreign body reaction to silicone oil. Conclusion and importance: Silicone oil can elicit an inflammatory reaction in the conjunctiva that could mimic a neoplasm. Excessive leakage of oil into the subconjunctival space should be avoided to prevent this complication.

Intraocular Lens and Lens Capsule Spread of Iridociliary Melanoma: Clinical and Histopathologic Features of 2 Cases.

Purpose: The aim of this study was to describe 2 patients with intraocular lens (IOL) and lens capsule spread of iridociliary melanoma. Methods: Two pseudophakic patients with iridociliary body melanoma that spread onto the surface of their IOL and remaining lens capsule were included. Their eyes were enucleated and the histopathologic features were evaluated. Results: Case 1 was an 82-year-old woman with diffuse primary iridociliary melanoma affecting the iris, lens capsule, IOL surface, and ciliary body. Case 2 was a 68-year-old female who developed melanoma recurrence in the anterior segment after plaque brachytherapy for iridociliary melanoma. The melanoma in both cases grew as a pigmented membrane onto the surface of the IOL. Conclusions: IOL and lens capsule spread of iridociliary melanoma can occur primarily or develop secondarily after plaque brachytherapy of a pseudophakic eye. Since the extent of the melanoma may be uncertain and there is a high likelihood of glaucoma, enucleation is a reasonable option.

Unilateral Xanthocoria and Retinal Vascular Anomalies in a 3-Year-Old Boy: Retinoblastoma or Coats Disease?

The authors describe a challenging case of unilateral retinoblastoma in a patient referred for xanthocoria. A 3-year-old boy was referred for unilateral xanthocoria and disordered retinal vasculature, suggestive of Coats disease. Further investigation revealed diffuse subretinal tumor seeding and areas of calcification, consistent with retinoblastoma. Enucleation was performed and histopathology confirmed exophytic retinoblastoma. This case highlights that xanthocoria, although often encountered in patients with Coats disease, can sometimes be associated with retinoblastoma. As such, retinoblastoma should be considered in the differential diagnosis for children with both leukocoria and xanthocoria. [J Pediatr Ophthalmol Strabismus. 2022;59(X):e32-e34.].

Cell Phone Selfies: Clinching the Diagnosis of Iris Microhemangiomatosis.

Four patients presented with a history of "blood" or a "dark spot" in the eye captured on cell phone photos. These episodes prompted presentation to an ophthalmologist where they all had normal slit lamp exams without a hyphema at initial evaluation. With evidence of a spontaneous hyphema seen on photos, further testing was performed including iris fluorescein angiography which revealed hyperfluorescent iris margin vascular tufts, confirming the diagnosis of iris microhemangiomatosis in each patient. All cases were managed conservatively, and only 1 needed topical antihypertensives. Without these patient-initiated photos, the diagnosis of iris microhemangiomatosis would likely remain elusive as slit lamp exam was normal at the time of initial examination in all 4 cases. Ophthalmologists should be aware of this rare diagnosis in the event a patient comes with a cell phone selfie documenting a spontaneous hyphema, especially when emanating from pupillary border.

Clinical and Histopathological Features of Corneal Primary Acquired Melanosis and Melanoma.

AIM: The aim of this study is to describe the clinical and pathologic features of corneal primary acquired melanosis (PAM) and melanoma. METHODS: We describe 3 cases in total: two cases of corneal melanomas and 1 case of corneal PAM. The eyes were processed routinely for histopathological examination. Clinical histories, treatments, and outcomes were reviewed. RESULTS: Corneal melanomas arose from recurrence of conjunctival melanoma or conjunctival PAM at the limbus. One patient had a recurrence after excision of a limbal melanoma, another had a de novo corneal melanoma, and the last patient had corneal PAM in the setting of conjunctival PAM with atypia. All lesions were excised with adjuvant alcohol debridement and cryotherapy with no recurrence ranging from 1 week to 8 years. CONCLUSIONS: Corneal melanomas arise at the limbus from corneal PAM or conjunctival atypia. They can appear after excisional removal of a conjunctival melanoma. Surgical excision with alcohol debridement and adjuvant cryotherapy is successful.

Secondary ocular malignancies: A review.

Secondary ocular malignancies most commonly spread to the choroid. Previously, the prognosis was poor however, with newer treatments including immunotherapy, patient's life expectancy have increased. It is therefore, important that ophthalmologists diagnose this condition in a timely manner and offer treatment to maximize visual potential and refer them on to oncology colleagues in order to optimize their systemic treatment for their primary cancer.

Approach to Cataract Surgery in an Ebola Virus Disease Survivor with Prior Ocular Viral Persistence.

A 46-year-old patient with previously documented Ebola virus persistence in his ocular fluid, associated with severe panuveitis, developed a visually significant cataract. A multidisciplinary approach was taken to prevent and control infection. Ebola virus persistence was assessed before and during the operation to provide safe, vision-restorative phacoemulsification surgery.

Treatment of presumed Nocardia endophthalmitis and subretinal abscess with serial intravitreal amikacin injections and pars plana vitrectomy.

A 64-year-old man with a past medical history of liver transplantation on chronic immunosuppressive therapy presented with gradual worsening of vision over 2 months in his right eye. His recent history of Aspergillus and Nocardia pneumonia with positive bronchoalveolar lavage, in concert with vitritis and subretinal abscess, were concerning for endogenous endophthalmitis. A sputum culture and transbronchial lung biopsy stains grew Nocardia farcinica although aqueous humor sampling was negative. He was treated with four serial amikacin intravitreal injections over the course of 4 weeks. Pars plana vitrectomy for worsening macular traction and subsequent cataract surgery resulted in significant clinical and anatomic improvement of vision to 20/60 and consolidation of the subretinal abscess.

Intravitreous Cutaneous Metastatic Melanoma in the Era of Checkpoint Inhibition: Unmasking and Masquerading.

PURPOSE: Cutaneous melanoma metastatic to the vitreous is very rare. This study investigated the clinical findings, treatment, and outcome of patients with metastatic cutaneous melanoma to the vitreous. Most patients received checkpoint inhibition for the treatment of systemic disease, and the significance of this was explored. DESIGN: Multicenter, retrospective cohort study. PARTICIPANTS: Fourteen eyes of 11 patients with metastatic cutaneous melanoma to the vitreous. METHODS: Clinical records, including fundus photography and ultrasound results, were reviewed retrospectively, and relevant data were recorded for each patient eye. MAIN OUTCOME MEASURES: Clinical features at presentation, ophthalmic and systemic treatments, and outcomes. RESULTS: The median age at presentation of ophthalmic disease was 66 years (range, 23-88 years), and the median follow-up from diagnosis of ophthalmic disease was 23 months. Ten of 11 patients were treated with immune checkpoint inhibition at some point in the treatment course. The median time from starting immunotherapy to ocular symptoms was 17 months (range, 4.5-38 months). Half of eyes demonstrated amelanotic vitreous debris. Five eyes demonstrated elevated intraocular pressure, and 4 eyes demonstrated a retinal detachment. Six patients showed metastatic disease in the central nervous system. Ophthalmic treatment included external beam radiation (30-40 Gy) in 6 eyes, intravitreous melphalan (10-20 µg) in 4 eyes, enucleation of 1 eye, and local observation while receiving systemic treatment in 2 eyes. Three eyes received intravitreous bevacizumab for neovascularization. The final Snellen visual acuity ranged from 20/20 to no light perception. CONCLUSIONS: The differential diagnosis of vitreous debris in the context of metastatic cutaneous melanoma includes intravitreal metastasis, and this seems to be particularly apparent during this era of treatment with checkpoint inhibition. External beam radiation, intravitreous melphalan, and systemic checkpoint inhibition can be used in the treatment of ophthalmic disease. Neovascular glaucoma and retinal detachments may occur, and most eyes show poor visual potential. Approximately one quarter of patients demonstrated ocular disease that preceded central nervous system metastasis. Patients with visual symptoms or vitreous debris in the context of metastatic cutaneous melanoma would benefit from evaluation by an ophthalmic oncologist.

Trends in Radiation Practices for Female Ocular Oncologists in North America: A Collaborative Study of the International Society of Ocular Oncology.

BACKGROUND: The aim of this study was to determine the known radiation exposure, attitudes, and consequent risk modifications among female ocular oncologists in North America who routinely administer radioactive plaque brachytherapy treatment and are members of the International Society of Ocular Oncology. METHODS: Nineteen female ocular oncologists completed an anonymous 17-question radiation exposure survey. RESULTS: Eleven of the participants chose to routinely wear lead protection during surgery; 8 did not. Fifteen of 19 participants reported using an unloaded "nonactive" template to prepare for plaque implantation. During pregnancy, 11 of 13 participants continued to perform plaque brachytherapy. Eight of these 11 undertook measures to decrease radiation exposure self-reported as lead wear and other. The average reported anxiety regarding fertility was 2.1 (SD, 2.2) on a scale from 1 to 10. CONCLUSION: This study corroborates prior literature that surgeons' exposure to radiation during plaque brachytherapy is minimal. Nonetheless, there remains some anxiety regarding exposure risk to women, due to potential effects on fertility and fetal health. We found variability in exposure monitoring, required training, and precautions during pregnancy amongst this group of surgeons. Improved education and clearer pregnancy guidelines may equip female ocular oncologists with optimal knowledge regarding risk of radiation exposure.

Scleral Thinning after Transscleral Biopsy for Uveal Melanoma Using Lamellar Scleral Flap.

PURPOSE: The purpose of the study is to describe the clinical history and histopathologic findings of three cases of scleral thinning after lamellar scleral flap, including one case with confirmed extraocular tumor extension. METHODS: The medical records and pathology specimens of three patients with scleral thinning after biopsy and plaque brachytherapy and lamellar scleral flap performed during a transscleral biopsy were reviewed. RESULTS: The first two patients developed scleral thinning and visible pigmentation, but had tumors that were regressing in size on ultrasound. The two patients were followed by serial observation. The third patient exhibited scleral thinning and evidence of tumor growth on ultrasound, raising the suspicion for extraocular tumor extension. Histopathologic examination of the enucleated eye confirmed extrascleral tumor extension and showed necrotic and intact melanoma with associated pigmented macrophages. CONCLUSIONS: Patients with scleral flaps created for biopsy of uveal melanoma are at risk for scleral thinning and extrascleral extension of tumor recurrence through the flap.

Uveal Melanoma Nuclear BRCA1-Associated Protein-1 Immunoreactivity Is an Indicator of Metastasis.

PURPOSE: To examine the BRCA1-associated protein-1 (BAP1) expression of primary uveal melanomas without and with metastasis, and to analyze the correlation between the BAP1 immunoreactivity of primary uveal melanoma and other clinicopathologic features. DESIGN: Retrospective case series. PARTICIPANTS: Forty patients with uveal melanoma (mean age, 57.98±14.75 years) were included in this analysis, of whom 20 had no metastatic disease and 20 had metastasis. METHODS: Medical records and histology slides of patients with primary uveal melanoma treated by enucleation were reviewed. BAP1 expression was evaluated by immunohistochemical staining of formalin-fixed, paraffin-embedded sections. Immunoreactivity in the nucleus and cytoplasm were graded by estimating the percentage of primary tumor cells showing a positive staining of their nucleus or cytoplasm per 1 high-power field 200× (grades 0-3). MAIN OUTCOME MEASURES: Tumor size, histologic features, nuclear and cytoplasmic BAP1 immunoreactivity grade, and patient outcome, including development of metastasis. RESULTS: Significantly lower (P = 0.025) nuclear BAP1 immunoreactivity was observed in the metastatic melanoma group. Greater tumor thickness, basal diameter, and more advanced TNM stage were associated with an increased odds ratio of developing metastasis (P < 0.05). In addition, tumors with a higher proportion of cells expressing nuclear BAP1 had decreased odds of developing metastatic disease in a multivariate model (P = 0.042). Metastasis-free survival was significantly longer in patients with uveal melanoma with high nuclear BAP1 stain (P = 0.004). CONCLUSIONS: Time to metastasis differs in patients with primary uveal melanoma with different grades of nuclear BAP1 immunoreactivity. Nuclear BAP1 stain is the only significant independent predictor of metastatic disease in this study. Our data support the role of BAP1 immunohistochemical staining of primary uveal melanoma to evaluate metastatic risk.