Renelle Pointdujour-Lim

Purpose: To review the demographics, clinical features, and response of orbital squamous cell carcinoma treated with cemiplimab. Methods: This is a retrospective multi-institutional series. Patient characteristics, drug dosing, duration, and response to treatment were evaluated. Results: The study cohort consisted of 11 patients from 5 institutions. All patients received a regimen of 350 mg q 3 weeks and an average of 11.2 cycles (SD 5.8). No patient experienced significant side effects requiring treatment or cessation of cemiplimab. Complete response was achieved in 9 patients (82%) treated with cemiplimab. Conclusions: Immune checkpoint inhibitors, such as cemiplimab provide a globe-sparing option for the treatment of orbital squamous cell carcinoma. It is important to consider these agents especially when orbital exenteration is the alternative.

Samter's triad, also known as aspirin-exacerbated respiratory disease, is characterized by nasal polyposis, bronchial asthma, and aspirin intolerance. Here, we present a case of a 36-year-old woman with a history of Samter's triad and recurrent dacryocystitis. After combined dacryocystorhinostomy and endoscopic sinus surgery, pathological specimens of the lacrimal sac showed respiratory fibrosis with chronic inflammation and eosinophilic infiltration. Our case demonstrates that Samter's triad is a potential etiology for inflammatory nasolacrimal duct obstruction.

Objective To demonstrate the spectrum of autoimmune retinopathy (AIR) associated with immunotherapy for advanced cutaneous melanoma. Methods and analysis Retrospective chart review on patients with advanced cutaneous melanoma who developed AIR after initiating immunotherapy. Complete ophthalmic examination and relevant ancillary testing were performed on each patient. The presence of AIR-associated anti-retinal antibodies was confirmed by western blot and/or immunohistochemical staining. Ophthalmic and systemic outcomes after treatment for AIR were followed over time. A systematic review of AIR associated with immunotherapy for cutaneous or non-ocular mucosal melanoma was carried out in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Results Case 1 developed photopsia and nyctalopia with electroretinographic findings characteristic for melanoma-associated retinopathy 1 week after initiating ipilimumab/nivolumab immunotherapy. Case 2 experienced new severe bilateral visual field loss associated with anti-retinal and anti-optic nerve antibodies while on maintenance nivolumab immunotherapy. Case 3 developed decreased visual acuity due to acute exudative polymorphous vitelliform maculopathy within 2 weeks of initiating ipilimumab/nivolumab immunotherapy. All patients had concurrent extraocular immune-related adverse events in addition to the presence of anti-retinal antibodies on serological testing. 14 published cases of AIR associated with immunotherapy for cutaneous or non-ocular mucosal melanoma were identified and reviewed. Conclusions Immune checkpoint inhibition can trigger the development of AIR with varied clinical manifestations in patients with advanced cutaneous melanoma. This study highlights the need for close monitoring in cutaneous melanoma patients receiving immunotherapy who develop new visual symptoms with or without funduscopic changes, as well as the potential role for screening of patients prior to initiating immunotherapy.

Background: Uveal melanoma is the most common primary intraocular malignancy in adults, often resulting in painless vision loss. We report a case of necrotic uveal melanoma presenting with orbital inflammation mimicking orbital cellulitis and present a comprehensive review of the literature and tabulation of reported cases. Summary: Our review found 44 published reports of spontaneously necrotic uveal melanoma involving 55 patients. Of these reports, 26 patients (47%) presented with orbital cellulitis. Presenting symptoms of necrotic uveal melanoma with orbital cellulitis included proptosis (82.8%), pain (80.7%), vision loss (61.5%), and restricted extraocular movements (46.2%). Key Messages: Uveal melanoma can rarely mimic orbital cellulitis. Autoinfarction and tumor necrosis causes secondary orbital inflammation. Intraocular malignancy must remain in the differential for patients with orbital inflammation and vision loss.

Uveal melanoma is the most common primary intraocular tumor in adults and can have varying presentations, although it is frequently asymptomatic. One rare presentation of uveal melanoma is neovascular glaucoma (NVG). We present a case of a 20-year-old male who presented with 2 weeks of left eye redness and decreased vision who was found to have NVG. He was referred for evaluation of glaucoma. Fundoscopic and ultrasonographic examination revealed a mushroom-shaped choroidal mass with low internal reflectivity consistent with choroidal melanoma. The patient underwent enucleation, and metastatic workup revealed hepatic metastases. The patient died 18 months after initial diagnosis. This case emphasizes the importance of a complete ophthalmic evaluation in cases presenting with NVG of unclear etiology to exclude the presence of a potentially life-threatening intraocular tumor.

Although immune checkpoint inhibitors have revolutionized the treatment of cancers, their role in the pathogenesis of chronic infections remains unclear. Here, we present the case of a patient on a cocktail of checkpoint inhibitors who presented with severe bilateral gonococcal conjunctivitis, orbital cellulitis and vitritis, without a history of genital infection.

Purpose: To describe the demographics, clinical presentation, treatment, and outcomes of a rare cohort with simultaneous orbital and intracranial abscesses. Methods: A historical cohort study of 17 patients with simultaneous orbital and intracranial abscesses between 2010 and 2018 was performed. The demographics, location of abscesses, treatment, and outcomes of these patients were analyzed. Results: The mean age was 26.9 years (range 5–83 years). Fourteen patients (82%) were male. In this cohort, the most common orbital abscess location was the superior orbit, involved in 14 patients (82%). The most common site of intracranial abscess was the frontal lobe, involved in 16 patients (94%). Concurrent sinus disease was present in 16 patients (94%). Surgical evacuation was the standard of treatment, with 94% of patients undergoing at least one surgical procedure. Streptococcus species were the most common, isolated from 6 sinus cultures (43%), 3 orbitotomy cultures (21%), and 4 craniectomy cultures (36%). Staphylococcus species were also common. Most patients (94%) had stable or improved mental status and visual function at the conclusion of their treatment. Conclusions: Simultaneous orbital and intracranial abscesses are rare. Local invasion from the orbit into the intracranial space may occur from direct spread, thus superior orbital abscesses pose the greatest risk for intracranial spread. Additional factors such as infection with Streptococcus and Staphylococcus species as well as male sex appear to be risk factors for intracranial spread. For those who develop intracranial abscesses, young age and absence of seizures or altered mental status at presentation may be associated with favorable outcomes.

Purpose: We performed an epidemiological study of orbital lymphoma in the United States to determine how histological subtypes confer differing prognosis, and understand other factors associated with survival. Methods: All patients in the Surveillance, Epidemiology and End Results database diagnosed with a histologically confirmed orbital lymphoma between 1973 and 2014 were included. Exclusion criteria included diagnosis at autopsy and the presence of other malignancies. Measures included patient demographic information, histological subtype and treatment modalities. Outcomes included overall and disease specific survival. Results: Of the 1504 cases identified, 702 were male (46.7%, mean age: 64.4 years, standard deviation [SD]: 15.3) and 802 were female (53.3%, mean age: 67.5 years, SD: 14.3). Mucosal associated lymphoid tissue (MALT) (49.5%) and diffuse large B cell lymphoma (DLBCL) (19.5%) were the two most common histologic subtypes. MALT lymphoma conferred the best prognosis (10-year cancer specific survival [CSS] 90.2%, 95% Confidence Interval [CI] 87.4% – 93.1%) and DLBCL conferred the worst prognosis (10-year CSS 68.6%, 95% CI 62.5% – 75.3%) (p < .001, log-rank test). Older age (Hazard Ratio [HR]: 3.71, 95% Confidence Interval [CI]: 2.94–4.66, p < .001), male sex (HR: 1.22, 95% CI: 1.039–1.441, p = .015), no radiation (HR: 1.72, 95% CI: 1.46–2.02, p < .001) and DLBCL histology were significant predictors of worse overall survival. Conclusions: DLBCL histology confers the worst outcomes whereas MALT lymphoma confers the best outcome in orbital lymphoma. Age, gender, and radiation treatment also influence survival. These epidemiological results can be used clinically to communicate outcomes on the basis of patient characteristics and disease histology.

Background: Conjunctival melanoma is a potentially lethal malignancy of the ocular surface. There have been no therapeutic advancements made in the past several decades despite increasing prevalence of the disease. Methods: The authors report the case of a 52-year-old Caucasian male with unresectable, recurrent conjunctival melanoma with V600 BRAF mutation who was treated with systemic BRAF/MEK inhibition. Results: There was complete regression of local disease within the first 9 months. The patient remains without local recurrence or systemic metastasis at 1 year. Conclusion: This is the first reported case of conjunctival melanoma with complete response to BRAF/MEK inhibition. As long as targeted therapy remains an option, patients with conjunctival melanoma should undergo mutational profiling of their tumor.