Objective: IgG4-Related Orbital Disease (IgG4-ROD) is an incompletely understood differential of idiopathic orbital inflammatory syndrome (IOIS). Accurate separation guides therapy and prognosis. This retrospective study also profiles its clinical features, therapy needs, and compares them with IOIS. Methodology: Fifty-four patients were biopsied between January 2016 and December 2023, comprising 18 who were diagnosed with IgG4-ROD (4 definite, 14 probable) and 36 with IOIS. Mean follow-up was 21.7 ± 26.2 months for IgG4-ROD versus 7.5 ± 10.3 months for IOIS. Results: Patients with IgG4-ROD were older than those with IOIS (mean 61.8 vs. 49.9 years). Gender distribution was balanced. The lacrimal gland (66.7% vs. 61.6%; p = 0.690) and extra-ocular muscles (55.6% vs. 30.6%; p = 0.076) were frequently involved in both entities, whereas paranasal sinus infiltration was significantly associated with IgG4-ROD (p = 0.003). Common shared symptoms (p > 0.05) included eyelid swelling (83.3% vs. 86.1%), exophthalmos (50% vs. 36.1%), and motility restriction (22.2% vs. 25%). Relative afferent pupillary defect (p = 0.042), chemosis (p = 0.02), and systemic disease (p = 0.005) were more prevalent in IgG4-ROD. During ≥ 6-month follow-up (n = 7), only 28.6% of IgG4-ROD patients achieved sustained remission; Kaplan–Meier analysis yielded a mean time to first event of 926 days. Additional steroid-sparing therapy was required more often in IgG4-ROD than in IOIS (p = 0.002). Conclusion: IgG4-ROD and IOIS share clinical features but differ in key aspects such as associated diseases, therapy requirements, and disease control. Understanding these differences is crucial for targeted diagnostics and individualized treatment strategies.

Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous malignancy with substantial risks of mortality and vision-threatening morbidity when periocular. We aimed to characterize the clinicopathologic features, management strategies, ophthalmic outcomes, and survival of periocular MCC. We conducted a retrospective cohort study of 18 periocular MCC cases treated at a tertiary center between January12010and February 1 2024. Demographic and tumor characteristics, treatment details, ocular complications, and survival endpoints were extracted from electronic records. Kaplan–Meier curves estimated overall survival (OS) and progression-free survival (PFS). Eighteen patients (61% female; mean age ± SD, 71.6 ± 11.4 years) were included; 67% presented with upper-eyelid disease. Half of tumors were AJCC stage I (50.0%). Initial therapy was excision alone in 33%, excision plus radiation in 28%, and multimodal in the remainder; an average of 1.62 resections was required to achieve negative margins or functional reconstruction. Functional globe preservation was achieved in 78% of patients, with severe ocular complications in 11%. Median OS was 8.5 years (95% CI, 4.6–12.3) and the estimated 2-year OS rate was 88.9%. Median PFS was 6.4 years (95% CI, 0.1–12.8) with a 2-year PFS of 54.7%. Median follow-up was 17 months. Periocular MCC in this single-center cohort was typically detected early and managed with eye-sparing surgery with acceptable morbidity, yielding favorable OS and PFS estimates; these site-specific benchmarks can guide counseling, reconstruction, and future multicenter validation.

Introduction: Extranodal natural killer/T-cell lymphoma, nasal type (ENKTL), is a rare and aggressive malignancy. Primary orbital involvement is exceptionally uncommon. We report a case of orbital ENKTL in a White patient presenting with rapidly progressive orbital symptoms. Case Presentation: A 69-year-old woman presented with eyelid swelling, diplopia, and exophthalmos. An initial diagnosis of conjunctivitis by a community ophthalmologist proved unresponsive to treatment, prompting referral to a tertiary eye center. Orbital magnetic resonance imaging revealed a retrobulbar mass. Biopsy confirmed Epstein-Barr virus-associated ENKTL, and staging excluded systemic disease. The patient received age-adjusted modified SMILE (mSMILE) chemotherapy (dexamethasone, methotrexate, ifosfamide, l-asparaginase, etoposide) followed by concurrent platinum-based chemoradiotherapy. She achieved complete remission and reported substantial improvement in quality of life. Conclusions: Even exceptionally rare orbital lesions should be considered in cases of rapidly progressive orbital disease. Early imaging, appropriate biopsy, and multidisciplinary management are essential to achieve optimal outcomes.