This study analyzed B-cell heterogeneity in lacrimal gland tissues from 4 patients with IgG4-related ophthalmic disease (IgG4-ROD) and 2 patients with IgG4-positive MALT lymphoma using single-cell transcriptome sequencing (scRNA-seq). The results revealed that while the B-cell differentiation trajectories have similarities between the two diseases, critical differences were evident. IgG4-ROD was predominantly composed of naïve B cells, with memory B cells mainly being the central memory type. These cells were enriched in hormone/innate immunity pathways, and the plasma cells exhibited features of functional exhaustion. Conversely, MALT lymphoma was dominated by memory B cells, particularly enriched in terminally differentiated subtypes, with aberrant activation of oncogenic pathways (BCR/NF-κB). In MALT lymphoma, naïve B cells showed upregulated expression of immunoglobulin genes (IGHG3/IGHG4) and abnormal activation of EBV/BCR/T-cell differentiation pathways, despite having suppressed basal metabolism. Germinal center-like B cells in MALT lymphoma revealed upregulated gene expression enriched in T-cell activation and PD-1/PD-L1 pathways. Plasma cells in MALT lymphoma displayed monoclonal expansion (high expression of IGHG2/IGHG3/IGHG4) and enhanced antibody secretion. Therefore, MALT lymphoma is characterized by abnormal activation and malignant transformation of B cells, whereas IgG4-ROD manifests as functional exhaustion and metabolic suppression. The differentiation state of memory B cells may represent a critical juncture for malignant transformation.

AimThis study aimed to characterize the clinical presentation, pathological features, and prognostic indicators of ocular mucoepidermoid carcinoma (MEC) based on an institutional cohort and a systematic literature review.MethodsA retrospective analysis was conducted including two distinct datasets: six patients with histopathologically confirmed ocular MEC treated at our institution and twenty-one cases identified through literature review. Institutional cases were evaluated for clinical course, treatment, and recurrence, whereas literature-derived cases were summarized descriptively due to reporting heterogeneity and incomplete follow-up.ResultsPatients in the institutional cohort (n = 6) had a median age of 64 years, with no sex predominance. The lacrimal gland was the most frequent primary site, followed by the eyelid and lacrimal sac. The predominant manifestation was a painless orbital mass, while diplopia and visual loss were less frequent. Intermediate-grade lesions were most common, and orbital tissue invasion was histologically confirmed in two patients. Both patients with invasion experienced tumor recurrence, whereas no recurrence was observed among patients without invasion during follow-u No disease-related deaths occurred within this cohort. The literature-derived group demonstrated substantial variability in grading, invasion patterns, and follow-up duration, reflecting selective reporting toward advanced or recurrent cases.ConclusionsOcular MEC demonstrates a high tendency toward local recurrence but generally low disease-specific mortality. Orbital tissue invasion appears to be associated with an elevated risk of recurrence in the institutional cohort, underscoring the need for prolonged surveillance. Given the marked heterogeneity of published cases, survival inference should be limited to consistently followed institutional data.

OBJECTIVE:To explore the role of serum IgE in IgG4-related ophthalmic disease (IgG4-ROD) by comparing clinical features and prognosis of patients with elevated and normal serum IgE levels. METHODS:This single-centre retrospective study included 54 IgG4-ROD patients diagnosed by histopathology from March 2022 to March 2024. Clinical features of the IgE+ group (serum IgE levels >165 IU/ml) and the IgE- group (serum IgE levels ≤ 165 IU/ml) were compared, and 50 patients were followed for at least 6 months to assess the prognosis. Statistical analyses included t tests, Mann-Whitney U, chi-square tests, Spearman correlation, Kaplan-Meier survival curves, and Cox regression. RESULTS:Among the 54 patients, both the IgE+ (n = 22) and IgE- groups (n = 32) were predominantly middle-aged women. Extraocular muscle involvement was more common in the IgE- group (P = 0.033), whereas bilateral ocular (P = 0.040) and sinus involvement (P = 0.046) were more frequent in the IgE+ group. Significant intergroup differences were observed in serum IgG4 (P = 0.004), C1q (P = 0.021), IL-10 levels (P = 0.030), IgG4/IgG (P = 0.022), and IgE/IgG4 ratios (P = 0.018). Serum IgE correlated positively with IgG4, IL-6, IL-10, and IL-12p70, and negatively with C1q and IgM (P < 0.05). The IgE+ group had a higher relapse risk (P = 0.038). Cox regression analysis identified serum IgE as an independent risk factor for relapse (P = 0.022). CONCLUSION:Elevated serum IgE in IgG4-ROD is associated with more severe disease and higher relapse risk, highlighting its potential as a prognostic marker.

AIM: To investigate the clinical features and prognosis of patients with orbital inflammatory myofibroblastic tumor (IMT). METHODS: This retrospective study collected clinical data from 22 patients diagnosed with orbital IMT based on histopathological examination. The patients were followed up to assess their prognosis. Clinical data from patients, including age, gender, course of disease, past medical history, primary symptoms, ophthalmologic examination findings, general condition, as well as imaging, laboratory, histopathological, and immunohistochemical results from digital records were collected. Orbital magnetic resonance imaging (MRI) and(or) computed tomography (CT) scans were performed to assess bone destruction of the mass, invasion of surrounding tissues, and any inflammatory changes in periorbital areas. RESULTS: The mean age of patients with orbital IMT was 28.24±3.30y, with a male-to-female ratio of 1.2:1. Main clinical manifestations were proptosis, blurred vision, palpable mass, and pain. Bone destruction and surrounding tissue invasion occurred in 72.73% and 54.55% of cases, respectively. Inflammatory changes in the periorbital site were observed in 77.27% of the patients. Hematoxylin and eosin staining showed proliferation of fibroblasts and myofibroblasts, accompanied by infiltration of lymphocytes and plasma cells. Immunohistochemical staining revealed that smooth muscle actin (SMA) and vimentin were ‌positive in 100%‌ of cases, ‌while‌ anaplastic lymphoma kinase (ALK) showed ‌positivity in 47.37%‌. The recurrence rate of orbital IMT was 27.27%, and sarcomatous degeneration could occur. There were no significant correlations between recurrence and factors such as age, gender, laterality, duration of the disease, periorbital tissue invasion, bone destruction, periorbital inflammation, tumor size, fever, leukocytosis, or treatment (P>0.05). However, lymphadenopathy and a Ki-67 index of 10% or higher may be risk factors for recurrence (P=0.046; P=0.023). CONCLUSION: Orbital IMT is a locally invasive disease that may recur or lead to sarcomatoid degeneration, primarily affecting young and middle-aged patients. The presence of lymphadenopathy and a Ki-67 index of 10% or higher may signify a poor prognosis.

Orbital mucosa-associated lymphoid tissue (MALT) marginal zone lymphoma is the most common subtype of ocular adnexal lymphoma. In elderly patients, it typically presents as a slowly enlarging, painless mass. Giant lesions (> 5 cm) are extremely rare. This article reports a rare case of giant orbital MALT lymphoma in a 72-year-old woman and reviews similar cases in the literature. The patient was a 72-year-old Asian woman with a left orbital mass that had been slowly enlarging for 6 years and a 4-year history of hypertension. Ophthalmic examination revealed a large, soft, dark-red mass in the left orbit protruding beyond the orbital rim, accompanied by vascular dilatation and congestion of the eyelid skin, complete ptosis, difficulty opening the eye, severe conjunctival chemosis, extensive corneal neovascularization, and sluggish pupillary light reflex. MRI demonstrated a mass measuring 7.4 cm × 6.2 cm × 4.9 cm involving the orbital apex. The patient underwent resection of the left orbital lesion. Postoperative pathology confirmed MALT lymphoma with CD20(+), CD79α(+), Bcl-2(+), and Ki-67 ≈ 20%. The surgical wound healed well. At 3-month follow-up, the patient declined adjuvant radiotherapy and has remained recurrence-free on regular monitoring. This case demonstrates that combined imaging and histopathological evaluation is essential for the definitive diagnosis of giant orbital MALT lymphoma. The patient’s delayed presentation led to significant disease progression, highlighting the need to strengthen health education, promote early diagnosis and treatment in elderly patients, and prevent diagnostic delays.

Abstract Background Primary high-grade lacrimal gland (LG) mucoepidermoid carcinoma (MEC) is a rare condition that presents diagnostic and treatment challenges. In this study, we aimed to elucidate the diagnosis, treatment, and mutational landscape of high-grade LG MEC. Methods We reviewed clinical symptoms, radiological images, treatment, prognosis, histopathology, and mutational landscape of 20 patients with high-grade LG MEC. Results Primary clinical presentation of high-grade LG MEC was proptosis (85%) with a low incidence of ocular pain (20%). CT scans showed frequent bone destruction (89.5%) and calcification (73.7%). MRI revealed hypointense areas on T2-weighted imaging (WI) in 85% of lesions and non-enhanced areas on contrast-enhanced T1WI in 95% of cases. All 20 patients with primary high-grade LG MEC underwent surgery and were stratified into 5 treatment groups. Multi-group analysis showed only the complete resection plus adjuvant radiotherapy group had significantly better RFS than the complete resection alone group (both P = 0.0016), with no significant OS differences across groups. The rates of recurrence, metastasis, and mortality for patients in T4 stage were 77.8%, 71.4%, and 100%, respectively, while those in stages T1-3 were 25%, 75%, and 62.5%. Log-rank tests demonstrated superior long-term RFS and OS in T1–3 patients (P < 0.05), whereas Gehan-Breslow-Wilcoxon tests yielded non-significant results. Whole-exome sequencing of tumors and adjacent normal tissues from 3 patients showed TP53 as the only consistent driver gene with missense mutations in all samples. Additionally, EGFR, DOCK2, GAN11, AKAP9, and SMC4 were identified as driver genes in 2 patients. Conclusions High-grade LG MEC predominantly manifested as painless exophthalmos and the characteristic imaging included calcification, bone destruction, hypointense areas on T2WI, and non-enhanced areas on contrast-enhanced T1WI. Adjuvant radiotherapy after complete resection reduced recurrence and improved RFS but did not significantly elevate the overall survival rate of primary high-grade LG MEC patients. Prognosis is especially poor for T4 stage. Potential somatic variants identified within this small exploratory cohort, including TP53, EGFR, DOCK2, GAN11, AKAP9, and SMC4 may represent the potential therapeutic targets. Nevertheless, their clinical and prognostic relevance requires rigorous validation in larger independent patient cohorts.

Kimura’s disease (KD) is a rare chronic inflammatory disorder that predominantly affects young Asian males and typically involves the head and neck region. Orbital involvement is uncommon and remains insufficiently characterized, particularly with respect to long-term outcomes. This study aimed to investigate the clinical features, imaging characteristics, treatment strategies, and long-term prognosis of orbital KD. We conducted a retrospective case series of patients with histopathologically confirmed KD involving the orbit at a single tertiary referral center between January 2010 and December 2025. Demographic characteristics, clinical manifestations, imaging findings, pathological features, treatment modalities, and follow-up outcomes were reviewed. For suspected recurrence, recurrent clinical manifestations were first identified during follow-up, and orbital imaging was used to confirm lesion recurrence and assess lesion extent when indicated. Pathological confirmation was obtained only when repeat surgery or biopsy was performed. Eight male patients were included, with a mean age of 21.25 ± 17.20 years (range, 6–47 years). Painless eyelid swelling was the most common presenting symptom (62.5

AIM: To analyze the general profile, clinical symptoms, pathological features, and prognostic characteristics of extranodal Rosai-Dorfman disease (RDD) with ocular involvement. METHODS: This was a retrospective series of case study. Clinical data from 35 cases who had extranodal RDD with ocular involvement were collected for analysis, including 5 cases diagnosed at our hospital and 30 reported in the literature which searched via PubMed, the Chinese National Knowledge Infrastructure (CNKI), the Chinese Science and Technology Periodical Database (VIP), and WanFang Data database from database creation to April 2023. Lesion location, clinical presentation, pathological presentation, treatment modality, follow-up time, and prognosis were recorded. RESULTS: Lesions of five cases were located in the orbit, eyelid, lacrimal gland, or conjunctiva. The main presenting features were proptosis, eyelid swelling, and conjunctival hyperemia with decreased vision. Four patients underwent surgical resection, one received surgery and adjunctive immunosuppression, and none experienced recurrence during follow-up. A total of 30 cases were retrieved from the literature. The mean age was 41.4y, and 66.7% were male. The lacrimal gland and conjunctiva/subconjunctiva were the most commonly affected sites (each 20.0%). Most patients received surgical management (50.0%) or immunosuppressive therapy (20.0%). Only one recurrence (3.3%) was reported during follow-up. CONCLUSION: When symptoms like ocular protrusion and visual acuity loss occur, RDD should be considered in the differential diagnosis. The diagnosis of RDD primarily depends on pathological histology, which serves as the key basis for confirmation. Although RDD generally has a favorable prognosis, long-term follow-up of patients is still essential to closely monitor for potential recurrence.

·Nonspecific orbital inflammation(NSOI)is an orbital inflammation that is not associated with an infection.Even though it's often considered the most common diagnosis in orbital biopsies,it's still an exclusionary diagnosis that means systemic illnesses and other possible causes have to be ruled out.Though it is always an excluded clinical diagnosis,acute orbital symptoms such discomfort,exophthalmos,periorbital edema,chemosis,diplopia,and vision impairment are commonly associated with NSOI.Clinical diagnosis and management of NSOI provide a substantial difficulty.There are presently no recognized diagnostic criteria or standard treatment strategy for NSOI,and the clinical symptoms and histological features show significant variation.This guide was formulated under the auspices of the Ocular Oncology Committee of the Opthalmology Branch of the Chinese Medical Doctor Association,Opthalmology Committee of International Association of Intelligent Medicine,Opthalmology Committee of International Association of Translational Medicine making a detailed summary of the definition,classification,diagnosis and treatment of the NSOI,with a view to aiding clinicians to improve diagnostic efficiency and formulate a better treatment plan for patients.