Clinical characteristics and treatment outcomes of patients with IgG4-positive ocular adnexal marginal zone B-cell lymphoma

Abstract

Purpose: To explore the clinicopathological characteristics of immunoglobulin G4 (IgG4)-positive ocular adnexal marginal zone B-cell lymphoma (OAML) and associated patient treatment outcomes.

Methods: Medical records from patients diagnosed with IgG4-positive OAML treated at the West China Hospital between January 2016 and August 2023 were retrospectively analyzed.

Results: This study included data from 22 patients (11 males, 11 females), aged between 36 and 83 years, with disease durations from 1 month to 30 years. Sixteen cases exhibited unilateral ocular involvement (ten left eyes, six right eyes), while six exhibited bilateral involvement. Common clinical symptoms included ocular masses, eyelid swelling, and proptosis, with the orbit and lacrimal gland being the most commonly impacted sites. Among the 22 patients, 13 who were clinically suspected of having IgG4-related ophthalmic disease (IgG4-ROD) underwent serum IgG4 testing pre-operatively, revealing elevated IgG4 levels in 11 of these patients. The use of computed tomography and magnetic resonance imaging facilitated the evaluation of the location and size of lesions. All 22 patients received surgical treatment. Subsequently, 14 of these patients underwent local radiotherapy, five received post-operative chemotherapy, and three were closely observed. The follow-up period of patients in this study was 3-77 months, with an average follow-up time of 36 months. Except for one patient who died of disease progression, all others showed favorable prognoses with significant improvements.

Conclusions: These results support the classification of IgG4-positive OAML as a distinct OAML sub-type with clinical features that partially overlap with IgG4-ROD. Therefore, accurate differentiation between OAML and IgG4-ROD is imperative, necessitating timely surgical intervention and precise pathological diagnosis to prevent diagnostic errors and inappropriate treatment. Currently, no standardized treatments for IgG4-positive OAML exist, but our results suggest that standard OAML therapies are generally efficacious.

Figure 1

Orbital CT scans of IgG4-positive OAML in patient 5. (a) Axial CT images reveal bilateral orbital tumors (arrow) located inside the muscle cone. (b) Axial CT-enhanced images reveal moderate tumor enhancement (arrow) visible inside the orbits of both eyes. (c) Conical CT images reveal bilateral orbital tumors (arrow), with the tumor in the right eye being located at the bottom surrounding the inferior rectus muscle, while the tumor in the left eye is located at the lower outer corner. (d) Coronal CT-enhanced images suggest uniform tumor enhancement (arrow) in the orbits of both eyes, with signal levels comparable to that of the extraocular muscles

Figure 2

Orbital MRI images of IgG4-positive OAML in patient 8. (a) T1-weighted (T1W) images suggest the location of an orbital tumor (arrow) in the left eye in the lacrimal gland area and surrounding the optic nerve inside the orbital muscle cone, presenting with a low signal level. (b) T2-weighted images reveal the iso intensity of the orbital tumor (arrow) signal in the left eye with that of the extraocular muscle. (c, d) Axial and coronal T1W fat suppression enhanced images suggesting the uniform enhancement of the left-sided orbital tumor (arrow) with signal levels comparable to those for the extraocular muscle

Figure 3

Histological and immunohistochemical analysis of IgG4-positive OAML in patient 8. (a) Gross tumor tissue. (b) Histological H and E staining revealing dense lymphocytic proliferation with sclerosis (Magnification: 400x). (c) IgG4 immunostaining reveals the presence of IgG4-positive plasma cells in the lymphoplasmacytic lesions (Magnification: 400x). (d) Representative Ki-67 staining results for biopsy tissue sections (Magnification: 400x)

Figure 4

Kaplan–Meier survival analysis of IgG4-positive OAML patients in the study cohort. The PFS rate was 95%