Single Institutional Experience on Orbital Inflammatory Pseudotumor: Diagnostic and Management Challenge

Abstract

Aims: Orbital inflammatory pseudotumor is considered a non-neoplastic inflammatory process. The finding of clonality of B or T-cell receptors in cases pathologically diagnosed as orbital inflammatory pseudotumor has unknown clinicopathologic significance. We sought to investigate potential B and T-cell clonality and concomitant diseases in cases pathologically diagnosed as orbital inflammatory pseudotumor. Methods: Cases diagnosed as orbital inflammatory pseudotumor at our institution were retrospectively analyzed. Hematoxylin and eosin-stained slides, immunohistochemically stained slides and polymerase chain reactions on cell block material for the investigation of clonality of B and T-cell receptors were evaluated, to confirm the diagnosis and investigate the prevalence of concomitant diseases. Results: A total of 13 cases showing characteristic histopathologic features of orbital inflammatory pseudotumor were identified. CD138, IgG, and IgG4 showed varying numbers of plasma cells in each case, with 5 cases (5/13, 38%) exhibiting relative increase in the presence of IgG4 plasma cells. However, no cases showed diagnostic findings of IgG4-related disease (IgG4-RD). polymerase chain reactions analysis showed clonal B-cell populations in 2 cases (2/13, 15%). No cases showed anaplastic lymphoma kinase expression by immunohistochemistry. There were no clinical reports of progression to lymphoma or development of systemic IgG4-RD in any of the patients (average follow-up of 300 days), with 38% of patients showing systemic autoimmune conditions. Conclusion: A small but significant percentage of typical orbital inflammatory pseudotumor on histology showed B-cell clonality on polymerase chain reactions analysis of B-cell receptors, or features suggestive, but not diagnostic of IgG4-RD. Close follow-up of these patients to identify development of lymphoma, systemic IgG4-RD, or other rheumatologic conditions may be clinically warranted.

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors

“…Contrast‐enhanced MRI with fat suppression is acknowledged to be superior to CT in the initial evaluation. Neither modality has findings specific for IgG4‐ROD; however, an enhancing focal or infiltrating orbital mass is typically more thoroughly defined on MRI 952 . Unfortunately, the nonspecific nature of these imaging findings makes it difficult to distinguish IgG4‐ROD from other more common orbital adnexal lymphoproliferative (OAL) disorders or in rare cases, fungal disease or vasculitis.…”

Interdisziplinäre Therapie der Erkrankungen der Orbita

“…Es handelt sich um eine heterogene Gruppe von Erkrankungen mit möglicherweise autoimmunologischem Ursprung [364]. Es kommt zu einer Proliferation und Destruktion von Strukturen durch eine unspezifische Entzündungsreaktion mit lymphozytärer Infiltration [365] (▶Abb. 15).…”

Distinct groups of autoantigens as drivers of ocular adnexal MALT lymphoma pathogenesis

“…The significance of environmental factors in supporting tumor cell growth is underscored by the fact that most gastric MALT lymphomas are cured by Hp eradication ( Wotherspoon et al, 1993 ; Fischbach et al, 2007 ; Ruskone-Fourmestraux et al, 2011 ; Nakamura et al, 2012 ; Zucca et al, 2020 ). In MALT lymphomas at other primary sites such as ocular adnexa, lung, and more rare locations, the etiology and role of the chronic inflammation remain unclear ( Das et al, 2019 ; Derakhshandeh et al, 2021 ). In some studies, an association between ocular adnexal MALT lymphomas (OAML) and chronic Chlamydia psittaci infection was proposed, an association that could not be substantiated by other studies ( Bende et al, 2009 ; Schreuder et al, 2017 ; Johansson et al, 2022 ).…”