Orbital Inflammatory Pseudotumor Research Articles

Inflammatory disease of the orbit: A case series

Inflammatory orbital pseudotumors are a heterogeneous group of diseases characterized by their sensitivity to corticosteroid treatment regardless of the underlying etiology. Histological examination is generally performed following a lack of response to steroids, progressive neurological involvement, or suspicion of malignancy on radiological imaging. Herein, we report three clinical cases presented to our internal medicine department. The first case underscores the need for enucleation of the orbit due to the aggressiveness of orbital pseudotumors. This first case revealed Takayasu disease in a 39-year-old patient. Following the failure of corticosteroid therapy, favorable progression was achieved with adalimumab. Our second case concerns a 49-year-old woman presenting with exophthalmos, demonstrating rapid improvement in both clinical and biological parameters on corticosteroid administration. However, despite clinical amelioration, the partial radiological persistence of the orbital tumor prompted a positron emission tomography scan, revealing intense metabolic activity in the orbit despite the absence of clinical symptoms. Given the impracticability of biopsy, treatment with anti-CD20 was proposed, yielding a favorable radiological response. The third case describes an inflammatory orbital pseudotumor revealed by hemolytic anemia, with improved clinical and biological parameters observed over a 6-month period.

CASE REPORT: IDIOPATHIC SCLEROSING ORBITAL INFLAMMATION

Idiopathic sclerosing orbital inflammation (ISOI) also known as sclerosing orbital pseudotumor is a rare, idiopathic, chronic, slowly progressive orbital inflammation, characterized by scarring of the tissue that represents a mass effect. The latter leads to bulging of the eye, restriction of ocular movements and visual impairment up to potential loss of vision. It is a subtype of non-specific inflammatory orbital pseudotumor, which both radiologically and clinically mimics a malignant process or other orbital inflammatory disease, and therefore is challenging to diagnose and manage. This case highlights the challenges in diagnosis and management of ISOI.

Bilateral Orbital Myositis: A Rare Case Report

The orbital idiopathic myositis is included in the group of orbital inflammatory pseudotumors. It’s an isolated inflammation, unspecific of orbital muscles. This pathology is rare in Children and it is a source of real diagnosis problems. We report the case of a 10-year-old boy who was hospitalized for bilateral painful exophthalmos. We report the case of a 10-year-old boy who was hospitalized for bilateral exophthalmos of abrupt onset, painful, axial, and non reducible with complete ophthalmoplegia of both eyes. The examination showed a retained Visual Acuity (VA) of 10/10 with a normal Fundus Examination (FO) in both eyes. The inflammatory and thyroid work-up are negative. The orbital tomodensitometry revealed a grade I exophtalmitis and a thickening of external and inferior muscles. The corticotherapy leaded to the decline of exophtalmitis andnormalization of eye movements after 1 month of treatment. The etiologic survey was negative, then the diagnosis of idiopathic myositis was kept. The orbital idiopathic myositis stills an eliminating diagnosis. Faced to a child’s exophtalmitis, other differential diagnosis must be excluded by an advanced etiologic survey.

Rapidly Resolving Orbital Pseudotumor in Systemic Lupus Erythematosus

Rapidly Resolving Orbital Pseudotumor in Systemic Lupus Erythematosus

Role of imaging classification in the diagnosis and management of orbital inflammatory pseudotumor

Orbital inflammatory pseudotumor (OIP) is a kind of orbital idiopathic benign space-occupying lesion with no clear etiology and mainly characterized by inflammatory response. The clinical diagnosis of OIP should be based on exclusion of other diseases or surgical biopsy. Medication and surgical excision are both diagnostic and therapeutic methods. The choice of which is a problem that baffles clinicians. This article discusses a new strategy basing on the imaging features and classifications of OIP. It is expected to be further discussed and promoted in clinical practice to improve the diagnosis and treatment of OIP.

Value of diffusion-weighted magnetic resonance imaging (DWI) in differentiating orbital lymphoma from idiopathic orbital inflammatory pseudotumor

BackgroundDiffusion-weighted MR imaging can provide physiological information complementing morphological findings from conventional MRI. It detects early tissue changes associated with changes in water content, such as changes in the permeability of cell membranes, cell swelling or cell lysis. Areas of diseased tissue are highlighted with increased signal intensity on diffusion-weighted MR imaging. A decrease in the ADC is expected with increased intracellular tissue caused by either cell swelling or increased cellular density. DWI can be performed without the need for the administration of exogenous contrast medium, so it may of use when contrast administration is contraindicated. It yields quantitative and qualitative information that reflects changes at the cellular level and indicates the integrity of cell membranes. The purpose of this study was mainly to assess the diagnostic value of DWI for the discrimination of orbital lymphoma from idiopathic orbital inflammatory pseudotumor.ResultsOf our 53 cases presented with proptosis or visual disturbances, 32 cases (60.4%) had found to be present with idiopathic orbital inflammatory pseudotumor and 21 cases (39.6%) had orbital lymphoma. On conventional MR imaging, ill-defined tumor margin and orbital preseptal space involvement had a significant association with orbital lymphoma, whereas intense post-contrast enhancement of lesion and radiologic evidence of sinusitis were associated with orbital inflammatory pseudotumor. The mean ADC value of orbital lymphoma was significantly lower than those of benign inflammatory pseudotumor, yielding 100% sensitivity, 99% specificity, and 90.5% accuracy for differentiating both entities.ConclusionsDiffusion-weighted MR imaging (DWI) is valuable in discriminating orbital inflammatory pseudotumor from malignant orbital lymphoma that help patients to initial management.

Orbital Inflammatory Pseudotumor following mRNA COVID-19 Vaccination

ABSTRACT The authors present a case of orbital pseudotumor after mRNA COVID-19 vaccination. A 40-year-old otherwise healthy woman was referred to our oculoplastics unit because of left blepharoptosis of 2 months duration starting 1 week after she received her first Pfizer-BioNTech mRNA vaccination. On presentation, her best-corrected visual acuity was 20/20 in each eye. The external examination revealed left blepharoptosis and mild upper eyelid swelling. Orbital magnetic resonance imaging revealed left lacrimal gland enlargement with homogeneous contrast enhancement and diffuse mild enlargement of the left lateral and superior rectus muscles. The results of the extended workup for autoimmune and infectious etiologies and the systemic examination findings were normal. Systemic corticosteroids were started for the orbital pseudotumor. The presented case of orbital pseudotumor development after the mRNA vaccine may be considered to be an immunological process targeting the orbital tissue following immunization, although the cause–effect relationship remains uncertain.

Correlation Between IVIM-DWI Parameters and Pathological Classification of Idiopathic Orbital Inflammatory Pseudotumors: A Preliminary Study.

ObjectiveTo investigate the correlation between intravoxel incoherent motion diffusion-weighted imaging (IVIM-DWI) and the pathological classification of idiopathic orbital inflammatory pseudotumors (IOIPs).MethodsNineteen patients who were diagnosed with IOIPs (a total of 24 affected eyes) between November 2018 and December 2020 were included in the study. All the patients underwent magnetic resonance imaging orbital plain scans and IVIM-DWI multiparameter scans before an operation. The true diffusion coefficient (D), pseudodiffusion coefficient (D*), and perfusion fraction (f) values were obtained. Based on histopathology, the lesions were divided into three types: lymphocytic infiltration, fibrosclerotic, and mixed. The correlation between IVIM-DWI parameters and pathological classification was tested with the histopathological results as the gold standard. The data were analyzed using SPSS version 17.0, with P < 0.05 defined as significant.ResultsAmong the 19 patients (24 eyes) affected by IOIP, there were no significant differences between IOIP pathological classification and gender or age (P > 0.05). There were statistically significant differences between the D and f values for different pathological types of IOIP and IVIM parameters (P < 0.05), and there was no significant difference in D* value between the different pathological types (P > 0.05).ConclusionThe D and f values showed correlation with different types of IOIP, and the sensitivity of the D value was higher than that of the f value. The D* value showed no significant distinction between pathological types of IOIP.

Treatment and Prognosis of Inflammatory Pseudotumor of the Skull Base.

Background Inflammatory pseudotumor (IPT) of the skull base is a rare, locally destructive lesion managed with a variety of treatments. We explore the impact of treatment on outcome and assess the prognosis of IPT. Methods This is a retrospective review of IPT of the skull base at a tertiary academic medical center. The primary outcome was radiographic progression after treatment. Outcome versus tumor location was also examined and a prognostic model was developed using a logistic regression. Results The demographics of 21 patients with IPT are reported. Treatment consisted of corticosteroids (in 80.1% of patients), disease modifying antirheumatic drugs (DMARDs; 33.3%), surgical resection (28.6%), radiation (23.8%), antibiotics (14.3%), chemotherapy (rituximab; 9.5%), and antivirals (4.8%). At 50.7 months, 50.8% had radiographic progression. Local therapy trended toward having a better response than systemic therapy ( p = 0.60). IPT of the orbit required 2.4 treatment modalities, compared with 2.0 for pharyngeal IPT, and 1.3 for posterior skull base masses ( p = 0.14). A total of 75% orbital IPT underwent radiographic progression, compared with 71% of pharyngeal IPT and 50% of posterior skull base masses ( p = 0.62). Sixteen patients were used to create the logistic model of radiographic progression. The Cox-Snell R 2 was 0.71 ( p = 0.03). No individual variables were statistically significant. Conclusion To our knowledge, this is among the largest sample of cases describing the presentation, treatment, and prognosis of IPT of the skull base. Our data suggest that there may be an improved response with local therapy over systemic therapy and better prognosis among posterolateral skull base masses.

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

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.

A good visual recovery in a patient with central retinal artery occlusion associated with Churg-Strauss syndrome: case report

Background: Churg-Strauss syndrome (CSS) is a rare systemic necrotizing vasculitis of unknown etiology that affects small-to-medium-sized vessels. A multisystem disease that mostly affects the lungs and the skin. In addition, it could affect the renal, cardiovascular, gastrointestinal, central, and peripheral nervous systems. Ocular involvement in the CSS is rare. The reported ocular manifestations include corneal ulcer, uveoscleritis, conjunctival granuloma, orbital inflammatory pseudotumor, amaurosis fugax, retinal artery occlusion, ischemic optic neuropathy, oculomotor nerve palsy, and trochlear nerve palsy. Case Presentation: A 53-year-old man was admitted with sudden visual loss. CSS was diagnosed earlier, based on asthma, eosinophilia, peripheral neuropathy symptoms, and pulmonary infiltrates. His vision was completely lost in the left eye. Unilateral central retinal artery occlusion (CRAO) was observed by fluorescence angiography. Results: The treatment modality used in our patient was steroid pulse therapy, and his vision improved 24 hours later. This outcome might emphasize the importance of steroid pulse therapy since it may be effective for CRAO in CSS patients. Conclusion: This case report described a CSS patient with associated CRAO, in whom steroid pulse therapy improved his visual acuity. Therefore, since intensive treatment should be administered as early as possible to improve the prognosis of vision, rheumatologists should be aware of the presence of CSS-associated CRAO, and they should refer those patients to ophthalmology as soon as possible.

Tolosa-Hunt Syndrome: A Review of Diagnostic Criteria and Unresolved Issues.

Purpose:To review the diagnostic criteria for Tolosa–Hunt syndrome (THS) and utility of recent modifications.Methods:We searched PubMed for keywords Tolosa Hunt and magnetic resonance imaging. We compared the three editions of International Classification of Headache Disorders and isolated case reports and case series with the assessment of cavernous internal carotid artery (ICA) caliber to find the prevalence of vascular anomalies. We also evaluated cases of THS with the involvement of extracavernous structures and the possible role of idiopathic hypertrophic pachymeningitis (HP). Cases diagnosed falsely as THS were also reviewed for the presence of atypical features and relevance of criterion D. We assessed nonconforming cases (those with normal neuroimaging benign THS) and idiopathic inflammatory orbital pseudotumor (IIPO).Results:Vascular abnormalities were found in 36.36% of THS cases. Benign THS may also show changes in ICA caliber. Evidence suggestive of idiopathic HP could be found in 57% of cases with the involvement of extracavernous structures, such as facial nerve and pituitary gland. Both THS and IIPO are steroid-responsive pathologies with similar clinical and radiological features. False-positive diagnosis of THS results from early labeling, based solely on clinical features and symptom resolution after steroid therapy.Conclusions:Benign THS may be a result of limitation of resolution of available neuroimaging technique or early testing. Early and late vascular changes can be seen in both THS and its benign variant; some of them are not innocuous. THS may be considered a type of focal idiopathic HP. IIPO may represent an anterior variant of THS. In the absence of histopathological diagnosis, steroid-induced resolution of symptoms should be confirmed radiologically and followed-up.

Clinical Analysis of Castleman's Disease of the Lacrimal Gland.

Objective To explore the clinical manifestations, imaging characteristics, and pathological characteristics of Castleman's disease of the lacrimal gland, enhance the knowledge of the disease, and improve the level of its diagnosis and treatment. Methods In the retrospective study, the data of 5 patients diagnosed with Castleman's disease of the lacrimal gland in Tianjin Medical University Eye Hospital from 2014 to 2018 were analyzed, and the relevant literature was reviewed. Results All the 5 patients were confirmed by pathological examination. Clinical manifestations were characterized by mass occupying lesions in the lacrimal gland area, without obvious pain, accompanied by eyelid swelling and ptosis, as well as space-occupying symptoms. Imaging examination showed that there was a soft tissue mass in the enlarged lacrimal gland area, and the mass was rich in blood flows while showing no obvious specificity, which could invade the surrounding muscles. All patients underwent surgical resection. Pathological results showed that 1 case was of the hyaline-vascular type, 3 cases were of the plasma cell type, and 1 case showed malignant transformation to plasma cell tumor. Conclusion Castleman's disease of the lacrimal gland is a rare orbital lymphoproliferative disease lacking specificity in clinical manifestations and imaging examination. As there are difficulties in differentiating the disease from orbital inflammatory pseudotumor and orbital lymphoma, its diagnosis still depends on pathological examination. The disease is mainly treated with surgical resection, and the pathological type is determined postoperatively.

Arterial spin labeling and diffusion-weighted MR imaging: Utility in differentiating idiopathic orbital inflammatory pseudotumor from orbital lymphoma

PurposeTo assess arterial spin-labeling (ASL) and diffusion-weighted imaging (DWI) and in combination for differentiating between idiopathic orbital inflammatory pseudotumor (IOIP) and orbital lymphoma. Material and methodsA retrospective study was done on 37 untreated patients with orbital masses, suspected to be IOIP or orbital lymphoma that underwent ASL and DWI of the orbit. Quantitative measurement of tumor blood flow (TBF) and apparent diffusion coefficient (ADC) of the orbital lesion was done. ResultsThere was a significant difference (P = 0.001) in TBF between patients with IOIP (n = 21) (38.1 ± 6.2, 40.3 ± 7.1 ml/100 g/min) and orbital lymphoma (n = 16) (55.5 ± 7.1, 56.8 ± 7.9 ml/100 g/min) for both observers respectively. Thresholds of TBF used for differentiating IOIP from orbital lymphoma were 48, 46 ml/100 g/min revealed area under the curve (AUC) of (0.958 and 0.921), and accuracy of (86% and 83%) for both observers respectively. There was a significant difference (P = 0.001) in ADC between patients with IOIP (1.04 ± 0.19, 1.12 ± 0.23 × 10−3 mm2/s) and orbital lymphoma (0.69 ± 0.10, 0.72 ± 0.11 × 10−3 mm2/s) for both observers respectively. Thresholds of ADC used for differentiating IOIP from orbital lymphoma were 0.84 and 0.86 × 10−3 mm2/s with AUC of (0.933 and 0.920), and accuracy of 89% and 90% for both observers respectively. The combined TBF and ADC used for differentiating IOIP from orbital lymphoma had AUC of (0.973 and 0.970) and accuracy of (91% and 89%) for both observers respectively. ConclusionTBF and ADC alone and in combination are useful for differentiating IOIP from orbital lymphoma.

Relapsing IgG4-related orbital inflammatory pseudotumor after achieving success with rituximab

IgG4-related disease (IgG4-RD) is a disease of fibroinflammatory with infiltration of IgG4-positive plasma cells. The lesions show focal or diffuse nodular formations in one or multiple organs; due to the infiltration of lymphocytes and plasma cells with fibrosis. The IgG4-DR is recognized as an important cause of idiopathic inflammatory disease that must be differentiated with other pathologies. The measurement of normal IgG4 levels is not enough to exclude IgG4-RD. The treatment covers the symptomatic aspect and use of systemic corticosteroids (CS), and immunomodulatory and biological therapy. The relapsing is might be misdiagnosis. We propose to consider the imaging follow-up for the therapeutic management

Differentiation of orbital lymphoma and idiopathic orbital inflammatory pseudotumor: combined diagnostic value of conventional MRI and histogram analysis of ADC maps

BackgroundThe overlap of morphological feature and mean ADC value restricted clinical application of MRI in the differential diagnosis of orbital lymphoma and idiopathic orbital inflammatory pseudotumor (IOIP). In this paper, we aimed to retrospectively evaluate the combined diagnostic value of conventional magnetic resonance imaging (MRI) and whole-tumor histogram analysis of apparent diffusion coefficient (ADC) maps in the differentiation of the two lesions.MethodsIn total, 18 patients with orbital lymphoma and 22 patients with IOIP were included, who underwent both conventional MRI and diffusion weighted imaging before treatment. Conventional MRI features and histogram parameters derived from ADC maps, including mean ADC (ADCmean), median ADC (ADCmedian), skewness, kurtosis, 10th, 25th, 75th and 90th percentiles of ADC (ADC10, ADC25, ADC75, ADC90) were evaluated and compared between orbital lymphoma and IOIP. Multivariate logistic regression analysis was used to identify the most valuable variables for discriminating. Differential model was built upon the selected variables and receiver operating characteristic (ROC) analysis was also performed to determine the differential ability of the model.ResultsMultivariate logistic regression showed ADC10 (P = 0.023) and involvement of orbit preseptal space (P = 0.029) were the most promising indexes in the discrimination of orbital lymphoma and IOIP. The logistic model defined by ADC10 and involvement of orbit preseptal space was built, which achieved an AUC of 0.939, with sensitivity of 77.30% and specificity of 94.40%.ConclusionsConventional MRI feature of involvement of orbit preseptal space and ADC histogram parameter of ADC10 are valuable in differential diagnosis of orbital lymphoma and IOIP.

Clinical, radiological and immunological features of orbital inflammatory pseudotumor with neurological involvement in a Japanese cohort

Clinical, radiological and immunological features of orbital inflammatory pseudotumor with neurological involvement in a Japanese cohort

Expression and significance of nuclear factor-κB-related proteins in idiopathic orbital inflammatory pseudotumor

Background Idiopathic orbital inflammatory pseudotumor (IOIP) is a commom orbital disease, with serious eye symptoms and replase tendency, and its pathogenesis is still unclear.Nuclear factor-κB (NF-κB)-related proteins participate in many important pathophysiological process, however, whether NF-κB plays a role in the IOIP process is worthy of attention. Objective This study was to explore the roles of NF-κB pathway in IOIP pathogenesis. Methods Twenty-four IOIP specimens were collected during surgery in Beijing Tongren Hospital from September 2010 to May 2016.The histopathological characteristics of IOIP were examined by hematoxylin and eosin staining.The expression and location of NF-κB/p65, p-p65, p50 and inhibitor of κB (IκB-α) were detected by immunohistochemistry and verified by immunocytochemistry and Western blot assay. Results The histopathological features of IOIP were numerous small lymphocyte infiltraion and fibrous tissue proliferation, and a lot of epithelioid cells were seen in lacrimal gland-involved specimens.NF-κB/p65 was positively expressed in the cytoplasm of all 24 specimens and the nucleus in 15 specimens with the expressing rate of 62.5%.p50 was expressed in the cytoplasm in 22 specimens with the expressing rate of 91.7% and in the nucleus in 17 specimens with the expressing rate of 70.8%.The positive expression of p-p65 was found in 22 specimens with the expressing rate of 91.7%, and IκB-α was expressed in the cytoplasm of 11 specimens with the expressing rate of 45.8%.These results were confirmed by immunocytochemistry and Western blot assay. Conclusions NF-κB pathway is activiated during IOIP process, and NF-κB pathway may be involved in the pathogenesis of IOIP. Key words: Nuclear factor-kappa B/metabolism; Orbital inflammatory pseudotumor/pathology; Inflammation; Immunochemistry; Humans; Nuclear proteins/metabolism; Signal transduction pathway

Epstein-Barr virus-encoded small RNAs in idiopathic orbital inflammatory pseudotumor tissues: a comparative case series.

To investigate the positive rate and types of cells that express Epstein-Barr virus-encoded small RNAs (EBERs) and to determine the distribution of EBER-expressing cells in idiopathic orbital inflammatory pseudotumor (IOIP) tissues. We retrospectively examined 40 archived paraffin specimens from two teaching hospitals in Southern China between January 2007 and January 2015 that were pathologically determined to exhibit IOIP. Eleven concurrent paraffin specimens of thyroid-associated ophthalmopathy (TAO) composed the control group. In situ hybridization was performed to detect EBERs. Immunohistochemistry was employed to detect CD3, CD20, Vimentin, and smooth muscle actin (SMA), and the positive rate, types of positive cells, and distribution and location of EBERs were evaluated. The positive expression rate of EBERs was 47.5% (19/40) in the IOIP group, which was significantly higher than that in the TAO group [0 (0/11), P=0.011]. In the IOIP group, the lymphocyte infiltrative subtype, fibrotic subtype, and mixed subtype exhibited EBER-positive rates of 57.1% (12/21), 12.5% (1/8), and 54.5% (6/11), respectively, and no significant differences were found between these subtypes (P=0.085). Positive signals of EBERs were mainly present in medium-small lymphocytes between or around follicles and in the nuclei of activated immunoblasts (14/19). The positive rate, types, and distribution of EBER-expressing cells in IOIP have been documented. These findings are conducive for a better understanding of the underlying mechanisms of Epstein-Barr virus infection in IOIP pathogenesis.

Latent orbital pseudotumor secondary to systemic lupus erythematosus.

Key Clinical MessageOrbital inflammatory pseudotumor is a rare complication of systemic lupus erythematosus. It may present a challenge for differential diagnosis, especially in the context of treatment with hydroxychloroquine, although dosage and duration of the treatment may guide us. Although high antibody titers can be found, this is not specific.