Orbital Mass Lesions Research Articles

IgG4-related disease initially presented as an orbital mass lesion mimicking optic nerve sheath meningioma

We report a case of an optic nerve mass lesion associated with IgG4-related disease. A 39-year-old man presented with right blurred vision and proptosis 8years before admission. Magnetic resonance imaging showed a mass lesion in the center of the right orbit, which was diagnosed as optic nerve sheath meningioma by neuroradiologists and neurosurgeons. Irradiation was selected for treatment of the lesion on the basis of the radiological diagnosis; subsequently, the lesion gradually reduced in size. However, regrowth of an optic nerve mass lesion observed during the previous 2years caused remarkable exophthalmos, and removal of the orbital mass lesion was performed via a transcranial orbital approach. Pathological examinations resulted in a diagnosis of IgG4-related disease, and hematological tests revealed an elevated level of serum IgG4. Additional radiological examinations showed mass lesions in the left maxillary nerve, bilateral inferior alveolar nerves, paravertebral tissue, and left kidney. Treatment with oral steroids has produced a reduction in the size of these lesions.

Wegener's Granulomatosis with Nervous System Involvement: A Hospital-Based Study

Background: The aim of this study was to ascertain the clinical manifestations of granulomatosis with polyangiitis (Wegener's) (GPA) with the involvement of the peripheral nervous system (PNS) and central nervous system (CNS). Summary: All neurologic inpatients in a hospital over a 12-year period were reviewed. Nine patients met both the ACR 1990 traditional format criteria for the classification of GPA and the Chapel Hill nomenclature mandatory criteria for GPA. We focused on the clinical presentation, serological data, biopsy reports, disease activities [as assessed by the Birmingham Vasculitis Activity Score (BVAS)], electrophysiology, and brain images. Nine patients met the diagnostic criteria for GPA. The neurological signs of the initial manifestation of GPA were found in 6/9 (67%) patients. Eight patients had GPA-related CNS involvement, including four patients with chronic hypertrophic pachymeningitis, with either diffuse or focal thickening; three had intracranial hemorrhages and two had orbital mass lesions with optic nerve compression. In addition, six patients showed PNS involvement, including three with asymmetric sensorimotor polyneuropathy, two with symmetric sensorimotor polyneuropathy, and one with bilateral mononeuropathy. Key Messages: Neurological manifestation is not uncommon and can be the first clinical sign of GPA. The involvement of both CNS and PNS raises the possibility of GPA in hospitalized neurologic patients.

Eosinophilic angiocentric fibrosis of the orbit: A case report and review of literature

Eosinophilic angiocentric fibrosis is a rare condition presenting with tumefactive lesions of the nasal cavity, paranasal sinuses, upper respiratory tract and rarely the orbit; and has characteristic histopathological features. We report the case of a 38-year-old lady with a mass lesion in right orbit. Histopathological examination of the biopsy specimen revealed extensive areas of perivascular fibrosis showing a characteristic angiocentric whirling with onion skin pattern; along with perivascular exudates of eosinophils accompanied by collection of plasma cells and lymphocytes. Rheumatology review and autoimmune screening was negative. She underwent surgical debulking followed by tapering course of oral steroids and was completely free of orbital disease at her last follow-up visit.

The role of multidetector computed tomography and magnetic resonance recent imaging techniques in the evaluation of intra-conal orbital lesions

ObjectiveThe purpose of our study was to assess the role of multi-detector computed tomography and magnetic resonance recent imaging techniques in the evaluation of intra-conal orbital lesions. Subjects and methods30 patients presented to the Ophthalmic and Radiology Departments at El-Minia University Hospital and El Minia Oncology Center with clinical diagnosis of visual loss and proptosis. They presented during the period from June 2012 to October 2013. Their ages range from 5 to 73years. All patients subjected to MDCT and MRI. Also, contrast injection was injected on some cases. 24 patients were subjected to surgery and histopathology to confirm the radiological diagnosis. ResultsA total of 30 patients were included (their mean age 26years). 8 lesions were diagnosed inflammatory in nature (4 were orbital abscess and 4 were idiopathic orbital inflammatory disease i.e. pseudo tumors). 10 lesions were diagnosed as vascular lesions (2 lesions were carotid cavernous fistula and 8 lesions were cavernous hemangiomas). 6 lesions were diagnosed as optic nerve gliomas and other 6 lesions were diagnosed optic nerve sheath meningiomas. ConclusionThe use of MDCT and MRI recent techniques are mandatory for accurate diagnosis of the nature of intra-conal orbital mass lesions.

Orbital Mass Secondary to Acute Lymphoblastic Leukaemia in a Child: A Rare Presentation

We report the case of a 3-year-old child, who presented with lid swelling which progressed to proptosis of the left eye. He also had systemic symptoms of fatigue and weight loss. An examination revealed hepatosplenomegaly and lymph node enlargement. Investigations showed a peripheral smear with blast cells, which were also revealed through a bone marrow biopsy. A CT scan showed a mass lesion in the left orbit that had infiltrated into the surrounding tissues. He was diagnosed with acute lymphoblastic leukaemia (ALL) with left-sided orbital mass secondary to it. Haematogenous masses in the orbit are commonly due to granulocytic sarcomas, which are usually associated with acute myelogenous leukaemia (AML), not ALL, and are rare especially when they precede systemic disease.

Location and frequency of lesions in patients with IgG4-related ophthalmic diseases

It is well-known that the lacrimal gland (LG) may be affected in IgG4-related ophthalmic disease (IgG4ROD). Recently, IgG4-related ophthalmic lesions other than those of the lacrimal gland have been reported. However, no study to date has revealed the details of these lesions. This study was conducted to evaluate the location and frequency of lesions found in conjunction with IgG4ROD using radiological imaging. Radiological images and clinical records of 65 patients collected from seven institutions in Japan were reviewed retrospectively. All patients had been pathologically diagnosed with IgG4ROD. Patients of mucosa-associated lymphoid tissue lymphoma associated with IgG4-related lesions were excluded. Orbital magnetic resonance imaging or computed tomography findings were evaluated. Of the 65 patients, 31 (47.7%) had lesions involving the LG alone, whereas 34 (52.3%) had lesions involving the areas other than LG, including eight patients who had lesions without any LG involvement. IgG4-related ophthalmic lesions included LG enlargement in 57 patients (87.7%), trigeminal nerve branch enlargement in 25 (38.5%), extraocular muscle enlargement in 16 (24.6%), diffuse orbital fat lesions in 15 (23.1%), orbital mass lesions in 11 (16.9%), eyelid lesions in eight (12.3%), and nasolacrimal duct lesion in one (1.5%). Six patients (9.2%) presented with visual disturbance due to optic nerve disturbance, eight (12.3%) with a restriction of ocular movement, and 19 (29.2%) with exophthalmos. Thirty-four (52.3%) of the 65 IgG4ROD patients had lesions in areas other than LG. Lesions were found in the trigeminal nerve branch including pterygopalatine fossa, extraocular muscles, orbital fat, eyelid, and nasolacrimal duct.

The role of fine needle aspiration cytology in the diagnosis of orbital lesions

Context: Fine needle aspiration cytology (FNAC) in orbital lesions has gained importance over the last 3 decades, especially with the advent of imaging studies. Aims: The study was undertaken to evaluate the role of FNAC as a diagnostic tool in patients presenting with orbital mass lesions. Subjects and Methods: Patients of different age groups presenting with orbital lesions were studied over a period of 2 years. The 38 patients selected for this study were evaluated clinically by thorough general and ophthalmological examination, and then were investigated with computed tomography (CT) scanning (both axial and coronal planes). Each patient included in this study was then subjected to FNAC under direct vision or under ultrasonography or CT guidance with a sterile 22 gauge needle without anesthesia. Results: The age of the patients varied from 2 to 72 years. On cytology, four cases were diagnosed as nonneoplastic and the remaining 34 (89.48%) as neoplastic lesions. In the neoplastic group, benign tumors (20; 58.8%) outnumbered malignant (14; 41.2%). The distribution of malignant lesions was more heterogeneous with a predominance of lymphoma involving the orbit. Surgical biopsy was done in 27 cases. Surgical biopsy report correlated with the FNAC report in 23 out of 27 cases. The sensitivity was 86.6%, specificity 100%, and positive predictive value 100%. Conclusion: FNAC is a useful, safe and cost-effective method of diagnosing orbital pathology. Image guidance is helpful especially in deeply situated nonpalpable lesions.

Role of fine needle aspiration cytology in the diagnosis of orbital masses: A study of 41 cases.

Background:Complexity in the anatomy of orbit and the fear of globe rupture are the main challenges faced in the diagnosis and typing of orbital masses. Fine needle aspiration cytology can be used as an initial investigative procedure in the evaluation of orbital masses, which in turn can aid the clinician to plan the treatment modalities. A close cooperation between ophthalmologist and pathologist adds to the success of the procedure.Aim:The study was conducted in an attempt to evaluate the role of fine needle aspiration cytology (FNAC) in the diagnosis of orbital lesions as a cost-effective diagnostic technique, and to assess its diagnostic efficacy by comparing it with histopathological diagnosis.Materials and Methods:The study was conducted on 50 patients, over a period of 3 years, who had presented with anterior orbital mass lesions with or without proptosis, and with those having accessible mass lesions. Patients with proptosis without anterior orbital masses, proptosis due to dysthyroid ophthalmopathy, arteriovenous fistulas, hamartomas and choristomas were excluded from the study. FNAC procedure was done after explaining about the procedure to the patient, and in the presence of an ophthalmologist.Results:Majority of patients belonged to the age group 50-59 years. Male: female ratio was 1.05: 1. The most common lesion on FNAC was non-Hodgkins lymphoma, [13 cases (31.7%)]. 11 (26.8%) cases out of this were confirmed to be non-Hodgkins lymphoma on histopathologic examination. Two cases turned out to be inflammatory pseudotumor.Conclusions:FNAC can be done in all palpable orbital mass lesions with minimal risk and complications, with close cooperation between ophthalmologist and pathologist. A good degree of correlation was obtained between FNAC and histopathology, which was assessed by kappa statistics.

AB1234 Clinical characteristics of IgG4-related orbital pseudo-tumor

BackgroundIgG4-related disease (IgG4-RD) is a new emerging disease entity of unknown etiology with multiorgan involvement. IgG4-RD includes a wide variety of diseases, including Mikulicz’s disease (MD), autoimmune pancreatitis (AIP), hypophysitis,...

CORE needle biopsy of orbital tumors

In some orbital mass lesions, histology may be the only way to establish an accurate diagnosis. Core needle biopsy (CNB) is widely performed in oncology, and unlike fine needle aspiration biopsy (FNAB), it can provide sufficient tissue sample for histology and immunohistochemistry. The purpose of this study was to evaluate the usefulness and possible complications of CNB of orbital tumors. Fifty orbital lesions from 46 patients (age: 2-84 years) were biopsied using 20-gauge and 18-gauge semi-automated needles; 11 procedures were performed under ultrasound guidance. All 50 samples received routine histopathological examination and immunohistochemical analysis. Specimens diagnostically sufficient for histological analysis were obtained in all biopsies. The histopathological diagnosis was established in 94% of specimens: 30 tumors were malignant: lymphoma (20); rhabdomyosarcoma (six); lacrimal gland carcinoma (one); breast carcinoma metastasis (two); melanoma (one); three were benign; ten inflammatory; and four were orbital fibrosis. Three biopsies were nondiagnostic: two (lymphoma, angiofibroma) showed undetermined identification and one was a false-negative (lacrimal gland adenocarcinoma was misdiagnosed as fibrous tissue). The diagnoses were confirmed by excisional or incisional biopsy in 26 patients. The concordance rate in patients with a surgically confirmed diagnosis was 88%. The sensitivity, specificity and accuracy for differentiating malignant from benign lesions were 94%, 100%, and 96%, respectively. Two patients suffered complications: mild retrobulbar hematoma with no decreased visual acuity. There was no damage to the globe or optic nerve, motility disorder, or infection. CNB of orbital mass lesions is a safe procedure that provides a sufficient amount of tissue for histology and immunohistochemistry, and may be useful for improving the quality of the pathological diagnosis of orbital tumors.

Three cases of IgG4-related orbital inflammation presented as unilateral pseudotumor and review of the literature

IgG4-related disease is an emerging disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. In IgG4-related disease, tumor-like lesions develop in multiple organs, such as the lacrimal gland, salivary gland, lung, pancreas, kidney, and lymph nodes. We report here three cases of IgG4-related orbital inflammation that presented as unilateral pseudotumors. The patients all were men, with an age range of 65-75 years. The patients had been pointed out unilateral intra-orbital masses, and histopathological examinations revealed marked accumulation of IgG4-positive plasma cells (IgG4/IgG ratio: 51.1-71.6%) with fibrosis. But storiform fibrosis was seen in only one case, and no obliterative phlebitis was seen. The serum levels of IgG4 were increased to 178-670 mg/dL. The masses had well-defined homogeneous signal intensities, and they were hypo-intense on T1-weighted MR images and iso-intense on T2-weighted MR images. Gadolinium enhanced mass lesions in two cases. All orbital mass lesions responded well to corticosteroid treatment.

Acute Recurrence of Orbital Cavernous Hemangioma in a Young Man

A 29-year-old man presented with a history of prominent left eye of 6 months' duration. He also reported fluctuating blurred vision since 15 days ago. On examination, proptosis of 3 mm was noted in the left eye. Computed tomography (CT scan) of the orbits showed a well-circumscribed, hyperdense, intraconal mass lesion in left orbit, located in the inferotemporal quadrant. Orbitotomy was performed, and the tumor was delivered with an intact capsule. The clinical diagnosis of cavernous hemangioma was confirmed on histopathologic examination. The presence of intact capsule was confirmed grossly and with histopathology. The patient returned 6 weeks after surgery with recurrent proptosis. Repeated CT scan showed a recurrent intraconal mass of similar characteristics as in the primary presentation. A repeated orbitotomy was done, and the tumor was removed intact. Histopathology of the recurrent tumor confirmed the diagnosis of cavernous hemangioma. We report a rare case of orbital cavernous hemangioma with short-term recurrence (6 weeks).

Rituximab as a new therapeutic option in granulomatosis with polyangiitis: a report of two cases

Findings of several reports suggest that rituximab, a chimeric monoclonal anti-CD20 antibody causing B-lymphocyte depletion, might represent a treatment option for people with granulomatosis with polyangiitis (GPA) (former Wegener's granulomatosis). This study presents the results of rituximab treatment in two patients with treatment-refractory GPA. First patient received rituximab for a granulomatous posterior orbital mass lesion, and eye symptoms were resolved after three courses of treatment. The second patient had eye and paranasal sinus involvement and benefited from two courses of rituximab treatment, with significant clinical improvement. Rituximab may represent an effective novel treatment for remission induction in GPA.

Multiple cavernous hemangiomas of the orbit.

Cavernous hemangioma of the orbit is often unilateral and solitary. Multiple cavernous hemangiomas of the orbit are extremely rare. The authors reported four patients who had more than two cavernous hemangiomas in one orbit. Case reports.The clinical features, imaging findings, differential diagnosis and surgical management were presented. We found 4 cases of multiple cavernous hemangiomas of the orbit out of 214 records of all patients with orbital cavernous hemangiomas who were referred to us from Jan 1,1986 to Dec 31,2000. Two patients were female and two were male. The mean age was 46.5 years, ranging from 37.0 to 57.0 years. The left orbits were affected in all patients, with two, three, eight and ten tumors respectively. The most common complaint was a painless, gradually progressive proptosis. The characteristic CT imaging features were multiple well circumscribed, round or ovoid masses that were sharply delineated from the surrounding tissue. B-scan ultrasonography showed round-shaped orbital masses with high internal reflectivity. The therapeutic outcomes of lateral or anterior orbitotomy were excellent. Although rare, cavernous hemangioma should be considered in the differential diagnosis of patients with multiple orbital mass lesions.

A prospective comparison of fine-needle aspiration cytopathology and histopathology in the diagnosis of orbital mass lesions

A prospective comparison of fine-needle aspiration cytopathology and histopathology in the diagnosis of orbital mass lesions

Orbital aspergillus infection diagnosed by FNAC

Fungal infections of the orbit represent a small minority of orbital infections. However, due to the virulent nature of some of the fungal species, they can have a devastating effect on ocular functions. Most of these fungi are saprophytes, which cause opportunistic infections. Aspergillus is one such fungus that can cause infection at various sites in an immunosuppressed individual. Sinonasal aspergillus infection with orbital extension and orbital aspergillus infection progress relentlessly. They can have a precipitous clinical course resulting in total loss of vision. Fine needle aspiration cytology (FNAC) is rarely used as a preoperative diagnostic tool in the investigation of orbital mass lesions. Further, fungal infections of orbit are seldom diagnosed on FNAC. Two cases of fungal infection of the orbital and periorbital tissue diagnosed on FNAC are presented. A 50-year-old diabetic male presented with diminishing vision, pain, and forward protrusion of the left eye. On examination, he had upper eye lid fullness. A 55-year-old diabetic male presented with a swelling on the right upper eye lid. The patients were evaluated radiologically and then subjected to FNAC. The smears showed giant cells, histiocytes, epithelioid granulomas, and fungal hyphae. A diagnosis of fungal infection was arrived at which was subsequently confirmed by culture and biopsy. Orbital aspergillus infection can have a precipitous course. Computerized tomography and magnetic resonance imaging of the orbit provide crucial information. However, FNAC can help in making an early definitive diagnosis of fungal infection and thus obviate the need for a biopsy.

Ocular Adnexal Lymphoma: Diffusion-weighted MR Imaging for Differential Diagnosis and Therapeutic Monitoring

To describe the magnetic resonance (MR) imaging and diffusion-weighted (DW) imaging features of ocular adnexal lymphomas (OALs), to determine the diagnostic accuracy of apparent diffusion coefficient (ADC) for discriminating OALs from other orbital mass lesions, and to assess whether variations in ADC constitute a reliable biomarker of OAL response to therapy. Institutional ethical committee approval and informed consent were obtained. In this prospective study, 114 white subjects (65 females and 49 males) were enrolled. Thirty-eight patients with histopathologically proved OAL underwent serial MR and DW imaging examination of the orbits. ADCs of OALs were compared with those of normal orbital structures, obtained in 18 healthy volunteers, and other orbital mass lesions, prospectively acquired in 58 patients (20 primary non-OAL neoplasms, 15 vascular benign lesions, 12 inflammatory lesions, 11 metastases). Interval change in ADC of OALs before and after treatment was analyzed in 29 patients. Analysis of covariance and a paired t test were used for statistical analysis. Baseline ADCs in OALs were lower than those in normal structures and other orbital diseases (P < .001). An ADC threshold of 775 x 10(-6) mm(2)/sec resulted in 96% sensitivity, 93% specificity, 88% positive predictive value, 98.2% negative predictive value, and 94.4% accuracy in OAL diagnosis. Following appropriate treatment, 10 (34%) of 29 patients showed OAL volumetric reduction, accompanied (n = 7) or preceded (n = 3) by an increase in ADC (P = .005). Conversely, a further reduction of ADC was observed in the seven patients who experienced disease progression (P < .05). ADC permits accurate diagnosis of OALs. Interval change in ADC after therapy represents a helpful tool for predicting therapeutic response.

Magnetic resonance imaging in the analysis of pediatric orbital tumors: Utility of diffusion-weighted imaging

To identify common radiographic features of pediatric orbital tumors by the use of magnetic resonance imaging (MRI) techniques, diffusion-weighted imaging (DWI), fat saturated T2, and pre- and postgadolinium T1. DWI is hypothesized to help identify and predict the malignancy of specific brain tumors. To our knowledge, a similar analysis in which the authors have used this combination of MRI techniques has not been performed with orbital tumors. We performed a retrospective chart review of all patients younger than 18 years of age, each diagnosed with an orbital mass lesion, imaged by MRI from 2005 to 2008. The MR images were analyzed by use of the aforementioned techniques. Mass lesions identified in the chart review included rhabdomyosarcoma (n = 4), myofibroma (n = 2), hemangioma (n = 4), lymphangioma (n = 2), neurofibroma (n = 4), Langerhans histiocytosis (n = 2), and one of each of the following: giant cell tumor, meningioma, lymphoid hyperplasia of the lacrimal gland (chronic sclerosing sialadenitis), optic nerve glioma, lipodermoid, and dermoid. DWI was used to differentiate tumors into those with increased diffusion, restricted diffusion, and a mixed diffusion pattern. Capillary hemangiomas and rhabdomyosarcomas 2 tumors with potentially overlapping appearances with traditional MRI techniques had contrasting appearances with DNI. DWI can help to distinguish among certain pediatric orbital tumors when combined with traditional MRI techniques. This technique may thus be considered an additional tool to help, refine the differential diagnosis of orbital tumors in children.

Orbital Pseudotumor

Orbital pseudotumor is a benign condition that accounts for approximately 10% of all orbital mass lesions. Any part of the orbit may be involved. The etiology is unknown. The presentation may be either acute or subacute. Patients may present with a palpable mass, a swollen eyelid, congestion, pain, diminished ocular motility, and/or decreased visual acuity. Approximately, 25% of patients present with bilateral disease. A modest proportion of patients experience resolution of their symptoms without treatment. Biopsy is indicated for those who do not respond to, or relapse after, first-line therapy. Oral corticosteroids are the initial treatment and approximately 80% of patients respond. Roughly half of those who respond to corticosteroids relapse. Second-line therapy consists of either low-dose radiotherapy (20-30 Gy at 2 Gy per fraction), cytotoxic chemotherapy, or immunosuppressive agents. Radiotherapy results in long-term local control rates of 50% or higher. Limited lesions may be successfully resected. A small subset of patients may experience inexorable progression to a fixed, painful, sightless eye and require orbital exenteration.

Intravascular Lymphoma Presenting as an Orbital Mass Lesion: A Case Report

We describe a case of intravascular lymphoma that presented as an orbital mass lesion. A 77-year-old female presented with longstanding fatigue and one-month of diplopia. Magnetic resonance imaging (MRI) revealed a frontal lobe brain lesion and a right orbital mass. Brain biopsy was interpreted as anaplastic oligodendroglioma. Orbital biopsy revealed intravascular lymphoma. On review of brain histopathology, the diagnosis was revised to CNS intravascular lymphoma. To the best of our knowledge, this case represents the first report of intravascular lymphoma presenting as an orbital mass lesion.