Rare Orbital Involvement Originating from Extranodal Marginal Zone Lymphoma.

Ocular adnexa region (OAR) primary lymphomas are uncommon, accounting for 1-2% of non-Hodgkin lymphomas and 8% of extranodal lymphomas. Extranodal marginal zone lymphoma (EMZL) originates from several epithelial tissues, including the stomach, salivary gland, lung, small intestine, thyroid gland, and ocular adnexa region. Here, we report a 66-year-old female patient who was diagnosed with EMZL of OAR. In consideration of the possible side effect of radiotherapy, such as conjunctivitis, visual acuity impairment, and even retinal complications, she received six cycles of triweekly targeted chemotherapy with rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP) without radiotherapy. Then, she remained in complete remission up to the present day.

Fine-Needle Aspiration Biopsy as a Diagnostic Modality for Orbital Adnexal Lymphoma.

INTRODUCTION: The aim of this study was to evaluate fine-needle aspiration biopsy (FNAB) as a diagnostic tool for lymphoproliferative orbital lesions in light of recent improvements in cytomorphological and immunologic analyses. METHOD: Retrospective case series including all orbital FNABs with a lymphoproliferative outcome at Karolinska University Hospital, Stockholm, Sweden during the period 2005-2015. RESULTS: Of the 38 patients included, 31 (82%) were conclusively diagnosed as having lymphoma according to the first FNAB. Disease in 20 patients (65%) could be subclassified. The diagnosis in 7 patients (18%) was either inconclusive, suggestive of lymphoma, or reactive lymphatic infiltrate. These 7 patients were re-investigated, and the initial suspected diagnosis of malignant lymphoma was confirmed in four. Two of the remaining 3 patients were initially diagnosed as having non-lymphoproliferative disease; however, this was later changed to a lymphoproliferative diagnosis following reinvestigation, while the results of both reFNAB and incisional biopsy were inconclusive in the third. CONCLUSION: In the majority of the 38 patients, a definitive diagnosis of lymphoma could be made based on FNAB alone, using cytomorphological and immunological workup, and subclassification was possible in 20 patients (65%). Primary low-grade malignant orbital lymphomas are traditionally treated with low-dose radiotherapy regardless of subtype, and incisional biopsy was not needed to initiate treatment. Our findings suggest that FNAB is a valid first option for the diagnosis of suspected orbital lymphomas due to the minimal risk of complications compared to incisional biopsy, and the fact that it can be performed as an outpatient procedure with no anesthesia.

Comprehensive analysis of orbital lymphoma in a Turkish cohort: clinical characteristics, histological subtypes, treatment modalities, prognostic factors, and implications for management.

The study analysed the clinical characteristics, treatment approaches, and survival outcomes of 97 consecutive patients with orbital lymphoma (OL) over a 25-year period at. The median age of the patients was 57.6 years, and 59.8% (n = 58) were male. Marginal zone lymphoma constitutes the most prevalent subtype, accounting for 67% of cases, whereas other common subtypes include diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, and T-cell lymphomas. Unilateral involvement was observed in the majority of cases (72.3%). Common clinical presentations included mass (30.9%), swelling (26.8%), and epiphora (11.3%). Of the patients, 7.2% received rituximab alone, 14.4% received radiotherapy alone, 48.5% received chemotherapy, 27.8% received radiotherapy plus rituximab, 22.7% received radiotherapy plus chemotherapy, and 5.2% underwent surgery as the first-line treatment. During a median follow-up of 4.3 years, 15.5% of patients experienced relapse or disease progression. The 5-year and 10-year progression-free survival rates were 84.1% and 79.1%, respectively. This study contributes to our understanding of OLs and provides a foundation for further investigations in this field. Male gender, presence of B symptoms, advanced stage, secondary orbital lymphoma, aggressive histological subtype, and elevated serum lactate dehydrogenase levels were associated with poorer (either inferior or worse) progression-free survival.

Primary unilateral conjunctival small lymphocytic lymphoma in a child: Case report and literature review.

Ocular adnexal lymphomas (OALs) are extranodal lymphomas primarily involving the ocular adnexa, which includes the conjunctiva. OALs are considered primary if the involvement of lymphoma is confined to the ocular adnexa alone and secondary if there is lymphoma of the identical type present at another site. Conjunctival lymphomas are uncommon in children, with very few reported cases in published literature, none of which were small lymphocytic lymphomas (SLLs). We report a case of SLL confined to the conjunctiva, in a 15-year-old child, with full pediatric oncology examination, including full body positron emission tomography (PET) scan, revealing no other site involvement. To our knowledge, this is the first case to be reported of a child with primary unilateral conjunctival SLL.

Orbital mass as the presenting symptom of diffuse large B-cell lymphoma causing spontaneous tumor lysis syndrome and rapid multisystem organ failure.

A 68-year-old male presented with a one-month history of progressive proptosis and vision loss in the left eye. Examination of the left eye showed visual acuity of NLP, marked relative proptosis of 10 mm, and complete external ophthalmoplegia. CT orbits showed an extensive left orbital lesion with proptosis. Urgent orbital biopsy was undertaken. Intraoperatively, the patient developed new atrial flutter and fever. Bloodwork revealed metabolic derangements suggestive of tumor lysis syndrome. Systemic evaluation revealed a large tumor burden involving the retroperitoneal space. Histopathology of the orbital specimen showed non-germinal center diffuse large B-cell lymphoma. The patient passed away 3 days postoperatively due to rapidly progressive multisystem organ failure. Our case demonstrates an unusually aggressive presentation of DLBCL in which orbital mass was the first presentation of spontaneous tumor lysis syndrome owing to large systemic tumor burden.

Simultaneous regression of non-small cell lung cancer and orbital extranodal marginal zone lymphoma with chemoradiotherapy for lung cancer.

This report describes the case of a 79-year-old Japanese man diagnosed with orbital extranodal marginal zone lymphoma (EMZL) and stage IIIA lung cancer. The patient received concurrent chemoradiation for lung cancer with carboplatin/paclitaxel treatment, resulting in regression of both the lymphoma and lung cancer. To our knowledge, this is the first reported case of concurrent orbital EMZL and lung cancer. In this case, a treatment strategy that prioritized lung cancer treatment was deemed appropriate. This case suggests that chemotherapy with carboplatin and paclitaxel may serve as an effective treatment for both lung cancer and lymphoma.

Orbital B-cell Lymphoma Masquerading As Dry Eye Disease.

Orbital lymphoma is a rare tumor with an incidence of 2.02 per million per year. It can occur as a part of systemic lymphoma or spontaneously arise primarily in orbit. The most commonly reported malignant lymphoma is non-Hodgkin's lymphoma. The typical clinical finding includes exophthalmos, swelling, and limited eye movement. Our patient presented with dryness and irritation of the eye without signs of proptosis until months later. The definitive diagnosis of orbital lymphoma requires a biopsy. Still, imaging studies such as MRI and CT scans play a critical role in distinguishing benign from malignant lesions and invasion of local tissues. The treatment of orbital lymphoma is multidisciplinary, involving surgical resection, radiotherapy, and chemotherapy depending on the histological type of tumor and the presence of metastasis.

Orbital Infiltration in a Patient with Waldenström Macroglobulinemia: Need for Multidisciplinary Approach and Comparison with the Literature.

The use of specific inhibitory drugs of intracellular signalling pathways (such as Bruton-Kinase inhibitors) for the treatment of Waldenström's macroglobulinaemia (WM) is a recognised risk factor for Aspergillus spp. infections. The overlapping clinical manifestations of the two diseases may require the involvement of different medical specialities. We describe the clinical course of a patient with pulmonary and encephalic aspergillosis, with concomitant orbital infiltration, which represented a difficult diagnosis: the case required a multidisciplinary approach to define the ocular lesions and an in-depth study of the literature.

Bilateral Large Orbital Lymphoma With Proptosis.

Diffuse large B-cell lymphoma (DLBCL) is the most common lymphoid malignancy in adults. It is an aggressive malignancy and requires a multidisciplinary approach with various modalities which include chemotherapy, radiotherapy as well as immunotherapy. A 63-year-old Malay male patient with underlying type 2 diabetes mellitus, hypertension, ischemic heart disease, and stage II chronic kidney disease presented with a one-month history of bilateral eye proptosis associated with lid swelling and red eye. He also complained of progressive right eye blurring of vision. Visual acuity was counting fingers on the right and 6/18 on the left. On examination, the relative afferent pupillary defect was negative. There was bilateral eye proptosis, conjunctival chemosis, and restricted extra-ocular movement in all gazes. There was also exposure keratopathy over the right eye, and intraocular pressure was raised. Bilateral cervical and axillary lymph nodes were palpable. A computerized tomography scan of the brain and orbit revealed bilateral orbital masses with no bony erosions. An incisional biopsy over the upper lid confirmed the diagnosis of diffuse large B-cell lymphoma with multiple myeloma-1 (MUM-1) positivity which defines the activated B-cell subtype (ABC). He was co-managed with a hematologist and was commenced on the rituximab-cyclophosphamide, doxorubicin, vincristine, prednisone (R-CHOP) chemotherapy regime. Bilateral eye proptosis, chemosis, and restriction of extra-ocular movement resolved after the completion of treatment. However, right eye vision remains poor as the patient developed central self-sealed corneal perforation with iris plugging which has healed with scarring. Diffuse large B-cell orbital lymphoma is a fast-growing and aggressive tumor, hence early diagnosis and prompt multi-disciplinary treatment are crucial for a good outcome.

Orbital Lymphoma Masquerading as Euthyroid Orbitopathy.

Thyroid eye disease (TED), also called Graves orbitopathy (GO), is the most common diagnosis of orbital tissue inflammation. It is typically associated with the onset of hyperthyroidism, an autoimmune response to excess amounts of thyroid hormone. However, a visible and palpable lump, strictly unilateral or gross asymmetric eye involvement, non-axial (eccentric) proptosis, a lack of lid retraction or lid lag on downward gaze, or weakened muscle function (suggestive of tendon involvement) are the key features of non-TED mediated ocular involvement, as was found in the case we report here. Orbital lymphoma should always be suspected and excluded in all cases of orbital inflammation. Our patient was diagnosed with mucosa-associated lymphoid tissue (MALT) lymphoma at 27 years of age, two years after the diagnosis of euthyroid ophthalmopathy. This case highlights the need to include space-occupying lesions in the differential diagnosis of proptosis and gaze restrictions, even in younger patients.

Diagnostic challenge: Diffuse large B-cell lymphoma masquerading as orbital cellulitis.

We report a clinical case of orbital lymphoma masquerading as orbital cellulitis. Orbital lymphoma and lymphoma of the orbital adnexa are relatively rare types of lymphoma. They represent the malignant end of a spectrum of lymphoproliferative lesions that occur in the orbit, conjunctiva and eyelid. Diffuse large B-cell lymphomas (DLBCL) with variable clinical presentations have a relatively poor prognosis with rapid visual loss. Therefore, thorough evaluation and assessment with a multi-specialty effort are warranted for earlier diagnosis and initiation of treatment to save life and prevent sight loss.

Ultra-low dose radiotherapy in the management of low-grade orbital lymphomas.

Background: Ultra-low dose radiotherapy (ULDRT) (2 × 2 Gy) has been used for symptomatic control of low-grade lymphomas with surprising local control rates, suggesting that these entities could respond to lower doses. These are particularly desirable for the treatment of orbital sites and some publications refer to high rates of complete responses. In this paper, we present our experience with the use of ULDRT for indolent orbital lymphomas. Materials and methods: Electronic files and treatment plans of patients treated with ULDRT for low-grade orbital lymphoma were retrospectively reviewed. Oncological outcomes and toxicities were collected and described for each patient. Results: Seven patients (median age of 75 years) with 8 lesions (3 follicular, 2 MALT, 1 marginal and 1 low-grade non-Hodgkin lymphoma) were considered for analysis. The majority had stage IE disease and one patient had bilateral disease. Six tumors were detected on imaging (median size of 20 mm). Involved orbital sites were periocular, conjunctival and palpebral; there was one case of intraocular (choroid) and one case of lacrimal gland involvement. One patient received consolidative rituximab after RT. The median follow-up time was 22 months. Two patients had partial response, one of them with persistent minimal choroidal disease and the other with partial response on CT. Five (71%) patients had clinical (n = 2) or radiologic (n = 3) complete response on treated sites. Reported late toxicities were minimal and included dry eye and pruritus. Conclusion: In our experience, ULDRT achieved a local control rate of 100% and complete response rate of 71% with minimal toxicity.

Primary diffuse large B-cell lymphoma of orbit: A population-based analysis.

Objective: Primary orbital lymphoma (POL) accounts for an essential part of adult orbital malignancies. Nevertheless, it remains a relatively rare lymphoid malignancy, accounting for <1% of all non-Hodgkin's lymphoma (NHL) cases. Orbital diffuse large B-cell lymphoma (DLBCL) is one of the most prevalent subtypes of POL that confers the worst outcomes. The prognostic determinants of orbital DLBCL remain unknown. Therefore, a retrospective analysis was conducted by investigating the Surveillance, Epidemiology, and End Results (SEER) database for independent predictive factors for the prognosis of orbital DLBCL. Materials and methods: Using the SEER program, we acquired patient data including demographics, clinical characteristics, and treatment strategies. Our cohort included cases of primary orbital DLBCL diagnosed from 2000 to 2017. We conducted Kaplan-Meier analyses to visualize the overall survival (OS) and cause-specific survival (CSS). The Cox proportional hazard regression models were applied to assess the effects of these prognostic factors on OS and CSS. Results: The present cohort included 332 patients with orbital DLBCL. Age was the most impacted variable by orbital DLBCL. Three independent prognostic variables of orbital DLBCL were identified on diagnosis: advanced age, no radiation treatment, and late-stage (Stage IV). Moreover, patients who underwent chemotherapy demonstrated a greater OS when compared with those who did not. In orbital DLBCL, being unmarried was also a poor prognostic factor. Conclusion: The current study is the largest population-based case series of orbital DLBCL. The age at the time of diagnosis, marital status, absence of chemotherapy or radiotherapy, and tumor stage were all found to be correlated with worse prognosis.

Beware the Retrobulbar Optic Neuritis Diagnosis.

A 48-year-old gentleman presented to the ophthalmology department with progressive monocular vision loss, a relative afferent-pupillary defect, decreased color perception, headache, proptosis, and retro-orbital pain. This particular patient's demographics and disease course did not suggest a "typical" retro-bulbar optic neuritis and highlights the importance of avoiding presumptive steroid treatment in such "atypical" cases. Further investigations revealed a compressive optic neuropathy secondary to an orbital tumor (B-cell non-Hodgkin's lymphoma) and were subsequently treated by a multi-disciplinary approach. Early detection and commencement of treatment is a crucial determining factor in orbital lymphoma prognosis and is therefore an important differential diagnosis for an ophthalmologist to consider when evaluating patients with "atypical" optic neuropathies.

Long-term follow-up and health-related quality of life among cancer survivors with stage IEA orbital-type lymphoma after external photon-beam radiotherapy: Results from a longitudinal study.

We assessed the long-term outcomes and treatment-related adverse effects of patients with Stage I, "orbital-type" lymphomas that were uniformly treated with photons. All consecutive patients diagnosed with low-grade, Ann Arbor Stage IEA orbital lymphoma treated between 1999 and 2020 at our department were retrospectively reviewed. We excluded patients with exclusive conjunctival involvement, typically treated with en face electrons. In order to quantify radiotherapy related side effects we applied the CTCAE criteria, analyzed changes in visual acuity, quantified dry eye symptoms by use of the Ocular Surface Disease Index (OSDI) score and applied the EORTC QLQ-C30 questionnaire for quality of life (QoL) assessment. In total 66 eyes of 62 patients were irradiated with a median dose of 30.6 Gy. The median follow-up was 43.5 months. The predominant histological subtype were MALT lymphomas. No local failure occurred in this cohort. Of nine outfield relapses, six solely occurred in the contralateral eye. The 5- and 10- years distant progression free survival rates (PFS) were 81.4% and 63.5%. The 5- and 10-years overall survival rates were 85.1% and 71.9% without any tumor related death. Of the acute toxicities none was higher than CTCAE grade 1. The predominant late toxicities were dry eyes (21.2%) of CTCAE Grade <2 and radiation induced cataracts (19.7%). During long-term follow up the average visual acuity did not deteriorate. The global QoL was worst before treatment and improved significantly after 24 months (p = 0.007). External beam radiotherapy of "orbital-type" lymphomas with photons is an effective and gentle treatment option with excellent local control rates. From the high control rates the trend to use slightly lower total doses of 24-27 Gy with conventional fractionation is supported. As non-coplanar radiotherapy techniques improved and total doses can slightly be reduced, the current status of radiotherapy as first line therapy is provided.

Atypical Posterior Reversible Encephalopathy Syndrome in Intraorbital Lymphoma after Tumor Biopsy: An Illustrative Case.

Introduction A 63-year-old male presented with visual loss and left eye proptosis. Magnetic resonance imaging revealed a left orbital tumor, measuring 1.4 cm × 0.9 cm. The patient underwent left frontotemporal craniotomy to perform a biopsy of the tumor. During the postoperative period, the patient developed the first episode of a generalized tonic-clonic seizure. Case Summary Computed tomography of the brain showed hypodensity of the bilateral basal ganglia and thalami with associated edematous white matter hypodensity of bilateral temporo-occipital lobes compatible with atypical posterior reversible encephalopathy syndrome (PRES). The patient received antiepileptic medication and was observed for clinical seizure. One week later, computed tomography of the brain showed the reversible process of PRES. The pathology report revealed diffuse large B cell lymphoma. Following pathological diagnosis, the patient received treatment with whole-brain radiotherapy. Conclusion This is the first reported case of atypical PRES associated with orbital lymphoma following craniotomy for the purpose of tumor biopsy. Early detection as well as seizure and blood pressure control, is essential for the proper treatment of PRES.

Chronic Inflammatory Orbitopathy Hiding Orbital Lymphoma.

The incidence of lymphoma is constantly increasing worldwide. The reasons for this increase are unclear and likely multiple. B cell lymphomas represent the majority of non-Hodgkin lymphomas. Primary orbital localization remains a rare entity to think about in order to avoid missing a therapeutic emergency. In this article, we report the case of a 52-year-old man who has been treated for five years for an inflammatory orbitopathy with steroids, but the worsening of the clinical condition and the installation of exophthalmia led to push investigations and the outcome was a primary orbital lymphoma marginal zone type. The patient was treated by systemic chemotherapy with immunotherapy (RCHOP protocol) with a very good evolution and complete disappearance of the lesion after chemotherapy.

Successful Use of Intravitreal Bevacizumab and Methotrexate in a Case of Neovascularization of the Iris and Pseudohypopyon Secondary to Recurrent Diffuse Large B-Cell Lymphoma.

In this case report, we aim to describe a rare case of recurrent diffuse large B-cell lymphoma (DLBCL) reportedly in remission presenting with primary anterior segment findings and use of intravitreal bevacizumab and methotrexate to treat the sequelae. The patient presented with hypopyon and neovascularization of the iris (NVI). Anterior chamber studies including flow cytometry and imaging revealed DLBCL recurrence with central nervous system (CNS) involvement. Over one month, he was treated with one intravitreal injection of bevacizumab, repeat injections of methotrexate, and systemic therapies with the resolution of ocular symptoms but persistent systemic disease. This case highlights the utility of anterior chamber paracentesis in diagnosis and intravitreal bevacizumab and methotrexate in the treatment for anterior segment manifestations of intraocular lymphoma (IOL).

Bilateral lacrimal glands and paranasal sinus diffuse large B-cell lymphoma following lung mucosa-associated lymphoid tissue lymphoma in one patient.

We report an atypical case of diffuse large B-cell lymphoma (DLBCL) of bilateral lacrimal glands and paranasal sinus following mucosa-associated lymphoid tissue (MALT) lymphoma of the lung. Bilateral DLBCL is rare in the literature, and only few cases of DLBCL in bilateral lacrimal gland are reported. A 71-year-old male presented with bilateral, slowly enlarging, and swelling of both eyelids. Computed tomography scan images showed bilateral symmetric, hyperdense, circumferential masses over lacrimal glands occupying most of the orbital compartment. Neither optic nerve involvement nor adjacent orbital walls erosion was noted. Bilateral excisional biopsy and pathological examination confirmed the diagnosis of DLBCL. Since DLBCL of bilateral lacrimal gland can occur in case of systemic MALT lymphoma, excision and pathological examination is mandatory, and further metastatic workup is essential.