Orbital Lymphoma Masquerading as Orbital Cellulitis

Ocular Immunology and Inflammation, 2010

Purpose: To describe a case of orbital mucosa-associated lymphoid tissue (MALT) lymphoma masquerading as unilateral panuveitis. Methods: Retrospective chart review. Results: A 53-year-old female patient with unilateral vitritis and exudative retinal detachment refractory to immunosuppressive treatment was eventually diagnosed with orbital MALT lymphoma. Following treatment with radiotherapy and rituximab, the patient's intraocular inflammation and retinal detachment resolved. Conclusions: Orbital MALT lymphoma can masquerade as refractory unilateral panuveitis with exudative retinal detachment and appears to respond to a combination of radiotherapy and specific B-cell-targeted systemic therapy.

Purpose: A case of B-cell non-Hodgkin lymphoma is presented in the right orbit, which, after treatment with excisional biopsy plus chemotherapy, reached a favorable resolution after 9 months of treatment. Case report: 83-year-old male, who at physical exam presents with infraorbital proptosis at the right eye and a 1 cm mobile mass in the right orbit. An excisional biopsy via transconjunctival inferior orbitotomy showed a non-Hodking B cell lymphoma. Discussion: Surgical resection plus chemotherapy gave a favorable outcome at follow-up. The fundamental pillars for a good therapeutic approach is the correct histological classification, and the timely multidisciplinary management in this type of diagnosis. Conclusion: The correct histological classification and appropriate multidisciplinary management of these patients are the fundamental pillars of good therapeutic results.

Acta Medica Bulgarica, 2020

Background and purpose: The occurrence of primary orbital lymphoma comprises approximately 1% of non-Hodgkin’s lymphoma and 8% of extranodal lymphoma. The vast majority of orbital lymphomas are of B-cell origin, of which extranodal marginal zone B-cell lymphoma is the most common subtype. The purpose of this paper was to present the diagnostic challenges in a case of orbital lymphoma. Case presentation: An 84-year -old woman with orbital tumour was operated on after a long period of inappropriate treatment. It was later diagnosed as B-cell lymphoma. Conclusion: Orbital lymphoma can be easily mistaken for another ocular disease due to the slowly progressing non-specific complaints of the patients. We should be alert to the possibility of this ocular diagnosis when we are presented with an elderly patient with proptosis.

Cancer, 1980

Ophthalmic Plastic & Reconstructive Surgery, 2007

We describe an unusual case of composite lymphoma (CL) in the orbit. The clinical history and biopsy specimen of an 82-year-old woman with a right orbital mass were evaluated. The orbital biopsy contained a dense lymphocytic infiltration and nodules of large lymphocyte that immunostained positive for CD20. Flow cytometric immunophenotyping showed two distinct populations of cells, confirming the diagnosis of a simultaneous follicular lymphoma (FL) and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). The patient was treated with CD20 antibody (rituximab) and is in remission at 3 months follow-up. CASE REPORT An 82-year-old woman had a 6-to 8-week history of a progressively enlarging mass in her right lower eyelid. She suffered from dementia after a recent intracerebral hemorrhage due to amyloid angiopathy. She was also taking medications for hypothyroidism, asthma, and low back pain after a compression fracture. Her best corrected visual acuity was 20/50 OU, and examination of the left eye was normal. Examination of the right eye showed a firm, non-tender, movable 30 ϫ 22 mm mass in the lower eyelid/anterior orbit (Fig. 1A). MRI of the orbit showed an enhancing 17 ϫ 34 ϫ 25 mm mass located within the right orbit with anterior and inferomedial to the right globe, molding around the contour of the globe (Fig. 1B). The orbital mass was biopsied and submitted for histologic evaluation and flow cytometric immunophenotyping. Histologic examination showed a dense lymphocytic infiltration in the tissue (Fig. 2A). The infiltrate included small lymphocytes and large lymphocytes with open chromatin, prominent nucleoli, and abundant cytoplasm (Fig. 2B). Flow cytometry revealed two distinct populations of cells that were not related to each other. One population was approximately 40% of the total sample and showed CD5, CD19, CD20, and CD23 positive cells that were consistent with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Immunohistochemical staining demonstrated these CD5-positive lymphocytes (Fig. 3A). The second population made up about 20% of the sample and was CD10, CD19, and CD 20 positive with Lambda restriction compatible with low grade follicular lymphoma (FL). Immunohistochemical staining demonstrated these CD10-positive lymphocytes (Fig. 3B). Based on these data, a diagnosis of combined lymphoma (CL) consisting of FL and CLL/SLL was made. Abdominal and chest CT revealed the enlargement of several lymph nodes located in the upper abdomen and right lung. Bone marrow biopsy showed findings consistent with CLL/SLL. The patient was diagnosed as having stage IV lymphoma. Considering the patient's general medical condition, monoclonal CD20 antibody (rituximab) chemotherapy was utilized for control of the disease. Three courses of rituximab (525 mg/week for 4 weeks per cycle) were given. The patient was in complete remission 3 months after the end of therapy.

Ophthalmology Research: An International Journal

The diagnosis of lymphoproliferative disorder of orbit is quite challenging as both Pseudolymphomas and Non‑Hodgkin’s lymphomas can occur in orbit. Primary orbital lymphoma is a rare entity comprising of 1-2% of Non‑Hodgkin’s lymphoma, majority of them are B‑cell type. It is a slow growing tumor. It presents in the age group of 50–70 years, with a slight female preponderance. Proptosis is the most common presentation. We, herein, report a case of 48 years old male presenting with right non-axial proptosis, watering of eye, blurring of vision and redness for the last 9 months. After thorough clinical and radiological evaluation, a biopsy was taken for histopathological examination. A primary diagnosis of Lymphoproliferative Disorder was made. Following this, a panel of immunohistochemical markers was applied and a final diagnosis of Non-Hodgkin Lymphoma, B-cell type was given.

British Journal of Ophthalmology, 2002

A 55-year-old albanian male was referred to the Ophtalmology Department in Mother Theresa University Hospital Tirana, with blurried vision, painful proptosis, limitied eye movements diplopia and chemosis of the right eye for three days. Two years ago he was diagnosed with orbital cellulitis. Over the next 72 hours, he did not clinically improve as it was expected. a diffuse infiltrative mass of orbital muscles and fat on the right eye with exophtalmy of this side that suggested for infiltrative tumor. Based on the results of radiology, it was required biopsy. Histopathology revealed the presence of lymphocites, plasma cells, myofibrolastic cells and collagen, with diagnose of inflammatory chronic moderated lesion. Given the negative workup, the presentation was determined to be consistent with idiopathic orbital inflammation via a diagnosis of exclusion. Therefore, the patient was treated with intravenous steroids that produced pronounced improvement within 24 hours. The patient was discharged in improved condition with a prednisone taper and ophtalomoly follow-up. Orbital pseudotumor if often misdiagnosed as orbital cellulitis, because of the similar presentation. It is important to consider differential diagnose when the initial diagnose and treatment does not progress as we expect.

World Journal of Surgical Oncology, 2013

Background: Lymphomas of the orbit and orbital adnexae are rare tumors, comprising only 1% of all non-Hodgkin's lymphoma. The majority of non-Hodgkin's lymphomas of the orbit are extranodal marginal-zone B-cell lymphomas of mucosa-associated lymphoid tissue type. Because of nonspecific clinical signs and symptoms, some diagnostic delay may occur. The purpose of the study was to evaluate the diagnostic approach in orbital lymphomas and to analyze their treatment outcome. Methods: In the period from 2005 to 2012, from a group of 135 patients with tumors of the orbit, we identified 11 patients diagnosed with orbital lymphoma. This patient cohort was reviewed retrospectively. Results: The patient group consisted of 11 patients (seven females, male males) with a median age of 57.7 years (range 42 to 88 years). Orbital swelling, pain and motility impairment were the leading clinical symptoms. Diagnosis was confirmed by surgical biopsy. Depending on the anatomic location of the tumor, a surgical biopsy was taken using a blepharoplasty incision, a lateral orbitotomy or a navigation-guided biopsy. The predominant histology was extranodal non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue type (82%). All patients underwent complete clinical staging. These were clinical stage I EA in seven patients, and stages II EA (n = 2) and III EA (n = 2) in four patients. Patients in stage I EA were treated with radiation therapy alone, with radiation doses between 25 and 40 Gy, and patients with stage II EA received systemic chemotherapy with bendamustin/rituximab. Those two patients diagnosed with diffuse large B-cell lymphoma and mantle cell lymphoma received systemic chemotherapy according to the R-CHOP protocol. Conclusions: Owing to unspecific clinical symptoms, some diagnostic delay may occur in orbital lymphoma. If unspecific orbital symptoms are present, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis. Once diagnosis is established and staging is complete, radiation therapy is the recommended treatment for stage I EA patients. Systemic chemotherapy is indicated in selected stage II EA patients and in patients with stage III EA disease.

Ophthalmic Plastic & Reconstructive Surgery, 2004

Spontaneous and complete regression of malignant neoplasms is extremely unusual. To our knowledge, this case report is the first description of spontaneous regression of an extranodal malignant lymphoma occurring in the conjunctiva and orbit. A 40-year-old woman noticed a pink conjunctival mass at the medial aspect of her left eye that had been present for 3 weeks. She presented on May 5, 2003. Ophthalmologic examination showed a salmon-colored mass along the lateral side of the caruncle. CT revealed a mass in the medial orbit. Surgical biopsy exhibited a malignant lymphoma, diffuse large B-cell type. After biopsy, the tumor spontaneously decreased in size and completely disappeared in 5 weeks. At 6 months' follow-up, the tumor had not recurred. FIG. 1. A and B, Conjunctival tumor with salmon-pink appearance on the left eye of the patient. C and D, Five weeks later, the tumor completely disappeared after an incisional biopsy.

British Journal of Ophthalmology, 2001

Current Opinion in Ophthalmology, 2007

Purpose of review Malignant lymphoma of the ocular adnexa has been reported on for many years, but many steps forward have been recently made. This paper highlights the staging strategies and treatment options based on a review of the most updated and relevant bibliography. Recent findings A relevant improvement in the management of ocular adnexal lymphoma is represented by PET, which improves the diagnosis and the staging of the disease. Acquisitions have been made in the treatment: low-dose radiotherapy is confirmed for primary orbital mucosa-associated lymphoid tissue lymphoma; oral chlorambucil is proposed as an alternative; immunotherapy is proposed for the treatment of systemic disease. Interestingly, the role of Chlamydia psittaci as the possible cause of mucosa-associated lymphoid tissue lymphomas and the efficacy of doxycycline for the treatment of ocular adnexal lymphomas have been investigated with promising results. A large series of natural killer/T-cell lymphoma has been described and its lethality despite aggressive conventional chemotherapy has been confirmed. Summary Orbital lymphoma is the most common malignant tumor of the orbit and its incidence is increasing proportionally with the rise of the average survival rate of the general population. The combined efforts of orbital surgeons, hematologists, oncologists and radiotherapists have lately produced a mass of new information that can effectively improve the management of orbital lymphoma. Keywords lymphoproliferative disease of the orbit, orbital lymphoma, staging of orbital lymphoma, therapy for orbital lymphoma

Annals of African Medicine, 2007

Background: Ophthalmic involvement in lymphoma is a relatively rare condition that can result from a primary intraocular lymphoma or an intraocular manifestation of systemic lymphoma. This report reviews the ophthalmic manifestations of lymphoma. Methods: Review of relevant information from journal articles and Internet search. Results: Almost all the structures in the orbit, adnexiae and eye can be involved in lymphoma. Lymphoma of the eye and adnexiae are most frequently of B lineage. Most of the ocular manifestations frequently masquerade as other more benign intraocular conditions including allergic or infectious conjunctivitis, uveitis, multiple evanescent white dot syndrome, acute retinal necrosis or herpetic retinitis. Correct diagnosis thus depends on a high index of suspicion and frequently requires radiologic imaging, histologic analysis, particularly vitreous biopsy or flow cytometry, subretinal aspiration and retinal biopsy. Diagnosis is often delayed and may lead to a fatal outcome. Recognition of its modes of presentation facilitates early diagnosis and treatment that may improve prognosis. Conclusions: It is important to review the ocular manifestations of lymphoma to assist the ophthalmologist to play a pivotal role in the prompt diagnosis and treatment of ocular lymphoma, and the haematologist/oncologist to recognize the need for a complete ophthalmic evaluation in the diagnosis, follow-up and management of lymphoma patients.

International Journal of Research Publications

Orbital lymphoma represents a small fraction of all systemic lymphomas that has been accounted for approximately in 1-2% of non-Hodgkin lymphomas (NHL). Some diagnostic delays might be occurred due to the fact that there are non-specific clinical signs. Furthermore, an orbital CT scan can determine the location of the orbital mass and assist in ophthalmological surgery as well as to obtain an optimal sample for an accurate diagnosis. CT scan was performed and revealed the pattern of orbital lymphoma in order to pursue the proper diagnosis. In accordance to the imaging result, immunohistochemical studies were performed from biopsy specimens of all three patients. It showed that there were a non-Hodgkin's lymphomas originating in the orbit, lids, and oculi adnexa.

Acta Ophthalmologica Scandinavica, 2004

Background: The concomitant occurrence of both intraocular and periocular lymphomas is extremely rare. Periocular involvement by lymphoproliferative disease ranges from benign lymphoid hyperplasia to malignant lymphoma. Intraocular lymphomas usually appear in conjunction with primary central nervous system lymphoma. Case Reports: We describe clinical characteristics, standardized A-and B-scan ultrasonography, colour Doppler, computerized tomographic and magnetic resonance imaging, and immunohistological findings in three cases of concurrent choroidal and periocular involvement of lymphoma. Discussion: The clinical presentation of diffuse choroidal tumours may be variable, making diagnosis of intraocular lymphoma troublesome on clinical grounds alone. The high accuracy of colour Doppler imaging is known to be effective in differentiating the benign from the malignant and adds valuable information in the differential diagnosis of a low reflective lesion. We suggest the use of auxiliary examinations such as ultrasonography and colour Doppler imaging to help in the differential diagnosis of choroidal and orbital tumours.