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2013, World Journal of Surgical Oncology
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.
European Journal of Haematology, 2004
Extranodal presentation occurs in about 40% of patients with non-Hodgkin's lymphoma (NHL), and the orbit accounts for 5-14% of all cases (1). According to the Revised European-American Lymphoma (REAL) classification (2), the commonest subtype of lymphoma originating in the orbit is the extranodal marginal zone lymphoma (EMZL) of B-cell origin. When encountered in relation to an epithelial surface or glandular organ, it is thought to originate from mucosa-associated lymphoid tissue (MALT) (3). MALT can arise from the conjunctiva and lacrimal gland. However, some deep orbital MZL do not appear to have a conjunctival component. The REAL classification proposed the term extranodal marginal zone B-cell lymphoma to incorporate both the mucosa-and non-mucosa-associated MALT lymphomas (2). Other indolent lymphomas represent follicular, small B-lymphocytic, and mantle cell lymphomas. The commonest aggressive type is the diffuse large B-cell lymphoma (DLCL) (1). EMZL carries a better prognosis than other histologic types. Eighty-five percent of orbital lymphoma is of small cell (indolent) histology. In a large series of 212 patients (1) the frequency of EMZL was 43%, of lymphoplasmacytic/lymphoplasmacytoid lymphoma (LPL) 23%, of follicular lymphoma (FCL) 14%, of DLCL 10%, and of other types 10%. EMZL and diffuse LPL have a similar risk of dissemination at diagnosis, but the risk is increased in patients with FCL (4). DLCL is associated with a higher risk of progression and death. Seventy-five percent of orbital lymphomas appear hyperintense to fat on T2-weighted images and become brighter relative to their appearance on T1-weighted images. Eighty-five percent of orbital inflammatory syndrome appears isointense to fat on T2-weighted images and become slightly darker or unchanged relative to their appearance on T1-weighted images (5). Tumor density and enhancement are not useful for differentiation. This report summarizes a retrospective analysis of 15 orbital lymphomas treated at the Neurosurgical Clinic of Duisburg over a 9-yr period. The
International Journal of Radiation Oncology Biology Physics, 1995
Graefe's archive for clinical …, 1991
We investigated the clinicopathologic characteristics of 17 patients (13 men and 4 women) with primary orbital malignant lymphoma using the Working Formulation. Most of the cases belonged to the lowgrade malignancy group, and more women than men were in the histologically high-grade malignancy group. The phenotype of the tumor cells was investigated immunohistochemically. All cases showed the monoclonal feature of a B-cell lineage. All patients received chemotherapy with or without radiotherapy. Of 36 subjects, 15 achieved a complete remission; none of these patients has had a recurrence since the completion of the initial therapy (range of follow-up from 16 months to 10 years). One patient died.
Radiation Oncology, 2009
Background: Primary orbital lymphoma is a rare disease that accounts for 10% of all orbital tumors. Radiotherapy on the orbital cavity is the treatment of choice for this unusual presentation of localized non-Hodgkin's lymphoma (NHL). The aim of this study is to retrospectively evaluate the effectiveness and the toxicity of radiation treatment in patients with primary orbital lymphoma.
Ophthalmology, 2008
Objective: To evaluate the risk for systemic lymphoma (SL) in the patients with orbital lymphoproliferative tumor (OLT).
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.
Journal of Oral and Maxillofacial Surgery, 2008
Purpose: To present a treatment plan for localized mucosa-associated lymphoid tissue lymphoma of the orbit in order to preserve vital structures and function from the side effects of radiotherapy. Materials and Methods: Study of 2 clinical cases by means of clinical and radiologic examination, with 2 and 3 years follow-up, respectively. Results: Immediate remission of the disease after 6 cycles of chemotherapy, with no signs of recurrence after 2 and 3 years. Clinical examination of the oculomotor mechanism and visual activity gave excellent results. Conclusion: Although radiotherapy is preferable for localized lymphoproliferative lesions, chemotherapy should also be considered as an effective treatment that preserves the integrity and function of the ocular adnexa.
Orbit, 2001
Primary orbital lymphoma is a rare condition involving primarily the lacrimal glands. We present the second Indian case of right-sided primary orbital lymphoma in an elderly male who presented with the typical features and showed a good response to chemotherapy.
Tumori Journal, 1997
Aims and Background The orbit is an uncommon primary site for non-Hodgkin's lymphomas (NHL), and it accounts for less than 1% of all sites of primary presentations. We report the experience of the Department of Radiation Oncology at Ankara University Faculty of Medicine with radiation therapy in treatment of patients with stage I primary orbital NHL. Methods From February 1978 through August 1993, 14 patients with stage I primary orbital NHL were treated with radiation therapy. According to the Working Formulation classification, 8 patients had low-grade and 6 had intermediate-grade lymphomas. The most commonly used radiation therapy technique was a single anterior field with a Cobalt-60 unit, delivering 40 Gy in 2 Gy daily fractions. Two patients with intermediate-grade lymphomas received the CHOP regimen following radiation therapy. Results Follow-up ranged from 0.8 to 18.3 years (median, 10.3 years). Local control was achieved in all patients. Two patients with low-grade lymp...
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.
Lymphoma of the orbit is a rare presentation of non- Hodgkin's lymphoma (NHL). According to the data from the literature, orbital lymphoma represents 8% of all extranodal NHL and about 1% of all NHL [1-3]. Symptoms which are usually caused by orbital lymphoma are proptosis, periorbital swelling, conjunctival (salmonpink) swelling, diplopia, and conjunctival redness and irritation [4]. Radiotherapy is widely used as the primary treatment for orbital lymphoma. Radiation in those cases is often technically challenging, because special effort is made to preserve the integrity of the orbit without compromising local control [1]. The studies have shown that a local control rate is 95-98% for low grade orbital lymphomas [1,5]. The localized primary orbital lymphomas respond well to radiotherapy. The advantages of the radiotherapy treatment are a high-local control rate and durable disease-free status. Carefully planned orbital radiotherapy is well tolerated by patients and obligatory use of a lens shield contributes to greatly reducing the incidence of complications.
Journal of the Egyptian National Cancer Institute, 2015
To analyze the clinical outcome of primary orbital lymphoma (POL) patients treated with a combined modality approach with local radiotherapy after induction chemotherapy. Methodology: We retrospectively retrieved demographic, treatment and outcome data of patients treated for POL from 2000 to 2010. The charts were reviewed and the data were tabulated in a predesigned pro-forma. Results: 23 patients of POL were found evaluable. Median age was 55 years (range 24-70 years). Of 23 patients, 15 were male and 8 female, making the male:female ratio approximately 1.9:1. Patients were thoroughly evaluated and staged. All but one patient received multi agent chemotherapy. Radiotherapy was delivered for all cases. Radiation was delivered by 3DCRT technique. Median dose of radiation was 45 Gy (range 20-45 Gy). Median follow up was 26.8 months. None of the patients had any evidence of local failure or systemic progression. Conclusion: A combined modality therapy with a combination of CHOP/COP based chemotherapy and moderate dose of radiotherapy imparts excellent long term local and systemic disease control.
International Journal of Radiation Oncology Biology Physics, 1999
Purpose: This retrospective study reviews the treatment technique, disease outcome, and complications of radiotherapy used in the management of lymphoma involving the orbits. Patients & Methods: Thirty-eight patients were treated between May 1969 and January 1995, with a median follow-up of 8.3 years. All patients had biopsy-proven orbital lymphoma. Twenty patients who had limited disease were treated with curative intent, and 18 patients who had known systemic disease were treated with palliative intent. Of the 20 patients treated with curative intent, 14 had low-grade and 6 had intermediate-or high-grade disease. None received chemotherapy. Most patients received treatment with 250 kVP or 60 Co radiation, using either an en face anterior field or wedged anterior and lateral fields. Median treatment dose was 25 Gy. Lens shielding was performed if possible. For patients treated for cure, cause-specific survival and freedom from distant relapse were calculated using the Kaplan-Meier method. Results: Control of disease in the orbit was achieved in all but 1 patient, who developed an out-of-field recurrence after irradiation of a lacrimal tumor and was salvaged with further radiotherapy. In the patients treated curatively, the 5-year rate of actuarial freedom from distant relapse was 61% for those with low-grade and 33% for those with intermediate/high-grade disease (p ؍ 0.08). Cause-specific survival at 5 years was 89% for patients with low-grade and 33% for those with intermediate/high-grade disease (p ؍ 0.005). Two patients with low-grade disease had contralateral orbital failures; both were salvaged with further irradiation. Acute toxicity was minimal. Cataracts developed in 7 of 21 patients treated without lens shielding and 0 of 17 patients treated with lens shielding. No patient developed significant late lacrimal toxicity. Conclusion: Radiotherapy is a safe and effective local treatment in the management of orbital lymphoma.
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.
International Journal of Approximate Reasoning, 2020
We report the case of a fifty-two year old lady with a primary large Bcell Non-Hodgkin lymphoma, NHL, of the orbit which is a rare presentation of NHL. The tumour was amenable to chemotherapy and radiotherapy. Lymphomas are malignant neoplasm arising from the lymphoid cells (Kumar et al 2005). They are broadly classified into two groups namely the Hodgkin Lymphoma, HL and NHL. Lymphomas are not rare in occurrence; they are considered as the eighth and ninth leading cause of death due to cancers in women and men respectively in the United State in 2017 (Longo et al 2012). NHL occurs 10 times more frequently than the HL and in general has a poorer prognosis as compared to the latter (Cancer treatment center of America, 2017).
Reports of Practical Oncology and Radiotherapy
Background: The extranodal marginal-zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) is the most common orbital and adnexal lymphomas. Radiotherapy is one of the most preferred treatment options for orbital lymphomas since they are localized and radiation sensitive. The objective of this study is to evaluate how radiation therapy affected the outcome of orbital MALT lymphoma. Materials and ethods: PRISMA guideline was used to conduct this systematic review of electronic databases (PubMed, EMBASE and Cochrane Library), then we assessed the quality of evidence of each paper. Results: Twenty-five studies were finally included. 94% studies were intended for definitive therapy and almost all of the studies used external radiation sources. The total doses given to the tumor bed ranged from 4 Gy to 55 Gy and were divided into three groups: ultra-low dose (4-6 Gy), standard-dose (24-30.6 Gy), and high-dose (> 30.6 Gy). 75-90% patients showed CR and local relapse was only reported at 3.5-5%. Higher 5-year PFS was reported in the patients group with lens shielding (90.1% vs. 82.1%) and an increase in Meiboscore after RT courses. Toxicities, including dry eye and cataract, were reported in several patients. Acute toxicities subsided gradually over a few months with artificial tears. The risk of early cataract formation increases in patients who received > 30 Gy and lower in the IMRT group. Conclusion: RT is a successful primary definitive therapy for low-grade orbital MALT lymphoma, with a high survival rate, low recurrence rate, and typically acceptable toxicity.
Primary Non-Hodgkin's lymphoma of the orbit is a rare presentation and the diffuse large B-cell lymphoma (DLCL) type of histology is much less commoner than the mucosa associated lymphoid tissue (MALT) and follicular lymphoma. A 70 years female patient presented with palpable mass arising from right orbit and proptosis of the right eye. CT scan suggested homogenous enhancing soft tissue mass affecting right lacrimal region without any bony destruction. Biopsy confirmed it to be a case of Primary non-Hodgkin's lymphoma of diffuse large B-cell type with strongly positive CD 20. She was given 6 cycles of chemotherapy with R-CHOP after surgery. The patient is now asymptomatic one year after the last cycle of the chemotherapy.
International Journal of Radiation Oncology*Biology*Physics, 1984
Lymphoreticular tumors in tbe orbit are uncommon. Only 42 patients were identified from over 2000 patients with tumors of lymphoid tisue seen between 1958 and 1979. The patients were divided into 3 groups: primary malignant, secondary malignant, and benign lymphoma. In the primary malignant lymphoma groups, there were 24 patients with a median age of 64 (40 to 87) years and a 2.4:1 female to male ratio: 2 cases were bilateral. In I9 (79%) patients with the lymphoma limited to one orbit the tumor was controlled in every case with doses from 25 to 45 Gy. There were no serious complications. Subsequently lymphoma developed at other sites in 7 (37%) patients. The 5 and 10 year cause specific actuarial survival rates were 70 and 62%. In the 8 patients with secondary malignant lymphoma, the orbital disease was controlled by irradiation, although all patients required further treatment and died of their disease. Radiotherapy controlled all 7 patients with benign lymphoma without any complictitions.
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