Orbital Lymphoma Research Articles

Lymphoma and inflammation in the orbit: Diagnostic performance with diffusion-weighted imaging and dynamic contrast-enhanced MRI.

To investigate the diagnostic performance of diffusion-weighted imaging (DWI), dynamic contrast-enhanced (DCE) magnetic resonance imaging (MRI), and the combination of both in the differential diagnosis of lymphoma and inflammation in the orbit. This retrospective study was approved by the Institutional Review Board and the informed consent requirement was waived. A total of 53 patients underwent preoperative 3T MRI. Parameters of DWI and DCE MRI were evaluated in these 30 patients with orbital lymphoma and 23 patients with orbital inflammation. The diagnostic performance was evaluated using receiver operating characteristic curve analysis. Apparent diffusion coefficient (ADC) values and parameters derived from DCE MRI of orbital lymphoma and orbital inflammation differed significantly (ADC, Tmax , contrast index [CI], enhancement ratio [ER], and washout ratio [WR]: P < 0.001, P = 0.008, P < 0.001, P < 0.001, and P = 0.005 for reviewer 1, respectively; P < 0.001, P = 0.004, P < 0.001, P < 0.001, and P < 0.001 for reviewer 2, respectively). Sensitivity, specificity, and accuracy values of DWI were 76.67%, 100%, and 86.79% for reviewer 1; 70%, 95.65%, and 81.13% for reviewer 2, respectively. The combination of both were 90%, 86.96%, and 88.68% for reviewer 1; 93.33%, 78.26%, and 86.79% for reviewer 2, respectively. The combination of both was significantly superior to DWI for differentiation of orbital lymphoma from orbital inflammation (P = 0.016 for reviewer 1; P = 0.001 for reviewer 2). The combination of DWI and DCE MRI can improve diagnostic performance in differentiating lymphoma from inflammation in the orbit compared with DWI alone. 3 J. MAGN. RESON. IMAGING 2017;45:1438-1445.

Radiothérapie des lymphomes malins non hodgkiniens localisés de type MALT (gastriques et de la région orbitaire)

Primary gastric and orbital MALT lymphomas are both low grade (indolent) B-cell non-Hodgkin's lymphomas. Traditionally, these tumors are radiosensitive and have a good prognosis. In localized orbital and stages IE–IIE gastric MALT lymphomas without Helicobacter pylori infection or in case of persistent H. pylori infection after eradication therapy, several retrospective studies have shown that radiotherapy was an effective and well-tolerated treatment.

Lymphoma of Ocular and Periocular Tissues - Clinicopathological Correlations

Lymphomas of the ocular adnexa and intraocular tissue include a wide range of lymphoproliferative neoplastic disorders. They are predominantly extranodal non-Hodgkin lymphomas (NHL). The World Health Organization (WHO) classification of lymphoid neoplasm and individual morphological, immunophenotypical, and molecular genetic features, indicate that they may be divided into B-cell (approximately 80 % of all NHL) and T-cell lymphomas (approximately 10-20 % of all NHL). The most common forms of ocular NHL are extranodal marginal zone lymphoma (EMZL) of the mucosa-associated lymphoid tissue (MALT-type), follicular lymphoma (FL), diffuse large B-cell lymphoma, and mantel cell lymphoma. The clinical signs and symptoms are usually very unspecific and depend on the location, size, and extent of the underlying lymphoma subtype. Typical low grade lymphomas have an indolent clinical course and often remain unrecognized for many years. On the other hand, high grade NHLs, such as DLBCL or MCL, are frequently aggressive, with rapid tumour growth and poor prognosis, despite early detection. Histopathology is still the gold standard in the diagnosis of ocular lymphomas. Basic understanding of the principal pathophysiological and clinical aspects of the development and progression of orbital and ocular lymphomas seems to be mandatory for optimal diagnosis and treatment and for improving survival and prognosis. Both residents in training and board certified ophthalmologists should be aware of these problems.

Comprehensive genomic profiling of orbital and ocular adnexal lymphomas identifies frequent alterations in MYD88 and chromatin modifiers: new routes to targeted therapies

Non-Hodgkin lymphoma of the orbit and ocular adnexa is the most common primary orbital malignancy. Treatments for low- (extra-nodal marginal zone and follicular lymphomas) and high-grade (diffuse large B-cell lymphoma) are associated with local and vision-threatening toxicities. High-grade lymphomas relapse frequently and exhibit poor survival rates. Despite advances in genomic profiling and precision medicine, orbital and ocular adnexal lymphomas remain poorly characterized molecularly. We performed targeted next-generation sequencing (NGS) profiling of 38 formalin-fixed, paraffin-embedded orbital and ocular adnexal lymphomas obtained from a single-center using a panel targeting near-term, clinically relevant genes. Potentially actionable mutations and copy number alterations were prioritized based on gain- and loss-of-function analyses, and catalogued, approved, and investigational therapies. Of 36 informative samples, including marginal zone lymphomas (n=20), follicular lymphomas (n=9), and diffuse large B-cell lymphomas (n=7), 53% harbored a prioritized alteration (median=1, range 0–5/sample). MYD88 was the most frequently altered gene in our cohort, with potentially clinically relevant hotspot gain-of-function mutations identified in 71% of diffuse large B-cell lymphomas and 25% of marginal zone lymphomas. Prioritized alterations in epigenetic modulators were common and included gain-of-function EZH2 and loss-of-function ARID1A mutations (14% of diffuse large B-cell lymphomas and 22% of follicular lymphomas contained alterations in each of these two genes). Single prioritized alterations were also identified in the histone methyltransferases KMT2B (follicular lymphoma) and KMT3B (diffuse large B-cell lymphoma). Loss-of-function mutations and copy number alterations in the tumor suppressors TP53 (diffuse large B-cell and follicular lymphoma), CDKN2A (diffuse large B-cell and marginal zone lymphoma), PTEN (diffuse large B-cell lymphoma), ATM (diffuse large B-cell lymphoma), and NF1 (diffuse large B-cell lymphoma), and gain-of-function mutations in the oncogenes HRAS (follicular lymphoma) and NRAS (diffuse large B-cell lymphoma) were also observed. Together, our study demonstrates that NGS can be used to profile routine formalin-fixed, paraffin-embedded orbital and ocular adnexal lymphomas for identification of somatic-driving alterations and nomination of potential therapeutic strategies.

18F-FDG PET/CT imaging on primary orbital lymphoma: report of two cases

18F-FDG PET/CT imaging on primary orbital lymphoma: report of two cases

The value of conventional MRI combined with diffusion weighted imaging in the differential diagnosis of orbital lymphoproliferative disorders

Objective To investigate the diagnostic value of conventional MRI combined with DWI in the differential diagnosis of orbital lymphoproliferative disorders (LPD). Methods As a retrospective study, 42 patients were enrolled, including 23 orbital lymphoma and 19 benign orbital LPD confirmed pathologically or clinically. These patients underwent conventional MR, DWI and contrast-enhanced MRI examination of orbit. Qualitative MR imaging features [location, margin, patient ratio of involved quadrants (≥2), signal intensity, flow void sign , sinusitis] and quantitative features [ADC and contrast enhanced ratio of the lesion to temporal muscle (CER)] were evaluated. Chi-square test and t test were used for the analysis of qualitative and quantitative features between lymphoma and benign orbital LPD groups, respectively. Multivariate Logistic regression analysis was employed to identify the significant variable for predicting malignant LPD. ROC analysis was used to evaluate the ability of the diagnostic models established based on identified variables. Results Flow void sign (9 lymphomas and 17 benign LPDs) and sinusitis (5 lymphomas and 13 benign LPDs) were statistically significant (P 0.05). Both ADC [(0.79±0.09)×10-3 mm2/s of lymphoma, (1.29±0.35)×10-3 mm2/s of benign LPD] and CER (1.43±0.17 of lymphoma, 1.79±0.31 of benign LPD) between the two groups had significant difference (t=-6.630, -8.257;P<0.01). Multivariate logistic regression analysis showed that ADC value, CER and flow void sign were significant variables for predicting malignant orbital LPD (P<0.05). ROC curve showed that ADC value was the most significant single variable in differentiating orbital LPD [threshold value, 0.74×10-3 mm2/s; areas under ROC curve (AUC), 0.97; sensitivity, 87.0%; specificity, 94.7%]. Combination of ADC and CER could further improve the specificity in differentiating benign from malignant orbital LPD (AUC, 0.90; sensitivity, 78.3%; specificity, 100.0%, accuracy 88.1%). Conclusions ADC, CER and flow void sign on T2WI were significant variables for predicting malignant orbital LPD. ADC value was the most significant single variable. Combination of ADC and CER could further improve the specificity in differentiating benign from malignant orbital LPD. Key words: Orbit; Lymphoma; Magnetic resonance imaging

Localized orbital adnexal mucosa-associated lymphoid tissue lymphoma treated with radiation and rituximab: a single institute experience.

e19034Background: Standard treatment for patients with orbital mucosa-associated lymphoid tissue (MALT) lymphoma is not established due to paucity of prospective trials, small numbers of patients i...

Recurrence patterns of mucose-associated lymphoid tissue lymphoma after definitive radiation treatment: A single center experience

Objectives: To evaluate the treatment outcomes in non-gastric and non-nodal mucose-associated lymphoid tissue (MALT) lymphoma patients treated by definitive radiation therapy (RT).Methods: A total of 134 patients were analyzed. The RT dose was 30.6 or 36 Gy.Results: The median follow-up duration for all patients was 51.1 months (range, 3.0–132.4 months). Among the 88 orbital MALT lymphoma patients, 12 had disease recurrence. There were 23 head and neck tumor patients. Two patients experienced relapse, all at out-of-field locations. Gastrointestinal MALT lymphoma was diagnosed in 13 patients, and three showed local (n = 1), distant (n = 1), or local/distant (n = 1) relapse.Conclusion: RT alone is highly effective in achieving local control and long-term survival in localized MALT lymphoma. In orbital MALT, excellent local control is achieved, and relapse is predominantly observed in the contralateral eye. Other head and neck lymphomas are also well controlled.

Radiotherapy of indolent orbital lymphomas

The aim of this work was to retrospectively analyze efficacy, toxicity, and relapse rates of conventional (CRT) and low-dose radiotherapy (LDRT) in patients with indolent orbital lymphomas. From 1987-2014, 45patients (median age 64years) with 52lesions of indolent orbital lymphomas were treated with CRT (median dose 36Gy, range 26-46Gy) and 7patients (median age 75years) with 8lesions were treated with LDRT (2 fractions of 2.0Gy). Median follow-up was 133months (range 2-329 months) in the CRT group and 25months (range 10-41 months) in the LDRT group. Overall response rates were 97.7 % (CRT) and 100 % (LDRT). The 2‑ and 5‑year local progression-free survival (PFS) rates were 93.5 and 88.6 %, distant PFS 95.0 and 89.9 %, and overall survival 100 and 85.6 % after CRT. In the LDRT group, 2‑year local PFS and overall survival remained 100 %, respectively, and distant PFS 68.6 %. Acute radiotherapy-related complications (grades 1-2) were detected in virtually all eyes treated with CRT. Cataracts developed in only patients who were irradiated with more than 34Gy. LDRT was well tolerated with only mild acute and late complications. Primary radiotherapy of indolent orbital lymphomas is an effective treatment with high response rates and excellent local control in CRT and LDRT. In combination with close follow-up, LDRT may be an attractive alternative since re-irradiation even with conventional doses is still feasible.

Place de la radiothérapie dans la prise en charge des lymphomes malins non hodgkiniens

The purpose of this review was to summarize recent data about lastest retrospective and prospective studies dealing with radiotherapy of non-Hodgkin lymphoma, in order to precise the schedule and the role of this treatment. A systematic review was done by searching studies on the website http://www.pubmed.gov (Medline) using the following keywords: radiotherapy, radiation therapy, non-Hodgkin lymphoma. The management of non-Hodgkin lymphoma varies a lot according to the histological type and stage. The dose of radiotherapy has been studied in only one randomized trial, which concluded that there was no difference between the low dose and the high dose arms. Radiotherapy is a very good option in follicular, cutaneous, digestive or orbital non-Hodgkin lymphoma. A recent post hoc analysis of randomized trials on radiotherapy for high-grade non-Hodgkin lymphoma strongly suggested a benefit of additional radiotherapy after chemotherapy in some situations. Radiotherapy of low-grade non-Hodgkin lymphoma is a very good option, while its use on high-grade non-Hodgkin lymphoma is sometimes recommended but further randomized trials are ongoing to better understand its role.

A Case of Primary Orbital Peripheral T-cell Lymphoma with Panniculitis-like Features

A Case of Primary Orbital Peripheral T-cell Lymphoma with Panniculitis-like Features

Ocular Adnexal Lymphoma: Clinical Presentation, Diagnosis, Treatment and Prognosis

Ocular adnexal lymphoma (OAL) is a rare tumor with an incidence of 0.2 per 1,00,000 individuals. However, it constitutes the most common orbital tumor especially in older population with an incidence ranging from 11% to 24%. OAL can involve conjunctiva, orbital soft tissues, eyelid, or adnexal structures such as lacrimal gland and lacrimal drainage system. The most common primary OAL is low-grade malignant extranodal marginal zone B-cell lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT) type. Though various classification and staging systems have been proposed for OAL, currently, American joint committee on cancer (AJCC) TNM (tumor node metastasis) staging is most commonly used. Most commonly, conjunctival lymphoma typically presents as ‘salmon-colored patch’ and orbital lymphoma presents as proptosis, which on imaging shows characteristic appearance of molding around the globe without any globe indentation or bone erosion. Orbital radiotherapy and systemic chemotherapy forms the mainstay of treatment. Newer treatment modalities include immunotherapy with interferon and anti-CD20 antibody rituximab that have been shown to be useful either when used alone or in combination with chemotherapy or as radio-immunotherapy.

Paying attention to the research on IgG4 related orbital disease

IgG4 related disease (IgG4-RD) is a systemic disease which was named and accepted in recent years.IgG4-RD is a multiorgan-involved disease, mainly including pancrease, parotid gland, bile duct, liver, etc.The study on the relationship between IgG4 and orbital disease is relatively few.In recent years, our study group aimed directly to the relationship between IgG4 and common orbital disease, including benign lymphoepithelial lesion of lacrimal gland, idiopathic orbital inflammatory pseudotumor, orbital lymphoma, etc.We found that IgG4 was associated with the pathogenesis and progression of these diseases.This article addressed systematically the pathogenesis of IgG4 related orbital disease and evaluated the values of IgG4 in diagnosis, differential diagnosis, treatment and montoring of these orbital diseases based on related literature and reseaching results from our study group, in order to deepen the recognition of IgG4 related orbital diseases in ophthalmologists. Key words: Orbital; Immunoglobulin; IgG4-related orbital diseases

Chemotherapy Alone Is Effective to Treat Localized and Disseminated Ocular Adnexa Lymphomas

Background:The treatment strategies for ocular adnexa lymphomas (OALs), including conjunctiva, orbital, and lacrimal gland lymphomas, remain controversial. In this study, we assessed the efficacies and outcomes of different modality treatment, including chemotherapy, radiotherapy, and surgery for OALs.Methods and Materials:Between Jan. 1990 to Dec. 2013, patients receiving first-line chemotherapy (oral alkylating agents, rituximab-based treatment, or CHOP [cyclophosphamide, doxorubicin, vincristine, and steroid]-like regimens), radiotherapy (30 to 50 Gy, in 1.8- to 2.0- Gy per daily fractions), and surgery for newly diagnosed OAL were included in this study. The clinicopathologic features, including patients' characteristics, primary lymphoma locations, pathology subtypes, treatment modality, event-free survival (EFS) following first-line therapy, and OS (OS) were analyzed.Results:There were 56 men and 37 women with median age of 58 years were included. Of them, 79 patients (85%) were diagnosed with low-grade mucosa-associated lymphoid tissue (MALT) lymphoma, 5 (5%) with high-grade transformed MALT lymphoma, and 9 (10%) with diffuse large B cell lymphoma. Orbit was the most common involved location (45 patients, 48%), followed by conjunctiva (35 patients, 38%), and lacrimal gland (13 patients, 14%). Of 79 patients with stage I-IIE1 disease, 22 received chemotherapy, 34 with radiotherapy, and 23 patients with other modality treatments (18 with surgery, 4 with combined radiotherapy and chemotherapy, and 1 with intra-lesional injection of rituximab). The 5-year EFS for chemotherapy, radiotherapy, and other modality treatment was 89.2%, 89.7%, and 83.1%, respectively, whereas the 5-year OS for chemotherapy, radiotherapy, and other modality treatment was 100%, 90.4%, and 87.5%, respectively. Of 14 patients with stage IIE2 to IV disease, the 5-year EFS for chemotherapy alone (n=9) and combined radiotherapy and chemotherapy (n=5) were 68.6%, 80%, respectively, whereas the 5-year OS for both group was 80.0%. Among 42 patients receiving radiotherapy, 7 (16.7%) patients developed cataract, 4 (9.5%) patients with keratitis, and 2 (4.8%) patients with maculopathy. In multivariate analysis, high-grade transformation (P=0.049) was the significant factor for shorter EFS of 1st -line treatment. Old age (> 60 years) (p = 0.014), advanced stage (stages III and IV) (p = 0.03), and high-grade transformation (p = 0.018) were the prognostic factors of poor OS.Conclusions:In addition to radiotherapy, our data indicate that chemotherapy as first-line therapy provides good disease control and less radiotherapy-associated side effects for patients with stage I to IIE1 OALs. Old age, advanced stage, and high-grade histology subtype remain poor prognostic factors for OALs, and new treatment strategies for these patients are warranted. DisclosuresNo relevant conflicts of interest to declare.

Partial orbit irradiation achieves excellent outcomes for primary orbital lymphoma

PurposePrimary radiation therapy (RT) achieves excellent local control and overall survival when treating localized orbital lymphoma. However, evidence supporting irradiation of partial orbit volumes to spare nearby critical structures is lacking. We sought to investigate outcomes for patients with localized orbital lymphoma treated with partial orbit irradiation. Methods and materialsWe retrospectively reviewed patients with orbital lymphoma treated with RT at our institution who met our inclusion criteria: biopsy-confirmed, low-grade lymphoma, localized disease, partial orbit treatment volumes, and follow-up >3months. The Kaplan-Meier method was used to measure overall survival (OS), and the cumulative incidence function adjusted for the competing risk of death was used to measure local failure (LF), contralateral orbit recurrence (COR), and progression. Patient characteristics were compared with outcomes using Fisher exact test for dichotomous variables and Wilcoxon rank-sum test for continuous variables. ResultsThirty-two patients meeting inclusion criteria were identified with median follow-up of 45.8months (range, 3.6-171.9). The majority had stage IEA disease; their sites included conjunctiva (n=20) and retrobulbar or lacrimal gland (n=12). Median partial orbit RT dose was 30.6Gy (range, 22.5-36). Five-year OS was 100%. Five-year cumulative incidence of LF, COR, and overall disease progression was 5.3%, 5.9%, and 21.4%, respectively. Five-year cumulative incidence of LF was 8.3% for conjunctival disease versus 0.0% for retrobulbar or lacrimal gland involvement (P=.15). No significant association was observed between the outcomes of LF, COR, or progression and pretreatment characteristics. Acute and late toxicity included grade 2 periorbital edema (n=3, 9.4%), dry eye (n=3, 9.4%), retinal vascular disorder (n=1, 3.1%), conjunctivitis (n=2, 6.3%), and grade 3 cataract (n=1, 3.1%). ConclusionsUse of partial orbit irradiation in treating low-grade, localized orbital lymphoma achieves excellent survival with low rates of LF, COR, or progression.

Établissement d’un arbre décisionnel diagnostique dans les orbitopathies inflammatoires non basedowiennes chroniques – à propos de 61 patients

Orbital inflammatory syndromes include a wide variety of inflammatory intraorbital processes which are very different in terms of clinical presentation and prognosis. We currently prefer to differentiate so-called "specific" inflammations, for which an etiology is able to be identified, from idiopathic orbital inflammatory syndromes (IOIS), for which the etiology remains unknown and the histology is nonspecific. To propose an efficient diagnostic approach for clinicians managing patients with non-Graves' orbital inflammations. This is a retrospective and prospective study concerning 61 patients managed by the medical team for non-Graves' orbital inflammations between May, 1999 and May, 2013 in the ophthalmology departments of Nice and Limoges university hospitals in France. Seventeen specific inflammations, 19 orbital lymphomas and 25 idiopathic orbital inflammatory syndromes were included. Patients were divided into two groups. Thirty-six patients (group 1) underwent primary biopsy, while for the other 25 (group 2), therapy was begun empirically without biopsy. We could therefore compare both approaches in terms of diagnostic efficiency and time until identification of a specific etiology. Our statistical results show that an approach without primary biopsy leads to a number of specific diagnoses statistically much lower than that obtained by the approach with primary biopsy. Also, the risk of missing a specific inflammation (with as a consequence an inappropriate treatment and a risk of functional sequelae as well as a fatal risk of missing a lymphoproliferative pathology) is very clearly higher in the case of not performing primary biopsy. Finally, the average time elapsed between the initial consultation with the ophthalmologist and a specific diagnosis was one month in the case of the first approach, while this delay was almost three times higher with the second approach, with a mean of 2.91 months (P<0.01). Our study shows that biopsy should be the mainstay of diagnostic management. A trial of empiric treatment is only performed first in myositis or in locations where biopsy could jeopardize functional prognosis. It should only be done after biopsy in all other cases. Of course, in all cases of relapse or recurrence after treatment, biopsy should be performed or repeated. The diagnostic work-up of a patient with an orbital inflammatory process must of course include blood testing and orbital imaging, but also a systematic primary biopsy for histological examination in the vast majority of cases. It must be repeated at least in the case of any doubt about the diagnosis or in the case of any recurrence or resistance to treatment.

Establishment of mouse model of orbital diffuse large B cell lymphoma

Background Recently, the morbidity of orbital lymphoma increased gradually, and we have made deeper research in pathology, therapy and pathogenesis of the disease.There were few reports of mice model of orbital lymphoma up to now for its lower morbidity and culture difficulty. Objective This study was to establish a mouse model of orbital diffuse large B cell lymphoma (DLBCL) by injection of systemic DLBCL cell line pfeiffer. Methods Ten SPF BALB/c mice and 5 nod-SCID mice were radiated firstly using 137Cs, with the absorption dose 3.5 Gy in the BALB/c mice and 2.6 Gy in the nod-SCID mice, and then pfeiffer cells were intraobitally injected in 4 eyes of BALB/c mice (orbital injection group) and suncutaneously injectied in 4 eyes of BALB/c mice and 4 eyes of nod-SCID mice (subcutaneous injection group) at the concentration of 1.5×108/ml.The developing status of tumors were examined once per day and the growth curve was drawn.The tumors and nearby lymph nodes were obtained 54 days after injection for the preparation of 4 μm thickness of serial sections.Hemotoxylin-eosin staining was used to examine the histopathology of the tumors, and immunochemistry was employed to detect the expressions of CD20, CD79α and CD45RO proteins.The tumors were typed based on the expressions of CD10, BCL-6 and mum-1 in the specimens, and the expressions of Ki-67 and survivin were assyed to assess the prognosis of the tumor.All the results were compared with 3 diagnosed human orbital DLBCL sections.The use and care of the mice complied with Chinese Administration Rule of Laboratory Animal. Results The tumor formation rates were 100% in both the orbital injection group and subcutaneous injection group, and the tumors grew much faster in nod-SCID mice than BALB/c mice.Infiltration of tumor cells in lymph nodes were found in the subcutaneous injection group rather than the orbital injection group.The pathological features were accordant among the orbital injection group, subcutaneous injection group and human orbital DLBCL sections.The number of <50% and ≥50% CD20+ specimens was 3 and 5, CD79α was 2 and 6, CD45RO was 8 and 0 in the BALB/c mice; while that in the nod-SCID mice was 1 and 3 in CD20, 0 and 4 in CD79α, 4 and 0 in CD45RO; the number of human orbital DLBCL specimens was 1 and 2 in CD20, 1 and 2 in CD79α, 2 and 1 in CD45RO, without significant differences among them (all at P=1.00). No significant differences were seen in Ki-67+ number and survivin+ number among the BALB/c mice, nod-SCID mice and human orbital DLBCL specimens (all at P=1.00). The detection of CD10, BCL-6 and mum-1 expressions indicated that the tumors of BALB/c mice, nod-SCID mice and human orbital DLBCL specimens all were the non-germinal center B cell-like types. Conclusions The orbital and subcutaneous DLBCL mouse models are successfully established by injection of pfeiffer cell line.There are the same findings and features in biological behavior, pathology and immunohistochemistry in orbital, subcutaneous models with human orbitl DLBCL.Nod-SCID mice appear to be more suitable for the growth of lymphoma cells. Key words: Orbital neoplasms/pathology; Lymphoma, non-Hodgkin/pathology; Cell line; Lymphoma, B-cell/immunology; Mice, inbred BABL/c; Mice, inbred NOD; Mice, SCID; Diseasse model, animal

Radiation Therapy in the Management of Orbital Lymphoma

To report our institution’s treatment techniques, disease outcomes, and complication rates after radiation therapy for the management of lymphoma involving the orbits. We retrospectively reviewed the medical records of 44 patients diagnosed with stage IAE (77%) or stage IIAE (23%) orbital lymphoma and curatively treated with radiation therapy between May 1969 and April 2013. The median follow-up was 4.9 years. Thirty-eight patients (86%) had low-grade lymphoma and 6 patients (14%) had high-grade lymphoma. Patients received treatment with one or more of the following beam energies: 250 kVP (34%), 60Co (36%), 2 MV (5%), 4 MV (16%), 6 MV (20%), or 8 MV (5%). Radiation was delivered with either wedge pair (61%), single anterior (34%), or anterior with bilateral wedges (5%) techniques. The median radiation dose was 25.5 Gy (range, 15 Gy – 47.5 Gy). Lens shielding was performed when possible. Cause-specific survival and freedom from distant relapse were calculated using the Kaplan-Meier method. The 5-year local control rate was 98%. Control of disease in the orbit was achieved in all but one patient who developed an out-of-field recurrence after irradiation of a lacrimal tumor and was salvaged successfully with further radiation therapy. The 5-year regional control rate was 91% (3 patients failed in the contralateral orbit and 1 patient failed in the ipsilateral parotid). Freedom from metastasis, cause-specific survival, and overall survival rates at 5 years were 76%, 89%, and 76%, respectively. Acute toxicity was minimal. Eight patients (18%) reported worsened vision following radiation therapy, and cataracts developed in eight patients. Cataracts developed in 6 of 28 patients treated without lens shielding (21%) and 2 of 16 patients (13%) treated with lens shielding. No patient developed significant late lacrimal toxicity. Radiation therapy is a safe and effective local treatment in the management of orbital lymphoma.

Shifts in Stereotactic Radiosurgery Platform Utilization for Brain Metastases in the United States

survival and freedom from distant relapse were calculated using the Kaplan-Meier method. Results: The 5-year local control rate was 98%. Control of disease in the orbit was achieved in all but one patient who developed an outof-field recurrence after irradiation of a lacrimal tumor and was salvaged successfully with further radiation therapy. The 5-year regional control rate was 91% (3 patients failed in the contralateral orbit and 1 patient failed in the ipsilateral parotid). Freedom from metastasis, cause-specific survival, and overall survival rates at 5 years were 76%, 89%, and 76%, respectively. Acute toxicity was minimal. Eight patients (18%) reported worsened vision following radiation therapy, and cataracts developed in eight patients. Cataracts developed in 6 of 28 patients treated without lens shielding (21%) and 2 of 16 patients (13%) treated with lens shielding. No patient developed significant late lacrimal toxicity. Conclusion: Radiation therapy is a safe and effective local treatment in the management of orbital lymphoma. Author Disclosure: S. Kharod: None.M.P. Herman: None. C.G. Morris: None. J. Lightsey: None. W.M. Mendenhall: None. N.P. Mendenhall: None.

Orbital and palpebral lymphomas

SummaryExtra nodal lymphomas may involve the ocular adnexae in 8% of cases. The mean age of onset is basically in the sixth decade. Lymphomas may present as a palpebral mass (39%), conjonctival infiltrate (39%) or proptosis (31%). It may involve orbital fat (47%), orbital muscles (26%), lacrymal gland (19%), preseptal tissues of eyelids (10%), or conjonctiva (60%). However, no specific location has been statistically associated to a better prognosis.Although MALT lymphoma (low grade with good prognosis) is the most common type (46%), other types such as follicular (20%), DLCB (11%), Mantel (5%), or other (17%) may occur. Lymphoma of ocular adnexae represent around 12% of all MALT lymphomas.The incidence of Chlamydia Psittaci infection in lymphoma patients may vary according to the geographic area, for instance 0% in Florida and 80% in Italy.The incidence of amyloïdosis in lymphoma patients arises from 0.85% to 7.5%. However amyloïd deposits are often not systematically looked for, and therefore may remain misdiagnosed.The handling of orbito‐palpebral lymphomas requires the collaboration of a multidisciplinary team involving pathologists, biological and clinical haematologists, microbiologists and orbital surgeons.