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Background The purpose of this study was to report the response to and toxicity of ultra-low-dose radiotherapy (RT) for B-cell ocular adnexal lymphoma (OAL). Methods We conducted a retrospective review of patients with indolent B-cell and mantle cell OAL treated with 4 Gy to the orbit(s) in two 2-Gy fractions. Disease response was assessed clinically and/or radiographically at 2 to 4-month intervals after RT. Data collected included rates of overall response, complete response (CR), partial response (PR), and treatment-related toxic effects. Results Twenty-two patients (median age, 65 years) had the following histologic subtypes: mucosa-associated lymphoid tissue (MALT; 14 patients; 64%); follicular lymphoma (5 patients; 23%); mantle cell lymphoma (MCL; 2 patients; 9%); and unclassifiable (1 patient, 4%). The overall response rate was 100%; 19 patients (86%) had a CR and 3 patients (14%) had a PR. The only acute toxic effect was grade 1 dry eye syndrome in 1 patient. Conclusion Ultra-low-dose RT in patients with OAL is associated with high response rates and minimal toxic effects, and is much shorter in duration and cost. © 2017 Wiley Periodicals, Inc. Head Neck 39: 1095-1100, 2017.

Conjunctival lymphoma is well documented in the medical literature, but veterinary reports are few. We report five cases of canine lymphoma, and three of feline in which the presenting sign was conjunctival involvement. All animals were in apparently good health at the time of presentation, and attended the referring clinic because of conjunctival disease. One dog showed generalized lymphadenopathy at presentation, although the ocular lesion was the reason for consultation, but all other patients were well with no detectable disease beyond the eye. All cats were presented for their ocular disease. All dogs were confirmed to have T-cell tumors, although the histological appearance of these was variable. In contrast, all cats had B-cell tumors. Referring clinicians and owners were contacted for follow-up information. Three dogs had been euthanased within 6 months of diagnosis for deterioration of general health. The remaining two were alive and showed no signs of systemic disease. Two cats had good survival following diagnosis, the other died of lesions that may not be related.

The American Joint Committee on Cancer has proposed the tumor, node, metastasis (TNM) staging system to overcome the limitations of the Ann Arbor staging system for ocular adnexal lymphoma. We performed this study to evaluate the feasibility of the TNM staging system for ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (OAML). The data form 66 total eyes from 54 patients with biopsy-confirmed OAML according to World Health Organization classification were retrospectively analyzed. Using the TNM staging system, we reclassified all patients into two categories: (1) T1N0M0 stage group (n = 26), for patients with lymphoma involving only the conjunctiva; and (2) above T1N0M0 or bT1N0M0 stage group (n = 28), for patients with lymphoma extending to the orbit, eyelid, or adjacent structures, and/or bilateral OAML. After a 24-month median follow-up period for all patients, the T1N0M0 group revealed higher progression-free survival (PFS) than the above T1N0M0 or the bT1N0M0 group (P = 0.041). In a separate analysis of only 50 patients categorized as Ann Arbor stage IE, the T1N0M0 group demonstrated higher PFS (100%) than the above T1N0M0 or the bT1N0M0 group (84.7%; P = 0.067). Our data show that the poor prognostic group classified as Ann Arbor stage IE can be further distinguished by using the TNM staging system. Thus, further studies to develop treatment strategies for reducing relapse after treatment for OAML should use the TNM staging system.

There is a causal association between Helicobacter pylori (Hp) gastric infection and the development of gastric MALT lymphoma. In contrast, the link between Hp gastric infection and the development of extragastric lymphoma has not been thoroughly investigated. We, therefore, studied the prevalence of gastric Hp infection at initial diagnosis of ophthalmologic and nonophthalmologic extragastric lymphoma patients. Three cohorts of patients were studied: a first one of 83 patients with OAL, a second one of 101 patients with extraophthalmologic extragastric lymphoma, and a third one of 156 control individuals (control) without malignant lymphoma. Gastric Hp infection was investigated by histopathological analysis and Hp-specific PCR assay on gastric biopsy tissue samples. We found gastric Hp infection in 37 OAL patients (45%), in 25 extraophthalmologic extragastric lymphoma cases (25%), and in 18 controls individuals (12%) (P < 0.0001 OAL/C and P < 0.01 OAL/extra-OAL cases). Gastritis was found in 51% and 9% of Hp-positive and Hp-negative lymphoma patients, respectively (P < 10−4). Gastric Hp infection only correlated with MALT/LPL lymphoma (P = 0.03). There is a significant association between gastric Hp infection and MALT/LPL OAL. This suggests a novel mechanism of indirect infection-associated lymphomagenesis whereby chronic local antigen stimulation would lead to the emergence of ectopic B-cell lymphoma. © 2010 Wiley-Liss, Inc. Am. J. Hematol.

Ocular adnexal MALT lymphoma (OAML) is linked to Chlamydophila psittaci (Cp) infection. Viability and infectivity of Cp, demonstrated by growth in culture, has not been yet investigated in these patients. We conducted a single-center prospective case-control study to assess the prevalence, viability and infectivity of Cp in 20 OAML patients and 42 blood donors registered in a 6-month period. The presence of Cp in conjunctival swabs and peripheral blood mononuclear cells (PBMC) of patients and donors was assessed by TETR-PCR and in vitro cultures. From an epidemiological point of view, OAML patients often resided in rural areas, and reported a history of chronic conjunctivitis and prolonged contact with household animals (85% vs. 38% of donors; p = 0.00001). Cp was detected in lymphoma tissue in 15 (75%) patients. Cp DNA was detected in conjunctival swabs and/or PBMC from 10 (50%) patients and in PBMC from 1 (2%) donor (p = 0.01). Viability and infectivity of Cp, demonstrated by growth in culture, were confirmed in conjunctival swabs and/or PBMC from 5 (25%) patients, but not in donors (p = 0.002). This prospective study demonstrates, for the first time, that Cp present in the conjunctiva and PBMC of OAML patients is capable to grow and be isolated in cell cultures. Cp infection is common in OAML patients and exceptional in blood donors. Epidemiological data of OAML patients (prolonged contact with household animals and chronic conjunctivitis) are consistent with Cp exposure risk. (C) 2008 Wiley-Liss, Inc. Ocular adnexal MALT lymphoma (OAML) is linked to Chlamydophila psittaci (Cp) infection. Viability and infectivity of Cp, demonstrated by growth in culture, has not been yet investigated in these patients. We conducted a single-center prospective case-control study to assess the prevalence, viability and infectivity of Cp in 20 OAML patients and 42 blood donors registered in a 6-month period. The presence of Cp in conjunctival swabs and peripheral blood mononuclear cells (PBMC) of patients and donors was assessed by TETR-PCR and in vitro cultures. From an epidemiological point of view, OAML patients often resided in rural areas, and reported a history of chronic conjunctivitis and prolonged contact with household animals (85% vs. 38% of donors; p = 0.00001). Cp was detected in lymphoma tissue in 15 (75%) patients. Cp DNA was detected in conjunctival swabs and/or PBMC from 10 (50%) patients and in PBMC from 1 (2%) donor (p = 0.01). Viability and infectivity of Cp, demonstrated by growth in culture, were confirmed in conjunctival swabs and/or PBMC from 5 (25%) patients, but not in donors (p = 0.002). This prospective study demonstrates, for the first time, that Cp present in the conjunctiva and PBMC of OAML patients is capable to grow and be isolated in cell cultures. Cp infection is common in OAML patients and exceptional in blood donors. Epidemiological data of OAML patients (prolonged contact with household animals and chronic conjunctivitis) are consistent with Cp exposure risk. (C) 2008 Wiley-Liss, Inc.

Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma) usually lacks CD5 expression. Herein is described two cases of CD5-positive MALT lymphoma of ocular adnexal origin. The differential diagnosis between CD5-positive MALT lymphoma and mantle cell lymphoma (MCL), notably cyclin D1-negative MCL, was difficult because both cases consisted histologically of small to medium-sized cells with diffuse or vaguely nodular growth pattern, and the neoplastic cells were positive for CD5 and negative for cyclin D1. Somatic mutation analysis of the immunoglobulin heavy chain variable region (VH) gene in case 1 found a relatively higher mutation frequency (5.0%), which was not definitive to rule out MCL. Interphase fluorescence in situ hybridization (FISH) on paraffin-embedded section using IgH/cyclin D1 (CCND1) probe showed that in both cases there was no molecular evidence of t(11;14), finally leading to the diagnosis of CD5-positive MALT lymphoma. Although the present two patients had no recurrence over 34 months after initial diagnosis, careful observation is needed because the clinicopathological significance of MALT lymphoma with this rare phenotype remains obscure.

BACKGROUND. There are conflicting reports regarding the association between Chlamydia psittaci (Cps) and ocular adnexal lymphoma (OAL) and the efficacy of antibiotics for OAL. In the current study, the authors attempted to clarify the association between Cps and OAL and the efficacy of antibiotics for OAL. METHODS. Two meta‒analyses were conducted. One focused on the association between Cps and OAL across geographic regions and among different studies. The other was a meta‒analysis of the response of OAL to antibiotic treatment. RESULTS. The authors identified 11 studies of Cps prevalence that included 458 cases of OAL from 10 different countries. Four studies regarding the efficacy of oral antibiotics to treat OAL were found. One hundred four of the 458 OAL specimens (23%) and 87 of the 346 mucosa‒associated lymphoid tissue (MALT) lymphoma specimens (25%) were found to be positive for Cps. Ninety-four of the 104 Cps‒positive OAL specimens (90%) came from 3 of the 11 studies. There was wide variation noted between geographic regions and even between studies from the same geographic region with regard to the rate of Cps positivity. The 4 studies concerning the efficacy of antibiotics for OAL were from Italy, Austria, Taiwan, and the U.S. and included 42 patients. Twenty patients (48%) achieved some response (complete response in 8 patients, partial response in 8 patients, and minimal response in 4 patients). Twenty patients also had stable disease, and 2 patients progressed during antibiotic therapy. Objective documentation of response (radiographs or clinical slit-lamp photographs) was available for only 3 of the 42 patients. Seven additional patients developed disease recurrence after their initial response or stable disease after antibiotic therapy; 6 of these cases of disease recurrence occurred during the first 12 months of follow‒up. CONCLUSIONS. The findings of the current study suggest a striking variability in the association between Cps and OAL across geographic regions and even between studies from the same geographic regions. The overall rate of Cps positivity in our meta‒analysis (23%) was much lower than that reported in the original report. The current study findings also suggest that antibiotics have variable efficacy against OAL. Future prospective trials with standard objective response criteria and a longer follow‒up period would be necessary to evaluate the role of antibiotics in the treatment of OAL further. Cancer 2007; 110:809–15. © 2007 American Cancer Society.

The majority of ocular adnexal lymphomas (OAL) are primary marginal zone B-cell lymphomas (MALT lymphomas). The present study correlated the clinicopathological variables with the histologic subtypes by World Health Organization (WHO) classification with emphasis on MALT lymphomas in OALs of Koreans. There were 68 cases (31 males and 37 females), with a mean age of 45.9 years (range 7-89 years). Histologically, 61 MALT-type, 2 diffuse large B-cell (DLBCL), 2 mantle cell type (MCL), 1 anaplastic large-cell (ALCL), and 2 NK/T-cell lymphomas (NK/T-L) were counted among them. Fifty-seven were primary cases (P-OAL), and 11 were secondary cases (S-OAL). Nearly all P-OALs were MALT lymphomas (n = 56, 98%), with an exception of 1 MCL. Eleven S-OALs included 5 MALT type, 2 DLBCL, 1 ALCL, 1 MCL, and 2 NK/T-L. All MALT lymphoma patients were alive (n = 59) except for 2 after a mean duration of follow-up of 27.6 months (range: 0-108 months): one died of an unrelated cause and one died of recurrence. One non-MALT type P-OAL was alive with no evidence of disease (42 months). Of the 11 S-OAL, 4 had marrow involvement and 5 had progression or relapse outside the orbit. Compared with the other subtypes, MALT lymphoma was more likely to present with local disease (P = 0.001), achieve complete remission (CR) (0.022), and be alive at last follow-up (0.197), and less likely to experience recurrence (P = 0.06). In conclusion, OALs in Koreans are characterized by a preponderance of primary lymphomas over systemic lymphomas, striking predominance of MALT type lymphomas, and young age of occurrence. Histologic subtype by WHO classification has a significant correlation with the final outcome, with the most favorable outcome associated with OALs of the MALT type.

Background: This study involved 73 patients with lymphoproliferative lesions of the ocular adnexa. The lesions were categorized using the Revised European American Lymphoma classification of lymphoid tissues and analysed to determine the frequency and prognostic impact of tumour type, location, stage and patient's age and sex. Methods: The clinical, histopathological, immunohistochemical and phenotypic analysis by flow cytometry and follow-up data were studied. Results: The ocular adnexal lymphoproliferative lesions included 70 lymphomas and six reactive lymphoid hyperplasia. Seventy-nine per cent had stage IE disease, 4% stage II, 1.5% stage III and 15.5% stage IV. Five patients (7%) had a past history of systemic lymphoma. Major histological types were extranodal marginal zone lymphoma (MZL) in 44 (63%), follicular (FL) in 12 (17%), diffuse large B-cell (DLBCL) in eight (11%), mantle cell (MCL) in two (3%), B-cell chronic lymphocytic leukaemia (CLL)/small lymphocytic lymphoma in two (3%), peripheral T-cell lymphoma (PTCL) one (1.5%) and natural killer cell lymphoma (NKCL) in one (1.5%). Longest survival was seen in those with low-grade lymphomas (MZL and FL) and worst in PTCL and NKCL. Lymphoma-related mortality was 2% for MZL, 33% for FL, 38% for DLBCL, and 100% for MCL, PTCL and NKCL. Systemic lymphoma was present prior to, at presentation or at subsequent follow up in 26/68 (39%) of all lymphoma patients, 17% for MZL, 38% for DLBCL, 83% for FL, and 100% for MCL, CLL, PTCL and NKCL. Conclusion: The majority of ocular adnexal lymphomas were low-grade B-cell lymphomas (MZL). Multivariate analysis showed that the only significant independent predictors of all causes of mortality were the histological type of lymphoma and the stage of disease at presentation.

Purpose There is a causal association between Helicobacter pylori (Hp) gastric infection and the development of gastric MALT lymphoma. In contrast, the link between Hp gastric infection and the development of extra-gastric lymphoma has not been thoroughly investigated. We therefore studied the prevalence of gastric Hp infection at initial diagnosis of ophthalmologic and non-ophthalmologic extra-gastric lymphoma patients. Methods Three cohorts of patients were studied: a first one of 83 patients with OAL, a second one of 101 patients with extra-ophthalmologic extra-gastric lymphoma, and a third one of 156 control individuals (control) without malignant lymphoma. Gastric Hp infection was investigated by histopathological analysis and Hp-specific PCR assay on gastric biopsy tissue samples. Results We found gastric Hp infection in 37 OAL patients (45%), in 25 extra-ophthalmologic extra-gastric lymphoma cases (25%), and in 18 controls individuals (12%) (p

The majority of ocular adnexal lymphomas (OAL) occurring in ophthalmologic sites are low-grade B-cell non-Hodgkin lymphomas, and about 15% of all cases are high-grade. Although numerous reports have already studied the features of low-grade OAL, high-grade OAL have been usually described as part of various published series of OAL without specifically focusing on these histopathologic subtypes of lymphoma. The aim of this study was therefore to retrospectively review a single-centre series of 15 patients with high-grade B-cell OAL treated at the Institut Curie. The main clinical and laboratory features of our selected patients, i.e., median age (66 years), PS greater than 1 (20%), stage IV (53%), bone marrow involvement (17%), more than one extranodal site (27%), and elevated serum LDH (14%), were not significantly different from those of nodal non-Hodgkin's lymphomas (NHL), except for a lower proportion of OAL patients with elevated LDH level. Similarly, the prognosis of these patients was not different, with a 5-year overall survival of 54%. When high-grade OAL were compared to a historical series of low-grade OAL, a lower rate of conjunctival involvement was observed, but both types presented a majority of orbital and/or lacrimal gland involvement as well as bilateral ophthalmological tumors. The recommended treatment for high-grade B-cell OAL can be the same as that for nodal CD20-positive NHL, namely a combination of rituximab and CHOP-like regimen.

As illustrated in the two cases described in this paper close morphologic and immunohistochemical similarities exist between Mediterranean lymphoma (MTL) and primary gastrointestinal lymphoma of follicle center cell (FCC) origin as it occurs in Western countries. Similarities between the two conditions include a dense noninvasive monotypic lamina propria plasma cell infiltrate, present in all cases of MTL and in some cases of Western gastrointestinal FCC lymphoma, and an invasive infiltrate of FCCs morphologically distinct from the plasma cells. A distinctive lesion produced by individual gland invasion characterizes both types of lymphoma. A clonal relationship between the lamina propria plasma cells and the invasive FCCs, long suspected but never proved in MTL, can be demonstrated in Western cases. Many of the histologic and clinical features common to these lymphomas can be explained in the context of the normal maturation sequences of gut associated lymphoid tissue. It is suggested that MTL and Western cases of primary FCC gastrointestinal lymphoma share a common histogenesis from mucosa associated lymphoid tissue.

A series of 1,467 Caucasian patients with non-disseminated lymphomas of extranodal origin was taken from data collected by the End Results Group of cancer registries in the years 1950-1964. Excluding Hodgkin's disease, about one fourth of the lymphomas reported arose in sites other than lymph nodes. Survival rates and distributions are listed for site of origin, major histologic types, sex, age, and extent of disease. For the more frequently reported sites, survival rates are given according to the type of initial treatment used. The prognosis of patients with extranodal lymphomas is compared with that for “all cancers” of the same site, and the lymphoma patients appear to fare appreciably better when the site of origin is stomach, lung, or tonsil.