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1. Kim TM, Kim KH, Lee MJ, Jeon YK, Lee SH, Kim DW, Kim CW, Kim IH, Khwarg SI, Heo DS: First-line therapy with doxycycline in ocular adnexal mucosa-associated lymphoid tissue lymphoma: a retrospective analysis of clinical predictors. Cancer Sci; 2010 May;101(5):1199-203
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  • [Title] First-line therapy with doxycycline in ocular adnexal mucosa-associated lymphoid tissue lymphoma: a retrospective analysis of clinical predictors.
  • This retrospective study was launched to evaluate the efficacy of doxycycline and to find independent predictors of a clinical response in patients with ocular adnexal lymphoma of mucosa-associated lymphoid tissue (OAML).
  • After a median follow-up of 26.4 months, doxycycline resulted in an overall response rate of 47% and a 3-year time-to-treatment failure (TTF) rate of 84%.
  • [MeSH-major] Anti-Bacterial Agents / therapeutic use. Doxycycline / therapeutic use. Eye Neoplasms / drug therapy. Lymphoma, B-Cell, Marginal Zone / drug therapy
  • [MeSH-minor] Adult. Aged. Female. Humans. Male. Middle Aged. Retrospective Studies. Treatment Failure

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  • (PMID = 20345477.001).
  • [ISSN] 1349-7006
  • [Journal-full-title] Cancer science
  • [ISO-abbreviation] Cancer Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; N12000U13O / Doxycycline
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2. Stefanovic A, Lossos IS: Extranodal marginal zone lymphoma of the ocular adnexa. Blood; 2009 Jul 16;114(3):501-10
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  • [Title] Extranodal marginal zone lymphoma of the ocular adnexa.
  • Lymphomas of the ocular adnexa are a heterogeneous group of malignancies, composing approximately 1% to 2% of non-Hodgkin lymphomas (NHLs) and 8% of extranodal lymphomas.
  • The most common subtype, accounting for up to 80% of cases of primary ocular adnexal lymphoma, is marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type.
  • In the recent past, there have been significant advances in our understanding of the clinical characteristics, morphology and phenotype, etiology, pathogenesis, diagnosis, natural history, treatment approaches, outcome, and prognostic factors of this disease entity.
  • Novel immunologic and molecular techniques have aided in the distinction between MALT lymphoma and other lymphoproliferative disorders and led to the identification of tissue markers of prognostic significance.
  • Modern imaging modalities provide invaluable tools for accurate staging and treatment planning.
  • Besides radiotherapy and chemotherapy, a variety of new treatment options have emerged in the management of patients with ocular adnexal MALT lymphoma, especially monoclonal antibody therapy and antibiotic therapy against Chlamydia psittaci, which has been associated with the pathogenesis of ocular adnexal lymphomas in some parts of the world.
  • In this review, we present a state-of-the-art summary of ocular adnexal MALT lymphomas.

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  • (PMID = 19372259.001).
  • [ISSN] 1528-0020
  • [Journal-full-title] Blood
  • [ISO-abbreviation] Blood
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA109335; United States / NCI NIH HHS / CA / R01 CA122105; United States / NCI NIH HHS / CA / R01-CA109335; United States / NCI NIH HHS / CA / R01-CA122105
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 99
  • [Other-IDs] NLM/ PMC2713468
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3. Nola M, Lukenda A, Bollmann M, Kalauz M, Petrovecki M, Bollmann R: Outcome and prognostic factors in ocular adnexal lymphoma. Croat Med J; 2004 Jun;45(3):328-32
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  • [Title] Outcome and prognostic factors in ocular adnexal lymphoma.
  • AIM: To classify ocular lymphomas in patients treated at the Zagreb University Hospital Center according to the new classification of the World Health Organization (WHO) and to determine factors with prognostic significance.
  • METHODS: From 1986 to 2003, histological diagnosis of ocular lymphoma was made in 24 patients.
  • The patients underwent staging procedures and clinical evaluations prior to the date of the initial therapy.
  • The main outcome measures were development of distant recurrence after new presentation with solely ocular adnexal disease, and death attributable to widespread lymphoma.
  • RESULTS: Ocular adnexal lymphomas were found in orbit in 20 patients, in eyelid in two, and conjunctiva in two patients.
  • Twenty patients had lymphoma stage IE, one had IIE, and three had stage IV.
  • Three patients had prior or concurrent systemic disease and 21 patients had primary lymphoma.
  • The main subtypes of non-Hodgkin lymphoma according to the WHO classification were extranodal marginal zone B-cell lymphoma (n=20), diffuse large cell B-cell lymphoma (n=2), mantle cell lymphoma (n=1), and plasmacytoma (n=1).
  • Radiotherapy was given to 11 patients, chemotherapy in 8 patients, whereas radiotherapy and chemotherapy were implemented in three patients.
  • In the group of patients with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (B-EMZL), the estimated 5-year overall survival was 92.9+/-6.6% (mean+/-standard deviation) and the 5-year failure-free survival was 80.1+/-10.3%.
  • Age, sex, side of involvement, anatomic localization of the lesion, clinical stage of disease, and mode of therapy did not have any prognostic significance during the follow-up period (median, 53; range, 9-131 months).
  • Patients with B-EMZL had almost 14 times higher chance for an unfavorable outcome if the tumor cells expressed CD43 on their surface, than the CD43-negative cases.
  • CONCLUSION: Most ocular adnexal lymphomas usually have a B-cell immunophenotype, the morphologic and immunohistochemical features of extranodal marginal zone B-cell lymphoma, and a favorable prognosis.
  • CD43 positive ocular lymphomas are associated with a higher rate of subsequent distant recurrence and the rate of lymphoma-related death.
  • [MeSH-major] Conjunctival Neoplasms / classification. Eyelid Neoplasms / classification. Lymphoma, B-Cell, Marginal Zone / classification. Orbital Neoplasms / classification
  • [MeSH-minor] Adult. Aged. Female. Humans. Male. Middle Aged. Prognosis. Recurrence. Risk Factors. Survival Analysis. Treatment Outcome. World Health Organization

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  • (PMID = 15185428.001).
  • [ISSN] 0353-9504
  • [Journal-full-title] Croatian medical journal
  • [ISO-abbreviation] Croat. Med. J.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Croatia
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4. Husain A, Roberts D, Pro B, McLaughlin P, Esmaeli B: Meta-analyses of the association between Chlamydia psittaci and ocular adnexal lymphoma and the response of ocular adnexal lymphoma to antibiotics. Cancer; 2007 Aug 15;110(4):809-15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Meta-analyses of the association between Chlamydia psittaci and ocular adnexal lymphoma and the response of ocular adnexal lymphoma to antibiotics.
  • BACKGROUND: There are conflicting reports regarding the association between Chlamydia psittaci (Cps) and ocular adnexal lymphoma (OAL) and the efficacy of antibiotics for OAL.
  • The other was a meta-analysis of the response of OAL to antibiotic treatment.
  • 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.
  • Twenty patients also had stable disease, and 2 patients progressed during antibiotic therapy.
  • 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.
  • 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.
  • [MeSH-major] Anti-Bacterial Agents / therapeutic use. Chlamydophila psittaci / isolation & purification. Eye Neoplasms / drug therapy. Lymphoma, B-Cell, Marginal Zone / drug therapy. Psittacosis / complications
  • [MeSH-minor] DNA, Bacterial / analysis. DNA, Bacterial / genetics. DNA, Ribosomal Spacer / genetics. Epidemiologic Studies. Humans. Lymphoma / drug therapy. Lymphoma / etiology. RNA, Ribosomal, 16S / genetics. RNA, Ribosomal, 23S / genetics. Treatment Outcome

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  • (PMID = 17594698.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Meta-Analysis
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / DNA, Bacterial; 0 / DNA, Ribosomal Spacer; 0 / RNA, Ribosomal, 16S; 0 / RNA, Ribosomal, 23S
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5. Sato T, Takimoto R, Iyama S, Ono K, Tanaka S, Araki N, Nishiie K, Murase K, Sato Y, Kobune M, Matsunaga T, Kato J, Niitsu Y: [Gastric relapse of stage I ocular adnexal mucosa-associated lymphoid tissue lymphoma]. Rinsho Ketsueki; 2007 Jan;48(1):56-60
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  • [Title] [Gastric relapse of stage I ocular adnexal mucosa-associated lymphoid tissue lymphoma].
  • A 57-year-old male with right ocular adnexal mucosa-associated lymphoid tissue lymphoma (MALT lymphoma) was successfully treated with local radiation therapy.
  • The gastroendoscopic examination revealed a slight inflammatory change of the gastric mucosa, and the urease test was positive.
  • Eradication therapy against Helicobacter pylori was successfully done, however, the patient developed gastric MALT lymphoma two years after the initial treatment.
  • Southern blot analysis of the immunoglobulin heavy chain gene rearrangement revealed that the lymphoma cells from the ocular adnexal and gastric MALT lymphomas were identical, indicating that the gastric MALT lymphoma was not the primary but the metastatic region from the ocular adnexal MALT lymphoma.
  • Further, immunohistochemical staining demonstrated the expression of integrin alpha4beta 7 on ocular adnexal MALT lymphoma cells, which is essential for the adhesion of lymphocytes to gastrointestinal mucosa.
  • These results indicate that ocular adnexal MALT lymphoma cells can metastasize to the stomach, depending on the adhesional function of integrin alpha4 beta7.
  • [MeSH-major] Eye Neoplasms / pathology. Lacrimal Apparatus. Lymphoma, B-Cell, Marginal Zone / pathology. Oncogene Proteins, Fusion / genetics. Stomach Neoplasms / secondary
  • [MeSH-minor] Gastroscopy. Helicobacter Infections / drug therapy. Helicobacter pylori. Humans. Integrins / biosynthesis. Male. Middle Aged

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  • (PMID = 17313077.001).
  • [ISSN] 0485-1439
  • [Journal-full-title] [Rinshō ketsueki] The Japanese journal of clinical hematology
  • [ISO-abbreviation] Rinsho Ketsueki
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / IgH-MMSET fusion protein, human; 0 / Integrins; 0 / Oncogene Proteins, Fusion; 0 / integrin alpha4beta7
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6. Woo JM, Tang CK, Rho MS, Lee JH, Kwon HC, Ahn HB: The clinical characteristics and treatment results of ocular adnexal lymphoma. Korean J Ophthalmol; 2006 Mar;20(1):7-12
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  • [Title] The clinical characteristics and treatment results of ocular adnexal lymphoma.
  • PURPOSE: To assess the clinical pattern, the histopathological findings, the response to treatments, the recurrence pattern and the prognosis of malignant lymphoma in the ocular adnexa.
  • METHODS: This study was performed on 22 total eyes from 17 patients who were diagnosed with ocular adnexal malignant lymphoma.
  • We retrospectively analyzed the medical records for patient information including the histological classification based on age, the gender of each patient, the symptoms and signs at the initial diagnosis, the presence of binocular invasion, the findings of the surgical biopsy, the clinical stage of each patient's tumor, and the treatment methods used and their effectiveness.
  • Fifteen cases consisting of 20 total eyes represented extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT).
  • Five of seven patients (71.4%) whose lymphoma occurred within the conjunctiva relapsed after irradiation or chemotherapy, and four of the relapsed patients were salvaged with further therapy.
  • CONCLUSIONS: Extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) constituted 88.2% of all lymphomas involving the ocular adnexa.
  • Lymphoma in the ocular adnexa responded well to conventional treatment, but the recurrence rate of lymphoma in the conjunctiva was significantly high.
  • [MeSH-major] Eye Neoplasms / pathology. Eye Neoplasms / therapy. Lymphoma, B-Cell, Marginal Zone / pathology. Lymphoma, B-Cell, Marginal Zone / therapy
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Biopsy. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Retrospective Studies. Treatment Outcome

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  • (PMID = 16768184.001).
  • [ISSN] 1011-8942
  • [Journal-full-title] Korean journal of ophthalmology : KJO
  • [ISO-abbreviation] Korean J Ophthalmol
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2908820
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7. Verma V, Shen D, Sieving PC, Chan CC: The role of infectious agents in the etiology of ocular adnexal neoplasia. Surv Ophthalmol; 2008 Jul-Aug;53(4):312-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The role of infectious agents in the etiology of ocular adnexal neoplasia.
  • Given the fact that infectious agents contribute to around 18% of human cancers worldwide, it would seem prudent to explore their role in neoplasms of the ocular adnexa: primary malignancies of the conjunctiva, lacrimal glands, eyelids, and orbit.
  • By elucidating the mechanisms by which infectious agents contribute to oncogenesis, the management, treatment, and prevention of these neoplasms may one day parallel what is already in place for cancers such as cervical cancer, hepatocellular carcinoma, gastric mucosa-associated lymphoid tissue lymphoma and gastric adenocarcinoma.
  • Antibiotic treatment and vaccines against infectious agents may herald a future with a curtailed role for traditional therapies of surgery, radiation, and chemotherapy.
  • Unlike other malignancies for which large epidemiological studies are available, analyzing ocular adnexal neoplasms is challenging as they are relatively rare.
  • This review discusses the pathogenetic role of several microorganisms in different ocular adnexal malignancies, including human papilloma virus in conjunctival papilloma and squamous cell carcinoma, human immunodeficiency virus in conjunctival squamous carcinoma, Kaposi sarcoma-associated herpes virus or human herpes simplex virus-8 (KSHV/HHV-8) in conjunctival Kaposi sarcoma, Helicobacter pylori (H. pylori,), Chlamydia, and hepatitis C virus in ocular adnexal mucosa-associated lymphoid tissue lymphomas.
  • Unlike cervical cancer where a single infectious agent, human papilloma virus, is found in greater than 99% of lesions, multiple organisms may play a role in the etiology of certain ocular adnexal neoplasms by acting through similar mechanisms of oncogenesis, including chronic antigenic stimulation and the action of infectious oncogenes.
  • However, similar to other human malignancies, ultimately the role of infectious agents in ocular adnexal neoplasms is most likely as a cofactor to genetic and environmental risk factors.
  • [MeSH-minor] Alphapapillomavirus / isolation & purification. Alphapapillomavirus / physiology. Carcinoma, Squamous Cell / virology. Chlamydophila psittaci / isolation & purification. Chlamydophila psittaci / physiology. Conjunctival Neoplasms / microbiology. Conjunctival Neoplasms / virology. Eyelid Neoplasms / microbiology. Eyelid Neoplasms / virology. HIV-1 / isolation & purification. HIV-1 / physiology. Helicobacter pylori / isolation & purification. Helicobacter pylori / physiology. Hepacivirus / isolation & purification. Hepacivirus / physiology. Herpesvirus 8, Human / isolation & purification. Herpesvirus 8, Human / physiology. Humans. Lacrimal Apparatus Diseases / microbiology. Lacrimal Apparatus Diseases / virology. Lymphoma, B-Cell, Marginal Zone / virology. Orbital Neoplasms / microbiology. Orbital Neoplasms / virology. Sarcoma, Kaposi / virology

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  • (PMID = 18572051.001).
  • [ISSN] 0039-6257
  • [Journal-full-title] Survey of ophthalmology
  • [ISO-abbreviation] Surv Ophthalmol
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / / Z01 EY000222-22
  • [Publication-type] Journal Article; Research Support, N.I.H., Intramural; Review
  • [Publication-country] United States
  • [Number-of-references] 248
  • [Other-IDs] NLM/ NIHMS58178; NLM/ PMC2507724
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8. Cohen VM: Treatment options for ocular adnexal lymphoma (OAL). Clin Ophthalmol; 2009;3:689-92

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment options for ocular adnexal lymphoma (OAL).
  • Most lymphomas that involve the ocular adnexal structure are low grade, B cell, non-Hodgkin's lymphomas.
  • The treatment depends upon the grade and stage of the disease.
  • High grade lymhoma requires treatment with systemic chemotherapy whereas the localized low grade (extranodal marginal zone lymphoma) can be successfully managed with local radiotherapy.
  • Chlamydia psittaci infection is associated with low grade ocular lymphoma; however there is wide geographic variation in the strength of this association.
  • Blanket antibiotic therapy is not advised unless there is proof of an infective agent.
  • The monoclonal antibody, rituximab, may be successful for CD20 positive lymphoma, although it is likely that rituximab will have better long-term results when used in combination with systemic chemotherapy.

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  • (PMID = 20054418.001).
  • [ISSN] 1177-5483
  • [Journal-full-title] Clinical ophthalmology (Auckland, N.Z.)
  • [ISO-abbreviation] Clin Ophthalmol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] New Zealand
  • [Other-IDs] NLM/ PMC2801639
  • [Keywords] NOTNLM ; Chlamydia psittaci / chemotherapy / extranodal marginal zone lymphoma / mucosa associated lymphoid tissue / ocular adnexal lymphoma / radiotherapy / rituximab
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9. Ferreri AJ, Dolcetti R, Du MQ, Doglioni C, Resti AG, Politi LS, De Conciliis C, Radford J, Bertoni F, Zucca E, Cavalli F, Ponzoni M: Ocular adnexal MALT lymphoma: an intriguing model for antigen-driven lymphomagenesis and microbial-targeted therapy. Ann Oncol; 2008 May;19(5):835-46
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ocular adnexal MALT lymphoma: an intriguing model for antigen-driven lymphomagenesis and microbial-targeted therapy.
  • Non-Hodgkin's lymphomas constitute one half of malignancies arising in the orbit and the ocular adnexae.
  • Mucosa-associated lymphoid tissue (MALT)-type lymphoma is the most common histological category in this anatomic region.
  • The incidence of ocular adnexal lymphoma of mucosa-associated lymphoid tissue-type (OAML) is increasing and recent studies offered new relevant insights in molecular, pathogenetic and therapeutic issues on these neoplasms.
  • A pathogenetic model of antigen-driven lymphoproliferation similar to that reported for Helicobacter pylori-related gastric MALT lymphomas has been hypothesized for OAML.
  • This notion is supported by the association between OAML and Chlamydophila psittaci infection, an association that is of likely pathogenetic relevance and may influence both the biological behavior and the therapeutic management of these neoplasms.
  • Lymphomatous lesions are frequently responsible for symptoms affecting patient's quality of life, requiring, therefore, immediate treatment.
  • Several therapeutic strategies are available, often associated with relevant side-effects.
  • However, the therapeutic choice in OAML is not supported by consolidated evidence due to the lack of prospective trials.
  • In this review, we analyze the most relevant biological, molecular, pathological and clinical features of OAML and propose some therapeutic guidelines for patients affected by this malignancy.

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  • (PMID = 17986622.001).
  • [ISSN] 1569-8041
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; N12000U13O / Doxycycline
  • [Number-of-references] 86
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10. Decaudin D, Dendale R, Lumbroso-Le Rouic L: Treatment of mucosa-associated lymphoid tissue-type ocular adnexal lymphoma. Anticancer Drugs; 2008 Aug;19(7):673-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Treatment of mucosa-associated lymphoid tissue-type ocular adnexal lymphoma.
  • New data have recently been reported in ocular adnexal lymphomas (OALs), particularly of mucosa-associated lymphoid tissue (MALT)-type, regarding their biological characteristics and therapeutic management.
  • A possible association between OAL and Chlamydia psittaci infection has suggested new mechanisms of lymphomagenesis and opened the way for specific targeted treatment.
  • Similarly, the place of rituximab monoclonal antibody therapy and more conventional chemotherapy, as well as a 'wait-and-see' policy in few clearly defined situations, must be defined in relation to the standard treatment option consisting of radiotherapy for low-grade lymphomas.
  • The aims of this review are therefore to present the various treatment modalities and to discuss the place of each modality in the management of ophthalmologic lymphomas.
  • [MeSH-major] Eye Neoplasms / therapy. Lymphoma, B-Cell, Marginal Zone / therapy
  • [MeSH-minor] Anti-Bacterial Agents / therapeutic use. Humans. Immunotherapy. Psittacosis / drug therapy

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  • (PMID = 18594208.001).
  • [ISSN] 0959-4973
  • [Journal-full-title] Anti-cancer drugs
  • [ISO-abbreviation] Anticancer Drugs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents
  • [Number-of-references] 91
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11. Ferreri AJ, Ponzoni M, Guidoboni M, Resti AG, Politi LS, Cortelazzo S, Demeter J, Zallio F, Palmas A, Muti G, Dognini GP, Pasini E, Lettini AA, Sacchetti F, De Conciliis C, Doglioni C, Dolcetti R: Bacteria-eradicating therapy with doxycycline in ocular adnexal MALT lymphoma: a multicenter prospective trial. J Natl Cancer Inst; 2006 Oct 4;98(19):1375-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Bacteria-eradicating therapy with doxycycline in ocular adnexal MALT lymphoma: a multicenter prospective trial.
  • BACKGROUND: An association between ocular adnexal MALT lymphoma (OAL) and Chlamydia psittaci (Cp) infection has been proposed, and recent reports suggest that doxycycline treatment causes tumor regression in patients with Cp-related OAL.
  • The effectiveness of doxycycline treatment in Cp-negative OAL has not been tested.
  • METHODS: In a prospective trial, 27 OAL patients (15 newly diagnosed and 12 having experienced relapse) were given a 3-week course of doxycycline therapy.
  • Objective lymphoma response was assessed by computerized tomography scans or magnetic resonance imaging at 1, 3, and 6 months after the conclusion of therapy and every 6 months during follow-up.
  • Cp infection in patients was determined by touchdown enzyme time-release polymerase chain reaction (TETR-PCR).
  • At a median follow-up of 14 months, lymphoma regression was complete in six patients, and a partial response (> or = 50% reduction of all measurable lesions) was observed in seven patients (overall response rate [complete and partial responses] = 48%).
  • Lymphoma regression was observed in both Cp DNA-positive patients (seven of 11 experienced regression) and Cp DNA-negative patients (six of 16 experienced regression) (64% versus 38%; P = .25, Fisher's exact test).
  • CONCLUSIONS: Doxycycline is a fast, safe, and active therapy for Cp DNA-positive OAL that was effective even in patients with multiple failures involving previously irradiated areas or regional lymphadenopathies.
  • The responses observed in PCR-negative OAL may suggest a need for development of more sensitive methods for Cp detection and investigation of the potential role of other doxycycline-sensitive bacteria.
  • [MeSH-major] Anti-Bacterial Agents / therapeutic use. Antineoplastic Agents / therapeutic use. Chlamydophila psittaci / drug effects. Doxycycline / therapeutic use. Lymphoma, B-Cell, Marginal Zone / drug therapy. Orbital Neoplasms / drug therapy. Psittacosis / drug therapy
  • [MeSH-minor] Adult. Aged. DNA, Bacterial / isolation & purification. Disease-Free Survival. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / drug therapy. Polymerase Chain Reaction. Prospective Studies. Treatment Outcome

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  • [CommentIn] J Natl Cancer Inst. 2006 Oct 4;98(19):1348-9 [17018775.001]
  • (PMID = 17018784.001).
  • [ISSN] 1460-2105
  • [Journal-full-title] Journal of the National Cancer Institute
  • [ISO-abbreviation] J. Natl. Cancer Inst.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / Antineoplastic Agents; 0 / DNA, Bacterial; N12000U13O / Doxycycline
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12. McKelvie PA: Ocular adnexal lymphomas: a review. Adv Anat Pathol; 2010 Jul;17(4):251-61
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ocular adnexal lymphomas: a review.
  • Ocular adnexal lymphomas comprise 1% to 2% of all non-Hodgkin lymphomas and about 8% of extranodal lymphomas.
  • They are a heterogeneous group of malignancies, the majority of which are primary extranodal lymphoma with most (up to 80%) of the marginal zone of mucosa associated lymphoid tissue type (MALT lymphoma).
  • Finally, traditional (surgery, radiotherapy, chemotherapy) and newer forms of therapy (immunotherapy and radioimmunotherapy) will be reviewed.
  • [MeSH-major] Eye Neoplasms / diagnosis. Lymphoma / diagnosis
  • [MeSH-minor] Cytogenetic Analysis. Humans. Lymphoma, B-Cell, Marginal Zone / diagnosis. Lymphoma, B-Cell, Marginal Zone / etiology. Lymphoma, B-Cell, Marginal Zone / therapy. Psittacosis / complications. Treatment Outcome

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  • (PMID = 20574170.001).
  • [ISSN] 1533-4031
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 115
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13. Strianese D, Tranfa F, Finelli M, De Renzo A, Staibano S, Schiemer R, Cardone D, Pacelli R, Perna F, Mascolo M, De Rosa G, Bonavolontà G: Hepatitis C virus infection in ocular adnexal lymphomas. Arch Ophthalmol; 2010 Oct;128(10):1295-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Hepatitis C virus infection in ocular adnexal lymphomas.
  • OBJECTIVE: To assess the influence of hepatitis C virus (HCV) infection on disease appearance and outcome of ocular adnexal non-Hodgkin lymphoma (ONHL).
  • Seropositivity for HCV infection was significantly associated with extraorbital lymphoma at the onset (P = .006).
  • High prevalence of mucosa-associated lymphoid tissue disease (79.8%) was registered.
  • Protocol therapy included radiotherapy and chemotherapy, depending on the stage of the disease.
  • Infection with HCV may influence the initial appearance of ONHL because it is associated with more widespread disease at the onset.
  • [MeSH-major] Eye Infections, Viral / virology. Eye Neoplasms / virology. Hepatitis C / virology. Lymphoma, Non-Hodgkin / virology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Conjunctival Neoplasms / mortality. Conjunctival Neoplasms / pathology. Conjunctival Neoplasms / therapy. Conjunctival Neoplasms / virology. Disease-Free Survival. Eyelid Neoplasms / mortality. Eyelid Neoplasms / pathology. Eyelid Neoplasms / therapy. Eyelid Neoplasms / virology. Female. Humans. Lacrimal Apparatus Diseases / mortality. Lacrimal Apparatus Diseases / pathology. Lacrimal Apparatus Diseases / therapy. Lacrimal Apparatus Diseases / virology. Male. Middle Aged. Neoplasm Staging. Orbital Neoplasms / mortality. Orbital Neoplasms / pathology. Orbital Neoplasms / therapy. Orbital Neoplasms / virology. Prevalence. Radiotherapy. Retrospective Studies. Survival Rate

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  • (PMID = 20937999.001).
  • [ISSN] 1538-3601
  • [Journal-full-title] Archives of ophthalmology (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch. Ophthalmol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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14. Charlotte F, Doghmi K, Cassoux N, Ye H, Du MQ, Kujas M, Lesot A, Mansour G, Lehoang P, Vignot N, Capron F, Leblond V: Ocular adnexal marginal zone B cell lymphoma: a clinical and pathologic study of 23 cases. Virchows Arch; 2006 Apr;448(4):506-16
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Ocular adnexal marginal zone B cell lymphoma: a clinical and pathologic study of 23 cases.
  • To better characterize ocular adnexal marginal zone lymphoma of mucosa-associated lymphoid tissue (MZL-MALT), we analyzed the clinical and pathologic features of 23 patients (11 men, 12 women, median age 66 years).
  • The tumor was confined to one ocular structure in 18 cases (conjunctiva, n=8; orbit, n=8; or lacrimal gland, n=2).
  • Concurrent extraorbital disease was detected by the staging procedure in five patients, and preferentially involved other MALT sites.
  • Histogenetic B cell marker studies, available in 13 cases, showed an early post-germinal center (GC) phenotype (BCL-6(-)/IRF4(+)/CD138(-)) (n=5) or a late post-GC phenotype (BCL-6(-)/IRF4(+)/CD138(+)) (n=8), which could be helpful for discrimination from other types of small-B cell lymphoma.
  • T(11;18)(q21;q21), another MZL-MALT-specific translocation, was detected by reverse transcriptase polymerase chain reaction in four of 15 patients tested.
  • Regardless of the disease stage at diagnosis, combined chemotherapy and radiotherapy seemed to be more effective than chemotherapy alone in ocular adnexal MZL-MALT, as persistent complete remission was achieved in nine patients receiving combination therapy, while six of 14 patients treated with chemotherapy alone relapsed.
  • [MeSH-major] Eye Neoplasms / pathology. Lymphoma, B-Cell, Marginal Zone / pathology. Orbital Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Biomarkers, Tumor / analysis. Cell Nucleus / chemistry. Cell Nucleus / pathology. Combined Modality Therapy. Cytoplasm / chemistry. Cytoplasm / pathology. Diagnosis, Differential. Female. Fluorescent Antibody Technique, Direct. Germinal Center / chemistry. Germinal Center / pathology. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Remission Induction. Reverse Transcriptase Polymerase Chain Reaction. Translocation, Genetic

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  • (PMID = 16323006.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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15. Raparia K, Chang CC, Chévez-Barrios P: Intraocular lymphoma: diagnostic approach and immunophenotypic findings in vitrectomy specimens. Arch Pathol Lab Med; 2009 Aug;133(8):1233-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intraocular lymphoma: diagnostic approach and immunophenotypic findings in vitrectomy specimens.
  • CONTEXT: Diagnosis and classification of primary intraocular lymphoma can be challenging because of the sparse cellularity of the vitreous specimens.
  • OBJECTIVE: To classify and clinically correlate intraocular lymphoma according to the World Health Organization (WHO) classification by using vitrectomy specimens.
  • DESIGN: Clinical history, cytologic preparations, flow cytometry reports, and outcome of 16 patients diagnosed with intraocular lymphoma were reviewed.
  • The cases included 9 primary diffuse large B-cell lymphomas of the CNS type; 2 diffuse large B-cell lymphomas, not otherwise specified; 1 extranodal, low-grade, marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT); 1 precursor B-lymphoblastic lymphoma; and 3 peripheral T-cell lymphomas, not otherwise specified.
  • Of note, all 11 cases of diffuse large B-cell lymphoma were CD10-.
  • All the patients received systemic chemotherapy and radiation therapy.
  • CONCLUSIONS: Intraocular lymphoma cases can be adequately classified according to the WHO classification.
  • Diffuse large B-cell lymphoma, CD10- and most likely of non-germinal center B-cell-like subgroup, is the most common subtype of non-Hodgkin lymphoma in this site, in contrast to ocular adnexal lymphoma for which MALT lymphoma is the most common subtype.
  • [MeSH-major] Eye Neoplasms / diagnosis. Immunophenotyping. Lymphoma, Non-Hodgkin / diagnosis. Vitrectomy / methods. Vitreous Body / pathology
  • [MeSH-minor] Adult. Aged. Cytodiagnosis / methods. Female. Flow Cytometry. Humans. Lymphoma, B-Cell, Marginal Zone / classification. Lymphoma, B-Cell, Marginal Zone / diagnosis. Lymphoma, B-Cell, Marginal Zone / surgery. Lymphoma, Large B-Cell, Diffuse / classification. Lymphoma, Large B-Cell, Diffuse / diagnosis. Lymphoma, Large B-Cell, Diffuse / surgery. Male. Middle Aged. Retrospective Studies. Survival Rate. Texas / epidemiology. World Health Organization. Young Adult

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  • (PMID = 19653716.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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16. Esmaeli B, McLaughlin P, Pro B, Samaniego F, Gayed I, Hagemeister F, Romaguera J, Cabanillas F, Neelapu SS, Banay R, Fayad L, Wayne Saville M, Kwak LW: Prospective trial of targeted radioimmunotherapy with Y-90 ibritumomab tiuxetan (Zevalin) for front-line treatment of early-stage extranodal indolent ocular adnexal lymphoma. Ann Oncol; 2009 Apr;20(4):709-14
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  • [Title] Prospective trial of targeted radioimmunotherapy with Y-90 ibritumomab tiuxetan (Zevalin) for front-line treatment of early-stage extranodal indolent ocular adnexal lymphoma.
  • BACKGROUND: To determine the efficacy and side-effects of (90)Y ibritumomab tiuxetan (Zevalin) as front-line treatment in patients with early-stage extranodal indolent lymphoma of the ocular adnexa (orbit, conjunctiva, or eyelid).
  • PATIENTS AND METHODS: From August 2004 to November 2007, 12 patients with stages I-E extranodal indolent lymphoma of the ocular adnexa were enrolled in a prospective trial of rituximab followed by (90)Y ibritumomab tiuxetan (Zevalin therapeutic regimen).
  • For each patient, clinical examinations and imaging studies were used to document response to therapy using the The International Working Group response criteria.
  • All patients had (111)In ibritumomab tixuetan imaging to confirm expected biodistribution before (90)Y-Zevalin therapy; in addition, three patients had an optional single photon emission computed tomography-computed tomography scan to estimate the absorbed radiation dose to the orbital and ocular tissues.
  • Nine patients had mucosa-associated lymphoid tissue lymphoma of conjunctiva or orbit; three patients had grades 1-2 follicular lymphoma of orbit.
  • One patient who had been deemed stage I-E initially was found to have another lesion in her deltoid muscle on positron emission tomography 2 weeks after enrollment.
  • Ten patients had a complete response and two partial response (PR) within 3 months of treatment.
  • One patient had a recurrence in the upper eyelid 6 months after an initial PR; he then received 30 Gy of external-beam radiotherapy (EBRT).
  • His disease later progressed again in the orbit and he is currently being considered for other treatments.
  • A second patient who attained a PR has remained stable with no progression 12 months after treatment.
  • With a median follow-up time of 20 months (range 6-44 months), there were no cases of distant (extraorbital) relapse.
  • The estimated absorbed radiation dose to the orbital soft tissues was <3 Gy, 10 times lower than that with EBRT.
  • CONCLUSIONS: Rituximab followed by (90)Y ibritumomab tiuxetan is an effective and safe front-line treatment for early-stage extranodal indolent B-cell lymphoma of the ocular adnexa.

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  • (PMID = 19150940.001).
  • [ISSN] 1569-8041
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Yttrium Radioisotopes; 0 / ibritumomab tiuxetan
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17. Apostol S, Filip M, Nechita A, Filip A: A lymphoid conjunctival tumor--clinical aspects; therapeutical options. Oftalmologia; 2005;49(1):26-9

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  • [Title] A lymphoid conjunctival tumor--clinical aspects; therapeutical options.
  • The therapeutical and also investigational approach was to excise all suspected tissue and to biopsy to determine if benign or malignant (clinically indistinguishable).
  • The conjunctival biopsy revealed a non-Hodgkin malignant conjunctival MALT lymphoma.
  • In this case, of ocular adnexal MALT lymphoma with localised disease, we took into account local treatment: external beam radiation therapy, topical use of chemotherapy agents and biologic therapy (subconjunctival injections with interferon-alpha).
  • Systemic chemotherapy was avoided.
  • [MeSH-major] Conjunctival Neoplasms. Lymphoma, B-Cell, Marginal Zone
  • [MeSH-minor] Administration, Topical. Adult. Anti-Bacterial Agents / therapeutic use. Antineoplastic Agents / therapeutic use. Antineoplastic Agents, Hormonal / therapeutic use. Biopsy. Combined Modality Therapy / methods. Female. Humans. Immunohistochemistry. Injections. Treatment Outcome

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  • (PMID = 15934333.001).
  • [ISSN] 1220-0875
  • [Journal-full-title] Oftalmologia (Bucharest, Romania : 1990)
  • [ISO-abbreviation] Oftalmologia
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Romania
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Hormonal
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18. Yoo SB, Kim YA, Jeon YK, Kim CW: CD5-undetected by immunohistochemistry, t(11;14)(q13;q32)-positive conjunctival mantle cell lymphoma: a case report. Pathol Res Pract; 2008;204(10):779-83
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  • [Title] CD5-undetected by immunohistochemistry, t(11;14)(q13;q32)-positive conjunctival mantle cell lymphoma: a case report.
  • Most primary ocular adnexal lymphomas are extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT).
  • A few cases of ocular adnexal mantle cell lymphomas have been reported in the literature.
  • We present a case of mantle cell lymphoma presenting as conjunctival mass.
  • Histopathologic examination showed proliferation of monomorphous small-to-medium sized lymphoid cells.
  • PCR analysis for immunoglobulin heavy chain gene rearrangement showed monoclonal B-cell proliferation. t(11;14)(q13;q32), involving the CCND1 and IGH genes, was detected in interphase fluorescent in situ hybridization using formalin-fixed, paraffin-embedded tissue; however, MALT1 gene translocation was not observed.
  • The final diagnosis was mantle cell lymphoma.
  • There was no lymphadenopathy; however, bone marrow involvement of the lymphoma was suspected.
  • The patient has been receiving systemic chemotherapy.
  • This case emphasizes the differential diagnosis of conjunctival mantle cell lymphoma from extranodal marginal zone B-cell lymphomas of MALT regarding the clinical and pathological aspects.
  • [MeSH-major] Antigens, CD5 / analysis. Conjunctival Neoplasms / diagnosis. Cyclin D1 / genetics. Immunoglobulin Heavy Chains / genetics. Immunohistochemistry. Incidental Findings. Lymphoma, B-Cell, Marginal Zone / diagnosis. Lymphoma, Mantle-Cell / diagnosis
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cell Proliferation. Diagnosis, Differential. Gene Expression Regulation, Neoplastic. Gene Rearrangement, B-Lymphocyte. Humans. In Situ Hybridization, Fluorescence. Male. Middle Aged. Translocation, Genetic

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  • (PMID = 18572329.001).
  • [ISSN] 0344-0338
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antigens, CD5; 0 / CCND1 protein, human; 0 / Immunoglobulin Heavy Chains; 136601-57-5 / Cyclin D1
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19. Bernardini FP, Bazzan M: Lymphoproliferative disease of the orbit. Curr Opin Ophthalmol; 2007 Sep;18(5):398-401
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • 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.
  • [MeSH-major] Lymphoma / pathology. Orbital Neoplasms / pathology

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  • (PMID = 17700233.001).
  • [ISSN] 1040-8738
  • [Journal-full-title] Current opinion in ophthalmology
  • [ISO-abbreviation] Curr Opin Ophthalmol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 21
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20. Ferreri AJ, Ponzoni M, Dognini GP, Du MQ, Doglioni C, Radford J, Dolcetti R, Cavalli F: Bacteria-eradicating therapy for ocular adnexal MALT lymphoma: questions for an open international prospective trial. Ann Oncol; 2006 Nov;17(11):1721-2
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  • [Title] Bacteria-eradicating therapy for ocular adnexal MALT lymphoma: questions for an open international prospective trial.

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  • [CommentOn] Ann Oncol. 2006 Mar;17(3):484-7 [16500916.001]
  • (PMID = 16760269.001).
  • [ISSN] 0923-7534
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Comment; Letter
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; N12000U13O / Doxycycline
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21. Ferreri AJ, Dognini GP, Ponzoni M, Pecciarini L, Cangi MG, Santambrogio G, Resti AG, De Conciliis C, Magnino S, Pasini E, Vicari N, Dolcetti R, Doglioni C: Chlamydia-psittaci-eradicating antibiotic therapy in patients with advanced-stage ocular adnexal MALT lymphoma. Ann Oncol; 2008 Jan;19(1):194-5
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  • [Title] Chlamydia-psittaci-eradicating antibiotic therapy in patients with advanced-stage ocular adnexal MALT lymphoma.

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  • (PMID = 18073219.001).
  • [ISSN] 1569-8041
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Letter; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; N12000U13O / Doxycycline
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