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      Orbital and Eyelid B-Cell Lymphoma: A Multicenter Retrospective Study


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          The treatment of orbital and eyelid B-cell lymphoma remains a field of progress. The aim of our study was to analyze patients diagnosed, staged and treated for orbital and eyelid B-cell lymphoma to assess clinical characteristics, treatment outcomes and recurrence patterns. We included in this study 141 cases of orbital and eyelid B-cell lymphoma. We found five lymphoma subtypes and we confirmed that the histopathologic subtype and the type of treatment were found to be the main factors influencing treatment outcome.


          Background: The aim of this study was to analyze patients diagnosed, staged and treated for orbital and eyelid B-cell lymphoma (OEL). Methods: One hundred and forty-one cases of OEL were included in this study. Primary endpoints were to analyze the histopathologic findings, the main risk factors and the type of treatment and to correlate them with recurrence of OEL. The secondary endpoint was to determine the progression-free survival (PFS) time. Results: Extranodal marginal zone B-cell lymphoma was the most frequent subtype (66%), followed by small lymphocytic lymphoma (12.7%), diffuse large B-cell lymphoma (DLBCL) (9.2%), follicular lymphoma (6.6%), mantle cell lymphoma (4.3%) and Burkitt lymphoma (1.2%). The probability of relapse was influenced by the histopathologic subtype DLBCL (OR = 7.7, 95% CI 1.8–32.3) and treatment with chemotherapy (OR = 14.9, 95% CI 2.6–83.7). Multivariate analysis showed that the histopathologic subtype DLBCL and chemotherapy treatment retained statistical significance for a poorer PFS, with hazard ratios of 8.581 ( p = 0.0112) and 9.239 ( p = 0.0094), respectively. Conclusions: Five lymphoma subtypes were found in patients with OEL. The histopathologic subtype and the type of treatment were found to be the main factors influencing treatment outcome.

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          Most cited references53

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          American Joint Committee on Cancer acceptance criteria for inclusion of risk models for individualized prognosis in the practice of precision medicine.

          The American Joint Committee on Cancer (AJCC) has increasingly recognized the need for more personalized probabilistic predictions than those delivered by ordinal staging systems, particularly through the use of accurate risk models or calculators. However, judging the quality and acceptability of a risk model is complex. The AJCC Precision Medicine Core conducted a 2-day meeting to discuss characteristics necessary for a quality risk model in cancer patients. More specifically, the committee established inclusion and exclusion criteria necessary for a risk model to potentially be endorsed by the AJCC. This committee reviewed and discussed relevant literature before creating a checklist unique to this need of AJCC risk model endorsement. The committee identified 13 inclusion and 3 exclusion criteria for AJCC risk model endorsement in cancer. The emphasis centered on performance metrics, implementation clarity, and clinical relevance. The facilitation of personalized probabilistic predictions for cancer patients holds tremendous promise, and these criteria will hopefully greatly accelerate this process. Moreover, these criteria might be useful for a general audience when trying to judge the potential applicability of a published risk model in any clinical domain. CA Cancer J Clin 2016;66:370-374. © 2016 American Cancer Society.
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            ESMO Consensus conferences: guidelines on malignant lymphoma. part 2: marginal zone lymphoma, mantle cell lymphoma, peripheral T-cell lymphoma.

            To complement the existing treatment guidelines for all tumour types, ESMO organizes consensus conferences to focus on specific issues in each type of tumour. In this setting, a consensus conference on the management of lymphoma was held on 18 June 2011 in Lugano, next to the 11th International Conference on Malignant Lymphoma. The conference convened ∼30 experts from all around Europe, and selected six lymphoma entities to be addressed; for each of them, three to five open questions were to be addressed by the experts. For each question, a recommendation should be given by the panel, referring to the strength of the recommendation based on the level of evidence. This consensus report focuses on the three less common lymphoproliferative malignancies: marginal zone lymphoma, mantle cell lymphoma, and peripheral T-cell lymphomas. A first report had focused on diffuse large B-cell lymphoma, follicular lymphoma, and chronic lymphocytic leukaemia.
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              Localized mucosa-associated lymphoid tissue lymphoma treated with radiation therapy has excellent clinical outcome.

              Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a distinct lymphoma with unique clinicopathologic features. We report the clinical outcome of stage I and II MALT lymphoma treated with involved field radiation therapy (RT). From 1989 to 2000, 103 patients with stage IE and IIE disease were referred. Their median age was 60 years, with a 2:1 female predominance. Presenting sites were stomach (17 patients), orbital adnexa (31 patients), salivary glands (24 patients), thyroid gland (13 patients), and other sites (18 patients). Ninety-three patients received RT--85 received RT alone, and eight received chemotherapy and RT--with a median dose of 30 Gy. The median follow-up time was 5.1 years. A complete response (CR) to RT alone was achieved in 84 of 85 patients. Among CR patients, 14 experienced relapse. Relapse sites were mostly contralateral paired-organ or distant MALT locations and, infrequently, lymph nodes. The crude local control rate with RT was 95.3% (81 of 85 patients). No relapses were observed in patients with stomach or thyroid lymphoma, whereas 14 of 63 patients (22%) experienced relapse in the other sites. The overall 5-year survival rate was 98%, and the disease-free survival rate was 77%. Transformed lymphoma was observed in 14% of patients (two of 14) experiencing relapse. Moderate-dose RT achieved excellent local control in localized MALT lymphomas and had curative potential for three fourths of the patients. Gastric and thyroid MALT lymphomas had better outcome, whereas distant failures were common for other sites. Despite relapse, the disease often maintained an indolent course.

                Author and article information

                Cancers (Basel)
                Cancers (Basel)
                07 September 2020
                September 2020
                : 12
                : 9
                : 2538
                [1 ]UOC Oncologia Oculare, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Roma, Italy; gustavo.savino@ 123456unicatt.it (G.S.); mariaantonietta.blasi@ 123456unicatt.it (M.A.B.); remo.battendieri@ 123456guest.policlinicogemelli.it (R.B.); martina.maceroni01@ 123456icatt.it (M.M.)
                [2 ]Istituto di Oftalmologia, Università Cattolica del Sacro Cuore, 00168 Roma, Italy
                [3 ]Moorfields Eye Hospital NHS Foundation Trust, London EC1V 2PD, UK; gabriela.grimaldi@ 123456nhs.net
                [4 ]Dipartimento di Neuroscienza, della Riproduzione e di Odontostomatologia, Centro di Patologia Orbitaria. Università “Federico II” di Napoli, 80138 Napoli, Italy; fausto.tranfa@ 123456unina.it (F.T.); p.napolitano3@ 123456studenti.unimol.it (P.N.); vittorialanni@ 123456hotmail.com (V.L.); adriana.iuliano@ 123456unina.it (A.I.)
                Author notes
                [* ]Correspondence: giulia.midena01@ 123456icatt.it ; Tel.: +39-0630154528
                Author information
                © 2020 by the authors.

                Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( http://creativecommons.org/licenses/by/4.0/).

                : 06 August 2020
                : 03 September 2020

                orbital neoplasms,ocular adnexal lymphoma,orbital lymphoma,eyelid lymphoma,ocular lymphoma prognosis,ocular lymphoma treatment,precision medicine


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