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Chemotherapy alone is an alternative treatment in treating localized primary ocular adnexal lymphomas.

ABSTRACT: This study investigated the treatment efficacy and long-term adverse effects of various treatment modalities for primary ocular adnexal lymphomas (POALs). We retrospectively reviewed 107 patients who received first-line chemotherapy, radiotherapy, and other treatment modalities from 1990 to 2015. Nighty-three (87%) patients were diagnosed with mucosa-associated lymphoid tissue (MALT) lymphoma, with the orbit being the most common site (49 patients, 46%). Among 91 patients with stage I-IIE1 disease, 26 underwent chemotherapy, 34 underwent radiotherapy, and 31 received other treatment modalities. For chemotherapy, radiotherapy, and other treatment modalities, the 5-year event-free survival (EFS) rates were 90.0%, 89.7%, and 85.8% and the 5-year overall survival (OS) rates were 100%, 90.4%, and 87.5%, respectively. Moreover, among 80 patients with stage I-IIE1 MALT lymphoma, the complete remission, 5-year EFS and OS rates were not significantly different between patients receiving chemotherapy and those receiving radiotherapy. Among 16 patients with stage IIE2-IVE disease, the 5-year EFS rates for chemotherapy alone (n = 11) and combined radiotherapy and chemotherapy (n = 5) were 61.7% and 80%, respectively, whereas the 5-year OS rate for both groups was 80.0%. Neutropenia (15.2%) was the most common side effect in patients who received chemotherapy, whereas cataract (16.3%) was the most common late sequela in patients who received radiotherapy. Multivariate analysis revealed that old age (> 60 y) and an advanced stage (stage III/IV) were prognostic factors for poor OS. Our results indicate that chemotherapy yields satisfactory disease control and fewer side effects, and acts as an alternative therapy for patients with localized POALs.

SUBMITTER: Ma WL

PROVIDER: S-EPMC5655287 | biostudies-other | 2017 Oct

REPOSITORIES: biostudies-other

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Chemotherapy alone is an alternative treatment in treating localized primary ocular adnexal lymphomas.

Ma Wei-Li WL Yao Ming M Liao Shu-Lang SL Tang Jih-Luh JL Wang Yao-Ching YC Kuo Sung-Hsin SH Cheng Ann-Lii AL

Oncotarget 20170615 46

This study investigated the treatment efficacy and long-term adverse effects of various treatment modalities for primary ocular adnexal lymphomas (POALs). We retrospectively reviewed 107 patients who received first-line chemotherapy, radiotherapy, and other treatment modalities from 1990 to 2015. Nighty-three (87%) patients were diagnosed with mucosa-associated lymphoid tissue (MALT) lymphoma, with the orbit being the most common site (49 patients, 46%). Among 91 patients with stage I-IIE1 disea ...[more]

PMID: 29113392

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Project description:BackgroundThe choice of a primary treatment for ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) depends on the extent of tumor spread. However, radiotherapy is commonly used as a first-line therapy despite ophthalmic complications, because most OAMLs are in a limited stage of progression. However, the initial therapeutic modality, including chemotherapy and treatment of the advanced stage, has not been fully established for OAML. Therefore, we evaluated the optimal therapeutic options and survival outcome-related parameters for patients with primary OAML.MethodsWe evaluated 208 consecutive patients with primary OAML who were diagnosed at the Catholic University Lymphoma Group between January 2004 and April 2015.FindingsDuring a median follow-up of 70.0 months (range, 3.2-182.0 months) in 208 patients with primary OAML, most patients were female and the median age was 46 years old. Overall survival (OS) and progression-free survival (PFS) at 13 years were excellent (92.7% and 69.7%, respectively). Of the 117 patients who received the first-line radiotherapy, 92% achieved complete remission (CR), usually by being treated with less than 30 Gy. Radiation-related ophthalmic complications including dry eye syndrome (59%) and cataract (22%) caused a decline in the quality of life (QoL). Chemotherapy alone was used to treat 86 OAML patients, with 84.9% achieving CR and 12.8% achieving partial remission with tolerable toxicities. There were no differences in survival outcomes between patients treated with radiotherapy versus those treated with rituximab-containing chemotherapy, although the latter group had more advanced stages of OAML (OS, p = 0.057; PFS, p = 0.075).InterpretationOAML patients were predominantly female and relatively young, and radiotherapy as a primary therapeutic option was more likely to lead to radiation-related complications, resulting in lower QoL. On the other hand, frontline chemotherapy showed consistent therapeutic outcomes with tolerable toxicities compared to radiotherapy, and there were no long-term or delayed adverse events. Therefore, when considering therapeutic efficacy and therapy-related QoL, chemotherapy is recommended for younger patients, and radiotherapy is recommended for older and chemotherapy-ineligible patients.FundingA National Research Foundation of Korea (NRF) grant funded by the Korean government (MSIP) (No. NRF-2016R1A2B4007282).

Project description: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.

Dataset Information

Chemotherapy alone is an alternative treatment in treating localized primary ocular adnexal lymphomas.

Publications

Chemotherapy alone is an alternative treatment in treating localized primary ocular adnexal lymphomas.

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