Outcomes of Primary Lymphoma of the Ocular Adnexa (Orbital Lymphoma) Treated with Radiotherapy

Woolf, David; Kuhan, H.; Shoffren, O.; Akinnawo, E.M.; Sivagurunathan, B.; Boyce, H. Worth; Plowman, P N

doi:10.1016/j.clon.2014.10.002

Cited by 35 publications

(25 citation statements)

References 46 publications

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“…Similar to gastric EMZL, there was no significant difference in the prevalence of early stage disease in ocular versus non‐ocular EMZL. Radiation therapy achieved a high rate of CRs among ocular adnexal EMZL patients with early stage disease, similar to previous reports (Son et al , ; Goda et al , ; Hashimoto et al , ; Harada et al , ; Woolf et al , ) yet the responses seemed less durable than gastric EMZL in our series. Rituximab monotherapy therapy was employed more frequently in patients with advanced stage disease and a favourable response was seen in the majority of patients.…”

Section: Discussionsupporting

confidence: 92%

Dual institution experience of extranodal marginal zone lymphoma reveals excellent long‐term outcomes

Starr

Caimi

et al. 2016

Br J Haematol

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Extranodal marginal zone lymphoma (EMZL) is a B-cell lymphoma arising from mucosa-associated lymphoid tissue (MALT). The disease characteristics, clinical course and treatment vary considerably based on site of involvement. Because long-term outcome data for EMZL are limited, we sought to describe the clinical details of a large number of patients with EMZL evaluated at the Case Comprehensive Cancer Center over a 12-year period to identify prognostic markers including the impact of site of involvement. We identified 211 cases of EMZL involving the stomach (30%), ocular adnexa (19%), lungs (16%) and intestines (9%). Initial treatment included antibiotics (18%), radiation (21%), rituximab (20%), chemotherapy (3%), rituximab + chemotherapy (7%), surgery (17%) or observation (8%). After a median follow-up of 44·3 months (range 2·2-214·9), median progression-free survival (PFS) was 68·2 months (95% confidence interval [CI] 54·5-111·3) and median overall survival (OS) has not been reached. Age >60 years, elevated lactate dehydrogenase level (LDH), ≥4 lymph node groups involvement, and high follicular lymphoma international prognostic index (FLIPI) were associated with inferior PFS/OS. In summary, patients with EMZL have excellent prognosis with median OS in excess of 10 years. Age, elevated LDH, advanced disease, and high FLIPI score are associated with worse outcomes.

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Section: Discussionsupporting

confidence: 92%

Dual institution experience of extranodal marginal zone lymphoma reveals excellent long‐term outcomes

Starr

Caimi

et al. 2016

Br J Haematol

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“…Previous studies using radiotherapy have demonstrated that the disease recurred predominantly in areas that were not irradiated, mostly the contralateral eye and distant extranodal organs [ 7 , 9 – 12 ], so that we reasoned that the relapse rate of nonirradiated areas might be reduced if the limited staged patients with the higher risk factors receive systemic chemoimmunotherapy rather than radiotherapy. In addition, the target volume of irradiation for conjunctiva-only disease is the whole conjunctiva and the risk of cataract can be reduced with lens shielding, but for disease extending beyond the conjunctiva, the whole orbit is generally covered without lens shielding so that the risk of cataract can be considerable [ 9 , 13 , 14 ].…”

Section: Introductionmentioning

confidence: 99%

Frontline treatment with chemoimmunotherapy for limited-stage ocular adnexal MALT lymphoma with adverse factors: a phase II study

et al. 2017

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BackgroundRadiotherapy is a commonly used treatment for limited-stage ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) but showed a substantial relapse risk if the disease involves beyond-conjunctiva or bilateral conjunctivae. Systemic chemoimmunotherapy may be an alternative frontline therapy for the limited disease with those adverse prognostic factors.Patients and methodsWe designed a multicenter, phase II study of the chemoimmunotherapy, rituximab, cyclophosphamide, vincristine, and prednisolone (R-CVP) for the treatment of patients with limited-stage OAML with bilateral or beyond-conjunctival involvement. Thirty-three patients with Ann Arbor stage I OAML with the adverse factors were enrolled. Patients received six cycles of R-CVP followed by two cycles of rituximab therapy.ResultsAt the end of treatment, all the enrolled patients had responded. The cumulative complete response achievement was 93.9% at 2 years. At a median follow-up of 50.6 months, three patients had progressed. Progression-free survival and overall survival at 4 years was 90.3±5.3% and 100%, respectively.ConclusionsThis phase II study demonstrated durable efficacy of R-CVP chemoimmunotherapy, which has promise as an alternative frontline therapy for the limited-stage OAML patients with adverse prognostic factors.Clinical trial registrationNCT01427114.

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“…20 Radiation therapy alone demonstrates excellent outcomes, especially in low-grade lymphomas, with 92% disease-free survival at 5 years. However, there is room for improvement in high-grade disease with 3 of 6 high-grade lymphoma patients dying of progressive distant disease Several studies recommend treatment of primary orbital lymphoma with a total dose of 30 Gy, 12,17 which balances the therapeutic benefits of treatment with an acceptable level of toxicity. Yadav and Sharma 21 suggest that local control is poor with doses <30 Gy, however, our results did not support this observation.…”

Section: Discussionmentioning

confidence: 98%

“…1,4,[7][8][9][10][11][12][13][14][15][16][17][18][19][20]22,23 Radiotherapy provides excellent local control of disease and should be used as the first-line treatment for primary orbital lymphomas. 20 Radiation therapy alone demonstrates excellent outcomes, especially in low-grade lymphomas, with 92% disease-free survival at 5 years.…”

Section: Discussionmentioning

confidence: 99%

Radiotherapy in the Management of Orbital Lymphoma

Kharod

Herman

Morris

et al. 2018

American Journal of Clinical Oncology

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Outcomes of Primary Lymphoma of the Ocular Adnexa (Orbital Lymphoma) Treated with Radiotherapy

Cited by 35 publications

References 46 publications

Dual institution experience of extranodal marginal zone lymphoma reveals excellent long‐term outcomes

Dual institution experience of extranodal marginal zone lymphoma reveals excellent long‐term outcomes

Frontline treatment with chemoimmunotherapy for limited-stage ocular adnexal MALT lymphoma with adverse factors: a phase II study

Radiotherapy in the Management of Orbital Lymphoma

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