Abstract
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.
Highlights
Ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (OAML) is the most common type of ocular lymphoma and its prevalence is higher in Asia than in western countries [1,2,3]
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, 9,10,11,12], 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
Previous studies using radiotherapy have demonstrated that the disease recurred predominantly in non-irradiated contralateral and distant areas but we did not observe distant recurrence in our study patients, which suggests that systemic chemoimmunotherapy may reduce the risk of distant recurrence
Summary
Ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (OAML) is the most common type of ocular lymphoma and its prevalence is higher in Asia than in western countries [1,2,3]. Limited-stage OAML has been reported to have a recurrence rate of 25% following radiotherapy [4], and the risk of recurrence is higher when the disease involves both eyes or spreads beyond the conjunctiva [5,6,7,8]. 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, 9,10,11,12], 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. Radiotherapy 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
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