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.
Highlights
Primary ocular adnexal lymphomas (POALs) present in the orbit, extraocular muscle, eyelids, lacrimal gland, or conjunctiva and represent up to 1% of all nonHodgkin lymphomas [1, 2]
We observed that cytotoxic chemotherapy and chemotherapy combined with rituximab were effective in treating localized and disseminated POALs, most of which received a histological classification of Mucosa-associated lymphoid tissue (MALT) lymphoma
Considering that low-dose alkylating chemotherapy is less toxic than a combined chemotherapy regimen, such as CVP or CHOP, our results indicate that in addition to radiotherapy, lowdose alkylating chemotherapy alone can be an alternative first-line treatment for patients who are concerned about potential late ophthalmic complications of radiotherapy, or whose conditions are not suitable for radiotherapy
Summary
Primary ocular adnexal lymphomas (POALs) present in the orbit, extraocular muscle, eyelids, lacrimal gland, or conjunctiva and represent up to 1% of all nonHodgkin lymphomas [1, 2]. A proportion of patients with a histologic diagnosis of low-grade MALT lymphoma of POAL (MALT-POAL) can be treated using doxycycline (conventionally used for eradicating Chlamydia psittaci [5, 6]), local radiotherapy is the standard first-line treatment for patients with MALT-POAL in Western countries [7]. Previous studies have demonstrated that radiotherapy provides an immediate effect and excellent local control of indolent POALs, including MALT lymphoma and FL; late recurrences have been reported [8,9,10]. Local radiotherapy to the ocular adnexal areas possibly causes acute and late side effects, such as cataract, dry eyes, keratitis, and retinopathy [11, 12]
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