Adnexal Mucosa-associated Lymphoid Tissue Lymphoma Research Articles

Long-term Results of Radiation Therapy for Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphomas

Long-term Results of Radiation Therapy for Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphomas

Comparison of Selection and Long-term Clinical Outcomes Between Chemotherapy and Radiotherapy as Primary Therapeutic Modality for Ocular Adnexal MALT Lymphoma

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).

Immunoglobulin G4 (IgG4)-Positive Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma and Idiopathic Orbital Inflammation.

To report clinical and pathological characteristics of idiopathic orbital inflammation and ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma with immunoglobulin G4 (IgG4)-positive plasma cells. A retrospective histopathological review and clinical case series. A total of 51 biopsy samples from January 2005 to December 2015 were used in this study, including 21 cases of biopsy-confirmed idiopathic orbital inflammation and 30 cases of biopsy-confirmed ocular adnexal MALT lymphoma. Most cases of ocular adnexal lymphoma were conjunctival tissue. Retrospective immunohistochemical studies were performed to estimate the IgG4 and IgG4/IgG ratios. Histopathologic features, demographic and clinical data, radiologic findings, treatment, and follow-up information for each patient were analyzed. Among idiopathic orbital inflammation, 6 (28.6%) of the 21 patients were diagnosed as "probable" ocular adnexal IgG4-related diseases and 13 (43.3%) of the 30 patients were diagnosed as MALT lymphoma with IgG4-positive plasma cells. Six cases of 13 IgG4-positive MALT lymphoma group had contralateral chronic inflammatory lesions infiltrated by IgG4-positive plasma cells, which was significantly (p = 0.007) higher than that in the IgG4-negative group. Conjunctival involvement was 69% of the IgG4-positive MALT lymphoma cases. Bilateral involvement of the ocular adnexa was significantly (p = 0.02) more frequent among IgG4-positive MALT lymphoma patients than that in IgG4-positive idiopathic orbital inflammation patients. Recurrence rate in the IgG4-positive group was higher (p = 0.05) than that in the IgG4-negative group but not significantly. This study presented an unusual framework of ocular adnexal IgG4-related inflammation, in conjunctiva. It is important to understand contralateral chronic inflammatory lesions and their relationship with IgG4-positive MALT lymphoma. Tissue biopsy and IgG4 immunostaining are required for all cases because IgG4-positive MALT lymphoma can arise from a pre-existing IgG4-positive chronic inflammatory lesions. This is the first study that performs IgG4 immunostaining for tissue from a relatively large number of conjunctival MALT lymphomas in a single center. Therefore, it will help to diagnose conjunctival lymphoproliferative disease.

A retrospective study of the treatment-associated mortality of the patients with primary ocular adnexal mucosa-associated lymphoid tissue lymphoma after the molecular and immunological diagnosis.

e19534Background: Differentiation between primary ocular adnexal mucosa-associated lymphoid tissue (POA-MALT) lymphoma and reactive lymphoid hyperplasias sometimes may be difficult. We have examine...

The clinical features of radiation cataract in patients with ocular adnexal mucosa-associated lymphoid tissue lymphoma

BackgroundTo examine the clinical features of radiation cataract in patients with ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma.MethodsTwenty-one patients with 26 eyes diagnosed with ocular adnexal MALT lymphoma (26 eyes), who were treated in Hokkaido University Hospital, were retrospectively reviewed based on medical records.ResultsOut of the 21 patients, 16 patients (21 eyes) received radiation therapy (RT) with a total dose of 30 Gy. All cases eventually achieved complete remission. Eight of these patients (11 eyes: 52.3%) required cataract surgery after RT. The mean age at surgery was 56.8 (40–70) years. The mean latency between RT and the indication for surgery was 43.3 months. The percentage of females was significantly higher in patients who required surgery (P < 0.01), compared with those without surgery. The eyes of patients who received bolus technique on radiation treatment developed cataract more frequently (P < 0.05). In contrast, none of the patients without RT required cataract surgery.ConclusionsPatients with ocular adnexal MALT lymphoma who underwent surgery for radiation cataract were seen more often in relatively young, female patients, and surgery was required about 3 years after RT. A long-term observation may be needed for patients after RT for a tumor. A female sex and the bolus technique may be risk factors for radiation cataract.

Treatment outcome and risk analysis for cataract after radiotherapy of localized ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma

PurposeWe retrospectively reviewed the results of radiotherapy for localized ocular adnexal MALT lymphoma (OAML) to investigate the risk factors of cataract.MethodsSixty-seven patients with stage IE OAML treated with radiotherapy at Seoul St. Mary’s Hospital from 2001 to 2016 were included. Median treatment dose was 30 Gy. Lens protection was done in 52 (76%) patients. Radiation therapy (RT) extent was as follows: superficial (82.1%), tumor mass (4.5%), and entire orbital socket (13.4%). The risk factors for symptomatic cataract were analyzed using the Cox proportional hazard model.ResultsMedian follow-up time was 50.9 months (range, 1.9 to 149.4 months). All patients were alive at the time of analysis. There were 7 recurrences and there was no local recurrence. Median time to recurrence was 40.4 months. There were 14 cases of symptomatic cataract. Dose >30 Gy had hazard ratio of 3.47 for cataract (p = 0.026). Omitting lens protection showed hazard ratio of 4.10 (p = 0.008).conclusionsRT achieves excellent local control of ocular MALT lymphoma. Consideration of RT-related factors such as lens protection and radiation dose at the stage of RT planning may reduce the risk of RT-induced cataract after radiotherapy.

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

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.

The expressions of metadherin and LEF-1 in mucosa-associated lymphoid tissue lymphoma of ocular adnexal.

To investigate the expressions of metadherin (astrocyte elevated gene-1, AEG-1) and lymphoid enhancer-binding factor-1 (LEF-1) in ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma. The expressions of AEG-1 and LEF-1 were detected on specimens harvested from patients suffering from MALT lymphoma and lymphadenosis of ocular adnexal in Ophthalmology Department, Affiliated Hospital of Qingdao University from 2000 to 2015 by immunohistochemical and polymerase chain reaction (PCR) analysis. AEG-1 and LEF-1 expressions in MALT lymphoma was respectively higher than that in lymphadenosis, both by immunohistochemical and PCR analysis (P<0.05). Diversity of AEG-1 and LEF-1 expressions in different Ann Arbor clinical stages showed a statistically significant result (P<0.05). A positive relevance between AEG-1 and LEF-1 was observed in MALT ocular adnexal lymphoma (r=0.435, P=0.016). The over expressions of AEG-1 and LEF-1 at the level of protein and mRNA participates in the tumorigenesis of ocular adnexal MALT lymphoma. They should act as a new biological marker for pathological diagnosis in the future.

Long-term course of patients with primary ocular adnexal MALT lymphoma: a large single-institution cohort study

AbstractWhile primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (POAML) is the most common orbital tumor, there are large gaps in knowledge of its natural history. We conducted a retrospective analysis of the largest reported cohort, consisting of 182 patients with POAML, diagnosed or treated at our institution to analyze long-term outcome, response to treatment, and incidence and localization of relapse and transformation. The majority of patients (80%) presented with stage I disease. Overall, 84% of treated patients achieved a complete response after first-line therapy. In patients with stage I disease treated with radiation therapy (RT), doses ≥30.6 Gy were associated with a significantly better complete response rate (P = .04) and progression-free survival (PFS) at 5 and 10 years (P < .0001). Median overall survival and PFS for all patients were 250 months (95% confidence interval [CI], 222 [upper limit not reached]) and 134 months (95% CI, 87-198), respectively. Kaplan-Meier estimates for the PFS at 1, 5, and 10 years were 91.5% (95% CI, 86.1% to 94.9%), 68.5% (95% CI, 60.4% to 75.6%), and 50.9% (95% CI, 40.5% to 61.6%), respectively. In univariate analysis, age >60 years, radiation dose, bilateral ocular involvement at presentation, and advanced stage were significantly correlated with shorter PFS (P = .006, P = .0001, P = .002, and P = .0001, respectively). Multivariate analysis showed that age >60 years (hazard ratio [HR] 2.44) and RT<30.6Gy (HR=4.17) were the only factors correlated with shorter PFS (P = .01 and P = .0003, respectively). We demonstrate that POAMLs harbor a persistent and ongoing risk of relapse, including in the central nervous system, and transformation to aggressive lymphoma (4%), requiring long-term follow-up.

R-CVP Chemoimmunotherapy for Limited-Stage Ocular Adnexal MALT Lymphoma with Adverse Factors: A Phase II Study

Background: Ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) has an indolent disease course and a generally good response to radiotherapy; hence, limited-stage disease is commonly treated with radiotherapy. However, after localized radiotherapy for limited-stage OAML, a relapse rate of up to 16% was reported and the main relapse sites observed in previous series were in nonirradiated areas. The relapse rate was higher if the disease involved bilateral conjunctivae or tissue beyond the conjunctiva. We designed a phase II study in which patients with newly diagnosed and limited-stage OAML with bilateral or beyond-conjunctival involvement were treated with a regimen of rituximab in combination with cyclophosphamide, vincristine, and prednisolone (R-CVP), which has been widely used for advanced-stage indolent CD20+ B cell lymphoma and in a previous trial has demonstrated efficacy in advanced-stage MALT lymphoma.Patients and methods: 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. The enrolment criteria for disease status were Ann Arbor stage IE OAML with bilateral or beyond-conjunctival involvement; bT1 or T>1 based on the tumor-node-metastasis (TNM) staging system for ocular adnexal lymphoma proposed by the American Joint Committee on Cancer. Patients were required to be ©ø18 years old, with Eastern Cooperative Oncology Group (ECOG) performance scores of 0-2, with no prior chemotherapy or radiation therapy. Exclusions were made for disease confined to unilateral conjunctiva (T1N0M0) or Ann Arbor stage III-IV disease. The primary end point of the study was response rate, defined as the CR and partial response (PR) rates, based on the revised response criteria for malignant lymphoma. The secondary end point was PFS and overall survival.Results: The median age of the patients was 49 years (range, 19-74 years). The anatomic location of disease was the conjunctiva in 12 patients (36.4%), the orbit in 13 (39.4%), the eyelid in five (15.2%), and the lacrimal duct or gland in three (9.1%). Fourteen patients (42.4%) had bilateral disease at presentation. All study patients (100%) responded to the treatment. After the treatment, 28 patients (84.8%) were in CR and five patients (15.2%) in PR. With the median follow up of 26.9 months (range, 7.4-41.0 months), the estimated cumulative CR rate was 93.9% at 3 years (Figure 1). The cumulative CR rate was significantly lower in the patients with beyond-conjunctival disease (T>1) than in the patients with bilateral conjunctiva-confined disease (bT1) (91.3% vs. 100%; P = 0.022). The cumulative CR rate was lower for patients aged ©ø49 than in patients younger than 49 years (90.4% vs. 100%; P = 0.034). Multivariate analysis indicated that in our study patients beyond-conjunctival involvement (T>1) was an independent predictor of cumulative CR rate (hazard ratio [HR] 0.424, P = 0.044). Among the study patients, one patient, who had bT1N0M0 disease, relapsed at the same location in the eye at 21.7 months after initiation of treatment. The estimated PFS at 3 years was 95.5 ¡¾ 4.4%. No death was observed and OS was 100%. No patients experienced ophthalmic complications (Figure2).Conclusions: The R-CVP regimen was efficient for disease control up to 3 years because all study patients responded, cumulative CR rate was 93.9% and the PFS was 95.9% at 3 years. The common relapse sites in patients who received radiotherapy were in nonirradiated areas, suggesting that the systemic antitumor coverage of this chemoimmunotherapy may contribute to lowering the risk of distant relapse in our patients. We conclude that the R-CVP regimen can be effective alternative treatment which avoids radiation hazards and efficiently achieves CR in patients with limited-stage OAML, even those with adverse factors of radiotherapy. [Display omitted] [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Localized orbital adnexal mucosa-associated lymphoid tissue lymphoma treated with radiation and rituximab: a single institute experience.

e19034Background: Standard treatment for patients with orbital mucosa-associated lymphoid tissue (MALT) lymphoma is not established due to paucity of prospective trials, small numbers of patients i...

Clinical Manifestations and Treatment Results in Accordance with Frontline Treatment Modalities for Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma (OAML)

Background: localized ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) was effectively treated with frontline radiotherapy. however it is sometimes associated with radiation-related complications of ocular structures, as well as distant relapse or delayed relapse. Although there are no definitive optimal treatment approaches for these patients, we investigated the results in accordance with frontline treatment modalities for OAML.Patients and methods: 159 patients with biopsy-confirmed primary OAML between 2007 and 2015 were analyzed retrospectively. All the patients were sorted by risk-stratification with Ann-Arbor staging and the tumor, node, metastasis (TNM) staging system, and treatment modalities according to anatomic location, prognosis factors, which were supported by ophthalmologist.Results: first-line radiotherapy had overall survival(OS) of 100%, event-free survival(EFS) of 90%. However frontline-radiotherapy group has 100% of survival with 55% of moderate to severe dry eye syndrome, 37.4% of cataract incidence, 11% of cataract-related operations, and 1% (n=1) of radiation-related blindness. Although 5-year EFS is good in radiotherapy group (90% vs 64% in radiotherapy group vs chemotherapy, p<0.001), most of aggressive factors (young age at diagnosis, conjunctival lesions, Ann Arbor stage I, early T1N0M0 stage, and no distant extranodal metastasis including of bone marrow involvement) showed in radiotherapy group in comparison with chemotherapy group. In subgroup analysis of first-line chemotherapy, rituximab-containing group has more good response of event-free survival (p= 0.041) with tolerable hematologic toxicities compared to frontline radiotherapy group.Conclusion: OAML with localized to conjunctiva alone was treated with frontline-radiotherapy and non-conjuctival lesions treated with chemotherapy. Radiotherapy as first-line is good local control-therapeutic option in patients with localized to conjunctiva alone, but management of radiation-associated complication is concerned troubles. In advanced OAML including of extra-conjunctival lesions, systemic chemotherapy is best options. rituximab-based regimen could be feasible to obtain good disease-free survival and response rate.Table 1Patient's characteristics between frontline-chemotherapy and radiation group (n= 159)FactorsPrimary Chemotherapy group(n = 68)Primary Radiation group(n = 91)P-valueGender (male)37 (54%)28 (30.8%)0.003Age at diagnosis (median, year)50430.003involvement of both eyes at diagnosis22 (32.4%)23 (25.3%)0.75Conjuctival lesion at diagnosis22 (32.4%)76 (83.5%)<0.001Ki-67 on pathologic stain (median, %)540.545Ann-Arbor stage I23 (66.2%)3 (96.7%)<0.001LDH at diagnosis (mg/L)3553440.216Patients no. with T1N0M021 (30.9%)78 (85.7%)<0.001Bone marrow involvement7 (10.3%)0 (0%)<0.001Patients no. with N1~N3 staging10 (14.7%)0 (0%)<0.001Patients no. with T0N0M1~27 (10.3%)0 (0%)<0.001Patients treated with frontline chemotherapy were classified in more aggressive or advanced stage which compare to patients treated with primary radiotherapy statistically.Table 2Patient's characteristics between Rituximab containing group and non-rituximab in frontline chemotherapy subgroup (n=68)FactorsRitixumab-containing(n = 26)No Rituximab(n = 42)P-valueGender (male)0.942Age at diagnosis (median, year)52490.411involvement of both eyes at diagnosis0.057Conjuctival lesion at diagnosis0.087Ki-67 on pathologic stain (median, %)430.540Ann-Arbor stage I0.532LDH at diagnosis (mg/L)3533560.782Patients no. with T1N0M00.585Patients no. with N1~N3 staging0.659Patients no. with T0N0M1~20.695There are statistically no differences between patients treated with Rituximab-containing regimen and non-rixuximab in frontline chemotherapy subgroup. [Display omitted] [Display omitted] [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Genome-Wide Analysis of Ocular Adnexal Lymphoproliferative Disorders Using High-Resolution Single Nucleotide Polymorphism Array.

We identified the genomic signature of ocular adnexal lymphoproliferative disorders (LPDs), especially ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma, IgG4-related ophthalmic disease (IgG4-ROD), reactive lymphoid hyperplasia (RLH), and diffuse large B-cell lymphoma (DLBCL). We included 52 subjects with ocular adnexal LPDs (13 orbital MALT lymphomas, 16 conjunctival MALT lymphomas, 13 IgG4-RODs, 4 RLHs, and 6 DLBCLs) who had been treated at the Tokyo Medical University Hospital from 2008 to 2012. Genomic DNA was extracted from the tumor tissues and subjected to high-resolution single nucleotide polymorphism array (SNP-A) karyotyping using GeneChip Human Mapping 250K SNP arrays. The array data were investigated using Copy Number Analysis for GeneChips (CNAG) software. In ocular adnexal MALT lymphomas, the most frequent copy number (CN) gain region was trisomy 3 detected in 31% (9/29), followed by trisomy 18 in 17% (5/29), and 6p and 21q in 14% (4/29). The most frequent CN loss regions were 6q and 9p, detected in 7% (2/29). Uniparental disomy was detected on 6q in 14% (4/29), followed by 3q in 10% (3/29). Copy number variations (CNVs) were not detected in IgG4-RODs and RLHs. Conversely, CNVs were more frequent in DLBCLs than in ocular adnexal MALT lymphomas. Copy number variations were detected in 77% (10/13) of orbital MALT lymphomas and in 67% (11/16) of conjunctival MALT lymphomas. High-resolution single nucleotide polymorphism array is a useful method for discriminating ocular adnexal lymphomas from benign LPDs. The differences in the chromosomal abnormality patterns may reflect the activity of ocular adnexal LPDs.

Suggestion of response evaluation criteria in patients with ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML).

Ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) has been recognized as most common primary orbital malignancy. However, little was known about the response criteria for OAML. Our aim was to suggest response criteria for nonconjunctival OAML; the response evaluation of which using the conventional response criteria is inappropriate. A retrospective chart review of 34 eyes from 30 patients diagnosed with nonconjunctival OAML was conducted, focusing on the change in tumor size based on linear bi-dimensional and three-dimensional methods in magnetic resonance imaging (MRI) of the orbit. The maximum tumor response period of each case was investigated, and the expected optimal response period was calculated using regression analysis. In 30 evaluable patients, the median time taken for the maximum tumor response was 6months (range, 3-18). More than 75% of patients attained maximal tumor response in 6months after initial therapy for follow-up period, the median value of which was 30months (range, 15-77). Based on the regression analysis, it took 4.7months for the maximum diameter (2r) of tumor to decrease by 50% of initial lesion size. We cautiously suggest that optimal response could be defined as 50% reduction of the maximum diameter in 6months since the treatment was initiated, and that only observation without additional therapy is enough for nonconjunctival OAML, if optimal response is achieved.

Comparison of Ocular Adnexal Mantle Cell Lymphoma and MALT Lymphoma

Purpose: Mantle cell lymphoma (MCL) is known to have systemic dissemination with poor prognosis, but very few cases have been reported in Korea. Therefore, we analyzed clinical features and prognosis of MCL by comparing 3 cases of ocular adnexal MCL and mucosa-associated lymphoid tissue (MALT) lymphoma. Methods: The medical records of patients with ocular adnexal MALT lymphoma and MCL from Chungnam National University Hospital and Pusan National University Hospital from January 1999 to April 2014 were retrospectively reviewed. Immunohistochemical examination was selectively used to diagnose lymphoma subtypes. Systemic dissemination, treatment response, and recurrence were identified using radiological testing and bone marrow aspiration. Results: This study included 39 patients with ocular and ocular adnexal MALT lymphoma and 1 patient with MCL from Chungnam National University Hospital and 53 patients with MALT lymphoma and 2 patients with MCL from Pusan National University Hospital. All 3 (100%) patients diagnosed with ocular adnexal MCL were over 60 years of age. However, 28 of 92 (30.4%) patients diagnosed with MALT lymphoma were over 60 years of age. In MALT lymphoma, 3 of 92 patients presented with systemic dissemination and most patients recovered with radiotherapy. Conversely, all 3 patients with MCL showed systemic dissemination and recurrence after radiotherapy or chemotherapy, therefore, additional chemotherapy was required. Conclusions: Contrary to MALT lymphoma, ocular and ocular adnexal MCL usually presented with systemic dissemination and complete remission was difficult even though many regimens of chemotherapy were attempted. Because MCL can be misdiagnosed as MALT lymphoma, careful evaluation should be performed. J Korean Ophthalmol Soc 2015;56(6):815-822

Clinical Outcome of Radiation Therapy for Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphomas

role of radiation therapy in localized (Ann Arbor stage I/II) NGNO MALT lymphoma. Materials/Methods: We retrospectively reviewed 47 patients who were histologically diagnosed as NGNO MALT lymphoma with Ann Arbor stage I/II from Feb 1993 to Oct 2012. Primary sites were head and neck (n Z 19), gastrointestinal tract (n Z 8), lung (n Z 7), and others. Their median age was 53 years (range 15-83 years). Forty-one patients had relatively good performance status (Eastern Cooperative Oncology Group performance score 1). Twenty-nine patients received radiation therapy (RT) containing treatment (14 RT alone, 11 surgery + RT, 2 chemotherapy (CTx) + RT, 2 surgery + RT + CTx) with a median RT dose of 30.6 Gy (22.5 to 45 Gy). Other 18 patients did not receive RT (9 surgery alone, 5 surgery + CTx, 4 CTx alone). Results: Median follow-up time was 45.6 months (range 4.8-224.5 months). The estimated 5-year progression-free survival (PFS) and overall survival rates were 61% and 92%, respectively. Among the stage I/II patients, 29 patients who received RT all achieved complete remission (CR) and showed significantly higher 5-year PFS than who did not receive RT (82% vs 34%; p Z 0.003). Four patients among these 29 patients experienced out-field relapse, which were well salvaged by RT (3 patients) or CTx (1 patient), and there was no in-field relapse. Ten out of 18 patients (61%) who did not receive RT with stage I/II achieved CR, and 11 out of these 18 patients have relapsed while 4 patients progressed into diffuse large B-cell lymphoma. Three of them who showed high grade transformation died within 3 months since relapse. Conclusions: In localized NGNO MALT lymphoma, low dose RT resulted in excellent outcome and relapses out of RT field were also successfully salvaged by RT. Early use of RT should be considered in localized NGNO MALT lymphoma and should be applied as a preferred treatment modality in case of localized relapse. Author Disclosure: J. Lee: None. K. Kim: None. J. Cho: None. C. Suh: None.

Localized Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma Treated With Radiation Therapy: A Long-Term Outcome in 86 Patients With 104 Treated Eyes

To evaluate the natural history, behavior of progression, prognostic factors, and treatment-related adverse effects of primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (POAML). Eighty-six patients with histologically proven stage I POAML treated with radiation therapy at National Cancer Center Hospital, Tokyo between 1990 and 2010 were retrospectively reviewed. The median age was 56 years (range, 18-85 years). The median dose administered was 30 Gy (range, 30-46 Gy). Seventy-seven patients (90%) were treated by radiation therapy alone. The median follow-up duration was 9 years (range, 0.9-22 years). The 5- and 10-year overall survival (OS) rates were 97.6% and 93.5%, respectively, and no patients died of lymphoma. Patients with tumor sizes ≥4 cm showed a greater risk of contralateral relapse (P=.012). Six patients with contralateral relapse were seen and treated by radiation therapy alone, and all the lesions were controlled well, with follow-up times of 3 to 12 years. There was 1 case of local relapse after radiation therapy alone, and 3 cases of relapse occurred in a distant site. Cataracts developed in 36 of the 65 eyes treated without lens shielding and in 12 of the 39 patients with lens shielding (P=.037). The majority of patients with POAML showed behavior consistent with that of localized, indolent diseases. Thirty gray of local irradiation seems to be quite effective. The initial bilateral involvement and contralateral orbital relapses can be also controlled with radiation therapy alone. Lens shielding reduces the risk of cataract.

Orbital and ocular adnexal Mucosa-Associated Lymphoid Tissue (MALT) lymphomas: a single-center 10-year experience

Orbital and ocular andexal Mucosa-Associated Lymphoid Tissue Lymphoma (MALT) or ocular adnexal MALT lymphoma (OAML) is the most common of all eye non-Hodgkin lymphomas. Autoimmune inflammatory disorders and chronic infections are important etiological factors and CD5 and CD43 (sialophorin) tumor markers are significant negative prognostic factors. Disease signs and symptoms can occur a long time before diagnosis. Varieties of treatment options are available. The aim of this retrospective analysis was to compare the efficiency of different treatment options and to investigate disease outcome. Twenty OAML patients, diagnosed in the Clinic of Hematology, Clinical Centre of Serbia, between 2003 and 2013, were enrolled. In most cases, OAML developed in the eighth decade with greater incidence in the male population. Median age was 67.5 years. The median period between the appearance of local signs and symptoms and diagnosis was 7 months. The dominant sign at presentation was swelling of involved tissue (40%). The most common was orbital involvement (55%). All patients had localized disease. Observed laboratory parameters on presentation showed low disease activity. Sialophorin prognostic significance was not registered. Our patients were initially treated differently but there was no significant difference in progression-free survival (PFS) due to initial treatment option (p = 0.2957). Median PFS was 22 months (3-89), and 5-year PFS was 60%. Median overall survival (OS) was 43 months (1-105) and 5-year OS 95%. Eight patients (40%) relapsed and one patient died due to non-hematological complications. In our experience, most modern induction treatment options appear to result in the same, favorable outcome.

Primary ocular adnexal mucosa-associated lymphoid tissue lymphoma: A bibliographic review

Primary ocular adnexal mucosa-associated lymphoid tissue lymphoma: A bibliographic review

Risk Potentiality of Frontline Radiotherapy Associated Cataract in Primary Ocular Adnexal Mucosa-associated Lymphoid Tissue Lymphoma

PurposeTo elucidate risk potentiality of frontline radiotherapy associated cataracts in primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML).MethodsData from eight consecutive patients of 41 total OAML patients who had undergone cataract surgery after frontline radiotherapy were analyzed.ResultsThe median patient age was 46 years (range, 36 to 69 years). The median total radiation dose was 3,780 cGy (range, 3,060 to 4,500 cGy), and the mean duration from radiation irradiation to cataract surgery was 36.60 ± 8.93 months. Preoperative lens opacification was primarily at the posterior lens subcapsule, and best-corrected visual acuity (BCVA) was 0.43 ± 0.21. Patients underwent the phacoemulsification surgical procedure with posterior chamber intraocular lens insertion. The average BCVA improved to 0.90 ± 0.14 after cataract surgery. Two patients underwent posterior continuous curvilinear capsulorhexis, and one had posterior capsule rupture. For posterior capsule opacification (PCO), three patients received Nd:YAG laser posterior capsulotomy after the initial surgery, and one patient is currently under consideration for laser posterior capsulotomy.ConclusionsRadiotherapy increased posterior subcapsule opacification at a relatively young age in primary OAML. Phacoemulsification was a manageable procedure without severe complications, and final visual outcomes were good. However, because after-cataracts progressed earlier than did senile cataracts, close follow-up should be considered for PCO management.