Primary Ocular Adnexal MALT Lymphoma Research Articles

824O Treatment and survival in patients with localized primary ocular adnexal MALT lymphoma: A large bicentric cohort study

824O Treatment and survival in patients with localized primary ocular adnexal MALT lymphoma: A large bicentric cohort study

Treatment-associated outcomes of patients with primary ocular adnexal MALT lymphoma after accurate diagnosis.

Differentiation between primary ocular adnexal mucosa-associated lymphoid tissue (POA-MALT) lymphoma and reactive lymphoid hyperplasias sometimes may be difficult. We have examined the treatment-associated mortality of POA-MALT lymphoma after confirmed diagnosis and evaluated their proper treatments. From 1991 through 2016, cases of POA-MALT lymphoma were retrospectively analyzed based on their pathological and molecular/immunological diagnoses. A total of 78 cases with POA-MALT lymphoma with a median age of 66years were analyzed over median/mean observations of 6.4/7.1years. Forty-four patients (56%) were diagnosed with IgH gene clonality and 10 patients (13%) were diagnosed with flow cytometric analysis in addition to the pathological decision. The rest (24 patients, 31%) were diagnosed employing pathological decisions of hemato-pathologists and clinical decisions. All patients, except cases of watchful waiting, achieved complete remission. After initial treatment, 68 patients (87%) presented disease-free during the observation period. As treatment, a radiotherapy-based strategy was followed with 15 patients (19%, group A). Immuno-chemotherapy was administered to 24 patients (31%, B). Surgical extraction only was selected for 36 patients (46%, C). Watchful waiting was selected with three patients (4%). Recurrence after the initial treatment was found in one patient (7%) out of A, in three patients (13%) out of B, and in six patients (17%) out of C, respectively. Progression-free survivals at 5 and 10years were 100 and 100% in A, 95 and 75% in B, and 88 and 81% in C, respectively. The recurrence rates between the patients who were diagnosed with only pathological decision (n = 24) and the patients who were diagnosed with molecular and immunological procedures (n = 54) did not show any statistical differences. Our results indicate that radiotherapy-based treatment strategies for patients with POA-MALT lymphoma show a low rate of recurrence and may improve their prognosis even after the accurate diagnosis. However, contamination of the cases with reactive (polyclonal) lymphoid hyperplasia into those with MALT lymphoma should be carefully removed to avoid unnecessary treatment for malignancies that do not exist.

Clinical presentation and treatment outcomes of primary ocular adnexal MALT lymphoma in Thailand.

BackgroundPrimary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (POML) is the most common subtype of lymphoma involving the eyes in Thailand. We sought to assess the characteristics and treatment outcomes of patients with POML in Thailand.MethodsWe retrospectively reviewed patient data and included patients diagnosed with POML between January 2004 and December 2016 at Chiang Mai University Hospital and King Chulalongkorn Memorial Hospital, Thailand. We collected and analyzed patients' clinical characteristics and treatment outcomes.ResultsAmong 146 patients with lymphoma involving the eyes, 121 (82%) were diagnosed with POML. Sixty-four (52.9%) were women with median age 58 (range, 22–86) years. The most common presenting symptom was orbital mass (71.1%). Common sites of origin were the orbit (46.3%) and lacrimal gland (34.7%). At presentation, 22.3% of patients had bilateral eye involvement. About half of patients had stage I disease (N=59, 56.2%) and 20% had stage IV. Most patients (73.3%) had a low-risk International Prognostic Index. Radiotherapy was the main treatment for patients with limited-stage disease (66.7% in stage I and 56.5% in stage II). The overall response rate was 100% with complete response rates 80%, 77.3%, and 64.7% for stages I, II, and IV, respectively. Five-year progression-free survival (PFS) and overall survival were 66.1% and 94.0%, respectively. For patients with limited-stage disease, radiotherapy significantly improved PFS compared with treatment not involving radiotherapy (5-year PFS 89.9% vs. 37.3%, P=0.01).ConclusionWe revealed that POML has good response to treatment, especially radiotherapy, with excellent long-term outcome.

Treatment Outcome of Primary Ocular Adnexal MALT Lymphoma by Radiation Therapy: Prognostic Effect of the AJCC Tumor-Node-Metastasis Clinical Staging System for Ocular Adnexal MALT Lymphoma

Treatment Outcome of Primary Ocular Adnexal MALT Lymphoma by Radiation Therapy: Prognostic Effect of the AJCC Tumor-Node-Metastasis Clinical Staging System for Ocular Adnexal MALT Lymphoma

Long-term Outcome and Patterns of Failure in Primary Ocular Adnexal MALT Lymphoma Treated with Radiotherapy

Long-term Outcome and Patterns of Failure in Primary Ocular Adnexal MALT Lymphoma Treated with Radiotherapy

The Results of Radiotherapy for Primary Ocular Adnexal Malt Lymphoma

The Results of Radiotherapy for Primary Ocular Adnexal Malt Lymphoma

Prognosis for Patients in a Korean Population with Ocular Adnexal Lymphoproliferative Lesions

To analyze the clinical features, treatment outcomes, and prognostic factors associated with lymphoproliferative lesions of the ocular adnexa in a Korean population. Data from 69 patients treated for adnexal lymphoproliferative disease in the 12-year period from 1991 to 2002 were retrospectively evaluated. The 69 patients had a median age of 46 years (range, 15-73 years); 60 of these patients were diagnosed with extranodal marginal zone B cell lymphomas (MALT lymphomas) and had 10 year cause specific survival and relapse free survival rates of 95.6% and 82.6%, respectively. Of 6 patients (8.7%) with concurrent systemic lymphoma, including 4 diagnosed with stage IV disease, 3 died from lymphoma. Only one patient with a primary ocular adnexal MALT lymphoma developed systemic lymphoma, which was treated with surgical resection. Local recurrence either at the primary site or in the fellow eye occurred in 11.6% of patients at a median follow-up time of 102 months (range, 79-132 months), and was controlled using repeat irradiation in all cases. Statistical analysis showed the presence of concurrent systemic lymphoma, bilateral disease, and an advanced stage at diagnosis, were linked to lymphoma-related death (Log-rank test, p < 0.05) and systemic progression (Fisher's exact test, p < 0.05), and that the tumor location was not a prognostic factor for lymphoma-related death or relapse at any site. Ocular adnexal lymphoproliferative disease in Koreans occurred at a relatively young age, and was mostly orbitally located and of the MALT subtype, which is highly localized and rarely associated with extraorbital relapse. Primary or secondary status, stage at presentation, and bilaterality were found to be prognostic factors.

Fluorescence in situhybridization (FISH) analysis of primary ocular adnexal MALT lymphoma

BackgroundIt remains unknown whether primary ocular adnexal extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a homogeneous entity, as there are few reports of the results of cytogenetic or molecular analyses of these tumors.MethodsWe performed interphase fluorescence in situ hybridization (FISH) analysis to detect translocations and aneuploidy in 34 cases of primary ocular adnexal MALT lymphoma, and reviewed the histopathological findings. Correlations between the results of FISH analysis, the histopathological features and the clinical data were also analyzed.ResultsAmong the 34 cases, FISH analysis revealed t(14;18)(q32;q21) in one case, trisomy 3 in 21 cases (62%), and trisomy 18 in 16 cases (47%). The cases with trisomy 18 had significantly more prominent lymphoepithelial lesions (LELs) and less nodularity in the tumors. In regard to the clinical correlations, tumors with trisomy 18 were observed predominantly in females and younger patients; also, in the majority of the cases, the tumor was of conjunctival origin. All the cases with recurrence showed trisomy 18 in the tumor.ConclusionPrimary ocular adnexal MALT lymphoma is a significantly heterogeneous entity. Cases with trisomy 18 may have unique clinicopathological features.

Primary ocular adnexal MALT lymphoma (POAML): A long-term follow up study of 114 patients (pts)

7593 Background: Although POAML is a recently recognized distinct entity, its natural history, prognostic factors, behavior of progression and death, and standard initial management have not been fully elucidated. Methods: 114 pts with a histologically verified POAML treated at our institution between 1970 and 2003 were retrospectively analyzed. Results: With a median follow-up duration of 5.7 years (0.6–34.0), estimated overall survival (OS) rate and progression-free survival (PFS) rate at 10 years was 89% and 57%, respectively. Older pts (&gt;60) showed significantly worse OS (p=0.002). However, other clinical factors did not affect significantly OS or PFS. 13 (11%) pts died, but only 3 (3%) due to progressive lymphoma. 31 (27%) pts progressed, 7(6%) at systemic diseases, 8 (7%) at contra lateral sites, and 16 (14%) at the same sites, and only 2 (2%) transformed high-grade lymphoma. All 8 pts who progressed at contra lateral sites were limited to those who had involved initially in the orbit (p=0.036) and their time to progression was significantly longer (p=0.039). Pts who received initially radiation-containing therapy were superior in PFS but not OS than those initially treated with other modalities (p=0.016 and 0.091, respectively). Moreover, when we compared the outcomes of no initial therapy cohort and immediate therapy cohort, there was no significant difference in OS and PFS (p=0.499 and 0.073, respectively). Conclusions: The majority of pts with POAML showed the behaviors of very indolent diseases, and only age was significant prognostic factor. Our preliminary observation suggesting that no initial therapy is an acceptable approach for selected pts (Ann Oncol 2006;17:135–40) was further confirmed in this study for all cohorts. Considering the possible heterogeneity of POAML among initial sites suggested by the present study and the genetic heterogeneity revealed by our previous study (Blood 2005;106:289a), further investigations on POAML are warranted. No significant financial relationships to disclose.

A Distinct Entity of Ocular Adnexal MALT Lymphoma with Trisomy 18.

Background: It remains unknown whether primary ocular adnexal MALT lymphoma is a homogenous disease, because there have been few reports on cytogenetic or molecular analysis of this disease.Patients and Methods: In 34 cases of ocular adnexal MALT lymphoma, we performed interphase fluorescence in situ hybridization (FISH) analysis to detect IgH/MALT1 and API2/MALT1 fusion genes. Aneuploidy of chromosome 3, 7, 12 and 18 was also identified using corresponding centromere probes. We defined trisomy when three centromeres were recognized in FISH analysis. Histopathologic features were reviewed and categorized according to the degree of plasma cell differentiation, monocytoid B cell feature, nodularity, abundance of reactive germinal centers, multikaryocytic cells, contaminated T cells, Duthcer bodies, and lymphoepithelial lesions (LELs). Correlations among FISH analysis, histopathologic features, and clinical data were analyzed.Results: FISH analysis showed 1 (3%) IgH/MALT1 fusion, 21 (62%) trisomy 3, 16 (47%) trisomy 18, and 3(9%) trisomy 7 cases. No cases showed API2/MALT1 fusion or trisomy 12. Existence of these cytogenetic change did not influence the degree of morphological features significantly, except for trisomy 18; Cases with trisomy 18 had significantly more abundant LEL cells, monocytoid B cells and residual reactive germinal centers, but less nodules, contaminated plasma cells and large cells. Cases with trisomy 18 showed distinct features of female dominance, younger age, and included more cases originated from conjunctiva. In total, five (15%) of 34 patients relapsed between 21 and 103 months, and all of them were found to have trisomy 18.Conclusions: Aneuploidy is found in a ceratin subset of ocular adnexal MALT lymphoma by FISH analysis. Cases with trisomy18 may make a distinct clinicopathlogic entity.

Ocular adnexal lymphoma—comparison of MALT lymphoma with other histological types

AIMSTo correlate histological features of ocular adnexal lymphoma using the revised European American lymphoma classification (REAL), with stage of disease at presentation, treatment modalities, and patient outcome. MALT lymphoma defines...