<b>Objective:b> This study aimed to see how different initial treatment regimens affected the long-term prognosis of patients with extranodal marginal zone mucosa-associated lymphoid tissue lymphoma confining to the ocular adnexal (OAML) . <b>Methods:b> Between April 2008 and April 2019, 109 patients with initial mucosa-associated lymphoid tissue confining to ocular adnexal were evaluated and followed-up, and the prognosis of various initial treatment regimens were examined. <b>Results:b> A total of 36 patients underwent complete surgical resection of the lesions, and 73 patients had residual lesions after surgery, of which 37 patients chose watchful waiting, and 36 patients chose treatment. The treatment regimen included local radiotherapy and systemic treatment (chemotherapy, immunochemotherapy, the combination of radiotherapy and chemotherapy, etc.) , and no serious toxic and side effects were observed in patients receiving systemic treatment. The median follow-up time was 61 (10-142) months. The 5-year and 10-year progression-free survival (PFS) of monocular involvement patients were 78.2% and 76.0% . The 5-year and 10-year PFS rates of patients with binocular involvement were 64.4% and 23.5%. There was significant diference in PFS between patients with monocular and binocular involvement (P=0.010) . Patients who received additional treatment had higher PFS than those patients in the watchful waiting group (P=0.046) . The 5-year PFS was 71.4% and 90.1% among patients in the watchful waiting group and those who received additional treatment, whereas the 10-year PFS was 63.5% and 75.1% , respectively. Patients with OAML were still a risk of disease progression after 5 years. <b>Conclusions:b> Patients with binocular involvement OAML at the start of the disease had a poor prognosis, but treatment could reduce the risk of recurrence/progression. Systemic therapy is one of the first-line treatment options for patients with OAML, who require long-term monitoring.
Eye Neoplasms/radiotherapy* ; Humans ; Lymphoid Tissue/pathology* ; Lymphoma, B-Cell, Marginal Zone/therapy* ; Prognosis ; Retrospective Studies ; Treatment Outcome

BACKGROUND/AIMS: The eradication of Helicobacter pylori (H. pylori) is an effective treatment in gastric mucosa-associated lymphoid tissue (MALT) lymphoma associated with H. pylori infection. However, the treatment strategy in gastric MALT lymphoma patients who are H. pylori-negative or unresponsive to H. pylori eradication therapy remains controversial. In this study, we investigated the clinical efficacy of treatments other than H. pylori eradication therapy in these groups of patients. METHODS: This was a retrospective single-center study based on the medical records of patients diagnosed with gastric MALT lymphoma at Yeungnam University Medical Center between January 2005 and December 2016. Patients were treated with H. pylori eradication therapy, chemotherapy, or radiotherapy according to their H. pylori infection status and stage of gastric MALT lymphoma. RESULTS: Of the 68 eligible patients, 50 were enrolled in the study. Of the 42 patients with H. pylori-positive gastric MALT lymphoma, 36 (81.7%) were treated with H. pylori eradication therapy as primary treatment and 25 (69.4%) achieved a complete response (CR). Patients without a CR after H. pylori eradication therapy (n=11, 30.6%) received radiotherapy as a secondary treatment. Two patients with H. pylori-positive gastric MALT lymphoma and eight with H. pylori-negative gastric MALT lymphoma received radiotherapy as the primary treatment. CR was achieved in all 21 patients treated with radiotherapy as primary or secondary treatment. The 5-year progression-free survival rate after radiotherapy was 92.9%. CONCLUSIONS: Radiotherapy may be a worthwhile treatment option in patients with H. pylori-negative MALT lymphoma or H. pylori-positive MALT lymphoma that is not responsive to H. pylori eradication therapy.
Academic Medical Centers ; Disease-Free Survival ; Drug Therapy ; Helicobacter pylori* ; Helicobacter* ; Humans ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Medical Records ; Radiotherapy* ; Retrospective Studies ; Stomach Neoplasms ; Treatment Outcome*

The stomach is the most common primary site of an extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type, which is characterized by an indolent clinical course. A diagnosis of gastric MALT lymphoma requires an endoscopic biopsy that should be confirmed by an experienced pathologist. Gastric MALT lymphoma shows a variable endoscopic appearance, including erosion, erythema, discoloration, atrophy, ulcer, and subepithelial lesion. The distribution is often multifocal. Therefore, clinical suspicion and multiple biopsies are essential for an accurate diagnosis. Gastric MALT lymphoma is almost invariably associated with a Helicobacter pylori (H. pylori) infection. H. pylori eradication therapy is the mainstay of treatment, which must be delivered to all patients regardless of the H. pylori infection status or stage. For patients who have failed to achieve remission following eradication therapy, radiotherapy or chemotherapy can be considered. Radiotherapy is an effective treatment modality for a localized stage and shows excellent outcomes. In the presence of disseminated or advanced disease, chemotherapy and/or immunotherapy with the anti-CD20 monoclonal antibody, rituximab, can be applied. Treatment should be individualized according to the stage and symptoms, as well as the patients' preference. Given that the clinical course of gastric MALT lymphoma is usually indolent, watchful waiting may be an adequate strategy in selected cases where scheduled follow-up is guaranteed.
Atrophy ; Biopsy ; Diagnosis ; Drug Therapy ; Erythema ; Follow-Up Studies ; Helicobacter pylori ; Humans ; Immunotherapy ; Lymphoma, B-Cell, Marginal Zone ; Radiotherapy ; Rituximab ; Stomach ; Ulcer ; Watchful Waiting

PURPOSE: To retrospectively analyze dosimetric parameters of volumetric-modulated arc therapy (VMAT) and three-dimensional conformal radiotherapy (3D-CRT) delivered to extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue in the stomach (gastric MALT lymphoma) to find out advantages of VMAT and conditions for definite benefits of VMAT. MATERIALS AND METHODS: Fifty patients with stage I-II gastric MALT lymphoma received VMAT (n = 14) or 3D-CRT (n = 36) between December 2005 and April 2018. Twenty-seven patients were categorized according to whether the planning target volume (PTV) overlaps kidney(s). Dosimetric parameters were analyzed by dose-volume histogram. RESULTS: Radiation dose to the liver was definitely lower with VMAT in terms of mean dose (p = 0.026) and V15 (p = 0.008). The V15 of the left kidney was lower with VMAT (p = 0.065). For those with PTV overlapping kidney(s), the left kidney V15 was significantly lower with VMAT. Furthermore, the closer the distance between the PTV and kidneys, the less the left kidney V15 with VMAT (p = 0.037). Delineation of kidney(s) by integrating all respiratory phases had no additional benefit. CONCLUSIONS: VMAT significantly increased monitor units, reduced treatment time and radiation dose to the liver and kidneys. The benefit of VMAT was definite in reducing the left kidney V15, especially in geometrically challenging conditions of overlap or close separation between PTV and kidney(s).
Humans ; Kidney ; Liver ; Lymphoid Tissue ; Lymphoma, B-Cell, Marginal Zone ; Organs at Risk ; Radiotherapy, Conformal ; Radiotherapy, Intensity-Modulated ; Retrospective Studies ; Stomach

PURPOSE: The purpose of this study was to evaluate the clinical features and the long-term outcomes of primary conjunctival marginal zone B-cell lymphoma (MZBCL) patients who were treated with radiation therapy (RT). MATERIALS AND METHODS: Retrospective data of 79 patients with 121 primary conjunctival MZBCL lesions were collected from January 1, 2001 till June 30, 2014. All lesions were treated by local RT (26 Gy) with patient-specific customized lens-shielding device. RESULTS: The current Korean patients' cohort showed younger median age at diagnosis (38 years), great female preponderance (78.5%) and more frequent bilateral involvement (53.2%) than the previous studies. Following 26 Gy's RT, excellent clinical outcomes were achieved: 5-year rates of overall survival, local relapse-free survival, and contralateral relapse-free survival were 100%, 98.1%, and 91.5%, respectively. Two patients (2.5%) developed local relapse and five (6.3%) developed relapse at initially uninvolved contralateral conjunctiva with median interval of 52.9 months, and late adverse events of grade 2 and 3 occurred in seven (8.8%) and two (2.5%) patients, respectively. CONCLUSION: 26 Gy's RT was highly effective and safe, with the use of lens-shielding device, in treating patients with primary conjunctival MZBCL.
Cohort Studies ; Conjunctiva ; Diagnosis ; Female ; Humans ; Lymphoma, B-Cell, Marginal Zone* ; Radiotherapy ; Recurrence ; Retrospective Studies

Lymphoproliferative disorder in a posttransplant setting has emerged as a difficult problem in kidney transplantation (KT). Lymphoma involving adnexa of the eye has rarely been reported due to scarcity of lymphoreticular tissue in the ocular area. This report presents a case of a 37-year-old KT recipient who was diagnosed with conjunctival mucosa-associated lymphoid tissue lymphoma with a chief complaint of seeing black spots. Unlike other post-transplant lymphoproliferative diseases associated with the Epstein-Barr virus (EBV) reactivation via immunosuppression, the lesion was not related to the virus. The patient received radiotherapy with concomitant conversion from the tacrolimus to the sirolimus. Overall, the results presented herein indicate lymphoma may be an important differential diagnosis when KT recipients complain of ocular discomfort.
Adult ; Diagnosis, Differential ; Herpesvirus 4, Human ; Humans ; Immunosuppression ; Kidney Transplantation ; Kidney* ; Lymphoid Tissue* ; Lymphoma* ; Lymphoma, B-Cell, Marginal Zone ; Lymphoproliferative Disorders ; Radiotherapy ; Sirolimus ; Tacrolimus ; Transplant Recipients*

BACKGROUND: Primary 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. METHODS: We 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. RESULTS: Among 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). CONCLUSION: We revealed that POML has good response to treatment, especially radiotherapy, with excellent long-term outcome.
Disease-Free Survival ; Female ; Humans ; Lacrimal Apparatus ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Orbit ; Radiotherapy ; Retrospective Studies ; Thailand*

PURPOSE: To determine the optimal radiotherapy technique for gastric mucosa-associated lymphoid tissue lymphoma (MALToma), we compared the dosimetric parameters and the risk of solid secondary cancer from scattered doses among anterior-posterior/posterior-anterior parallel-opposed fields (AP/PA), anterior, posterior, right, and left lateral fields (4_field), 3-dimensional conformal radiotherapy (3D-CRT) using noncoplanar beams, and intensity-modulated radiotherapy composed of 7 coplanar beams (IMRT_co) and 7 coplanar and noncoplanar beams (IMRT_non). MATERIALS AND METHODS: We retrospectively generated 5 planning techniques for 5 patients with gastric MALToma. Homogeneity index (HI), conformity index (CI), and mean doses of the kidney and liver were calculated from the dose-volume histograms. Applied the Biological Effects of Ionizing Radiation VII report to scattered doses, the lifetime attributable risk (LAR) was calculated to estimate the risk of solid secondary cancer. RESULTS: The best value of CI was obtained with IMRT, although the HI varied among patients. The mean kidney dose was the highest with AP/PA, followed by 4_field, 3D-CRT, IMRT_co, and IMRT_non. On the other hand, the mean liver dose was the highest with 4_field and the lowest with AP/PA. Compared with 4_field, the LAR for 3D-CRT decreased except the lungs, and the LAR for IMRT_co and IMRT_non increased except the lungs. However, the absolute differences were much lower than <1%. CONCLUSION: Tailored RT techniques seem to be beneficial because it could achieve adjacent organ sparing with very small and clinically irrelevant increase of secondary solid cancer risk compared to the conventional techniques.
Hand ; Humans ; Kidney ; Liver ; Lung ; Lymphoma, B-Cell, Marginal Zone* ; Planning Techniques ; Radiation, Ionizing ; Radiotherapy* ; Radiotherapy, Conformal ; Radiotherapy, Intensity-Modulated ; Retrospective Studies ; Risk Assessment* ; Stomach

Approximately 90% of gastric mucosa-associated lymphoid tissue (MALT) lymphomas are associated with Helicobacter pylori infection and 10% of gastric MALT lymphomas occur independent of H. pylori infection. The gene translocation t(11;18)(q21;q21) is frequently observed in cases presenting with H. pylori-negative gastric MALT lymphomas. H. pylori-negative gastric MALT lymphomas do not differ from H. pylori-positive gastric MALT lymphomas in terms of clinical features and endoscopic findings and show similar prognosis. H. pylori eradication therapy could be considered as first-line treatment for gastric MALT lymphomas regardless of their H. pylori infection status. In cases with H. pylori-negative gastric MALT lymphomas, the rate of response to eradication therapy varies from 0 to 83%. If gastric MALT lymphoma persists for more than a year, secondary treatment with radiotherapy, chemotherapy, and/or surgery should be considered. Of note, the comparative outcome is excellent.
B-Lymphocytes ; Drug Therapy ; Helicobacter pylori ; Helicobacter* ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Prognosis ; Radiotherapy

PURPOSE: We retrospectively reviewed the results of radiotherapy for localized ocular adnexal MALT lymphoma (OAML) to investigate the risk factors of cataract. METHODS: Sixty-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. RESULTS: Median 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). CONCLUSIONS: RT 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.
Cataract* ; Follow-Up Studies ; Humans ; Lymphoid Tissue* ; Lymphoma* ; Lymphoma, B-Cell, Marginal Zone ; Orbit ; Proportional Hazards Models ; Radiotherapy* ; Recurrence ; Retrospective Studies ; Risk Factors ; Seoul ; Treatment Outcome*