Clinical features and treatment outcomes of primary ocular adnexal mucosa-associated lymphoid tissue lymphoma: a single center retrospective analysis of 64 patients in China

AIM: To investigate the clinical features, treatment and prognosis of primary ocular adnexal mucosa-associated lymphoid tissue lymphoma(POAML).METHODS: A retrospective analysis was performed on 64 patients with POAML who were admitted to the First Affiliated Hospital of Zhengzhou University from January 2006 to December 2018.RESULTS: With a median follow-up of 61 mo(range, 2-156 mo), estimated overall survival(OS) rate and progressionfree survival(PFS) rate at 10 y reached 94.5% and 61.5%, respectively. Median OS time and PFS time were not reached. During this period, only 3 patients died, but none of them died directly due to disease progression. One patient(1.6%) developed transformation to diffuse large B-cell lymphoma(DLBCL). Of the 56 patients achieved complete remission after first-line treatment, 5(8.9%) developed local and/or systemic relapse eventually. Patients ≥60 y had significantly shorter PFS than younger patients(P=0.01). For patients with early stages(Ann Arbor stage I and stage II), univariate analysis confirmed that radiotherapydose lower than 32 Gy were independently associated with shorter PFS(P=0.04). Other factors including gender, bone marrow involvement, the initial location of the disease, and the laterality were not associated with PFS.CONCLUSION: The data from our center indicate that POAML has a slow clinical progression and has an excellent clinical outcome. Patients with POAML harbor a continual risk of relaps and transformation to aggressive subtype of lymphoma.

Guiding function of positron emission tomographycomputed tomography examination in the diagnosis and treatment of ocular adnexal mucosa associated lymphoid tissue lymphoma

AIM:To explore the role of positron emission tomographycomputed tomography(PET-CT)examination in the diagnosis and treatment of ocular adnexal mucosa associated lymphoid tissue lymphoma(OAML).METHODS:The general clinical data,postoperative PET-CT results,treatment regimens,and the prognosis of 21 histopathologically confirmed OAML patients between October 2017 and September 2021 were collected.Among the 21 patients,five patients underwent surgical treatment alone,13 patients underwent surgical treatment combined with radiotherapy,and three patients underwent surgical treatment combined with chemotherapy.RESULTS:The follow-up period ranged from 8 to 79mo,with four cases of recurrence and no deaths.Through PETCT examination,two patients exhibited both local ocular metabolic elevation and systemic metastasis,and one of these patients had cervical lymph node metastasis,while the other had submandibular and parotid gland metastasis.Nine patients showed only local ocular metabolic elevation,while 10 patients had no abnormal metabolic activity locally.CONCLUSION:PET-CT examination plays a crucial role in detecting residual lesions and recurrence following tumor resection,aiding in precise disease staging,and facilitating the development of personalized treatment plans,ultimately improving patient prognosis.

Nomogram model predicting the overall survival for patients with primary gastric mucosa-associated lymphoid tissue lymphoma

BACKGROUND Increasingly extranodal marginal B-cell lymphoma of mucosa-associated lymphoid tissue,known as mucosa-associated lymphoid tissue(MALT)lymphoma,is a type of non-Hodgkin’s lymphoma.The prognosis of primary gastric MALT(GML)patients can be affected by many factors.Clinical risk factors,including age,type of therapy,sex,stage and family hematologic malignancy history,also have significant effects on the development of the disease.The available data are mainly focused on epidemiology;in contrast,few studies have investigated the prognostic variables for overall survival(OS)in patients with primary GML.Based on the realities above,we searched a large amount of data on patients diagnosed with primary GML in the Surveillance,Epidemiology and End Results(SEER)database.The aim was to develop and verify a survival nomogram model that can predict the overall survival prognosis of primary GML by com-bining prognostic and determinant variables.AIM To create an effective survival nomogram for patients with primary gastric GML.METHODS All data of patients with primary GML from 2004 to 2015 were collected from the SEER database.The primary endpoint was OS.Based on the LASSO and COX regression,we created and further verified the accuracy and effectiveness of the survival nomogram model by the concordance index(C-index),calibration curve and timedependent receiver operating characteristic(td-ROC)curves.RESULTS A total of 2604 patients diagnosed with primary GML were selected for this study.A total of 1823 and 781 people were randomly distributed into the training and testing sets at a ratio of 7:3.The median follow-up of all patients was 71 mo,and the 3-and 5-year OS rates were 87.2%and 79.8%,respectively.Age,sex,race,Ann Arbor stage and radiation were independent risk factors for OS of primary GML(all P<0.05).The C-index values of the nomogram were 0.751(95%CI:0.729-0.773)and 0.718(95%CI:0.680-0.757)in the training and testing cohorts,respectively,showing the good discrimination ability of the nomogram model.Td-ROC curves and calibration plots also indicated satisfactory predictive power and good agreement of the model.Overall,the nomogram shows favorable performance in discriminating and predicting the OS of patients with primary GML.CONCLUSION A nomogram was developed and validated to have good survival predictive performance based on five clinical independent risk factors for OS for patients with primary GML.Nomograms are a low-cost and convenient clinical tool in assessing individualized prognosis and treatment for patients with primary GML.

CT and MR imaging findings of ocular adnexal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease: multi-institutional case series

AIM:To report CT and MR imaging findings of ocular adnexal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease(IgG4-MALT lymphoma),a rare but clinically important complication of ocular adnexal IgG4-related disease.METHODS:We retrospectively reviewed all cases of histologically confirmed ocular adnexal IgG4-related disease at three ter tiary and one secondary referral centers,between February 2003 and December 2016.Seven cases of histopathologically diagnosed IgG4-MALT lymphoma were identified.CT and MR images were analyzed by consensus of two experienced head and neck radiologists.RESULTS:Lacrimal glands were the main site of involvement in all seven patients.The lesions typically showed well-demarcated margins,iso-to hyperattenuation on precontrast CT,T2 hypo-to isointensity,T1 isointensity,and homogenous internal architecture with homogenous enhancement pattern.Lesions were mostly hyperdense and isointense to normal extraocular muscles on postcontrast CT and MR images,respectively.CONCLUSION:Unlike in typical ocular adnexal IgG4-related disease,T2 isointensity and hyperattenuation on precontrast CT images were noted in some IgG4-MALT lymphoma cases.Although the findings may be nonspecific,the possibility of accompanying MALT lymphoma may need to be considered,when ocular adnexal lesions in patients clinically suspected of having IgG4-related disease are refractory to glucocorticoids and show T2 isointensity and hyperattenuation on precontrast CT for the optimal management of the patients.However,this is a case series of a very rare complication of ocular adnexal IgG4-related disease,and thus caution is warranted to generalize the conclusion.

Laterally spreading tumor-like primary rectal mucosa-associated lymphoid tissue lymphoma:A case report

BACKGROUND Colorectal mucosa-associated lymphoid tissue(MALT)lymphoma is a rare disease,and only a few cases have been reported to date.It has no specific clinical presentations and shows various endoscopic appearances.There is no uniform consensus on its treatment.With the advancement of endoscopic technology,endoscopic treatment has achieved better results in individual case reports of early-stage patients.CASE SUMMARY We report a case of rectal MALT in a 57-year-old Chinese man with no symptoms who received endoscopy as part of a routine physical examination,which incidentally found a 25 mm×20 mm,laterally spreading tumor(LST)-like elevated lesion in the rectum.Therefore,he was referred to our hospital for further endoscopic treatment.Complete and curable removal of the tumor was performed by endoscopic submucosal dissection.We observed enlarged and dilated branch-like vessels similar to those of gastric MALT lymphoma on magnifying endoscopy with narrow-band imaging.And immunopathological staining showed hyperplastic capillaries in the mucosa.Histopathological findings revealed diffusely hyperplastic lymphoid tissue in the lamina propria,with a visible lymphoid follicle structure surrounded by a large number of diffusely infiltrated lymphoid cells that had a relatively simple morphology and clear cytoplasm.In addition,immunohistochemical analysis suggested strongly positive expression for CD20 and Bcl-2.Gene rearrangement results showed positivity for IGH-A,IGH-C,IGK-B,and IGL.Taking all the above findings together,we arrived at a diagnosis of extranodal marginal zone B-cell lymphoma of MALT lymphoma. Positron emission tomography-computed tomographyexamination showed no other lesions involved. The patient will be followed byperiodic endoscopic observation.CONCLUSIONIn conclusion, we report a case of rectal MALT with an LST-like appearancetreated by endoscopic submucosal dissection. Further studies will be needed toexplore the clinical behavior, endoscopic appearance, and treatment of rectalMALT.

Marginal zone lymphoma with severe rashes: A case report

BACKGROUND Marginal zone lymphoma(MZL)is an indolent subtype of non-Hodgkin lymphoma(NHL),which is rare clinically with severe rashes as the initial symptom.CASE SUMMARY This study reports a case of MZL with generalized skin rashes accompanied by pruritus and purulent discharge.First-line treatment with rituximab combined with zanubrutinib had poor effects.However,after switching to obinutuzumab combined with zanubrutinib,the case was alleviated,and the rashes disappeared.CONCLUSION For patients with advanced stage MZL not benefiting from type I anti-CD20 monoclonal antibody(mAb)combination therapy,switching to a type II anti-CD20 mAb combination regimen may be considered.This approach may provide a new perspective in the treatment of MZL.

Primary gastric non-Hodgkin lymphomas:Recent advances regarding disease pathogenesis and treatment

Primary gastric lymphomas(PGLs)are distinct lymphoproliferative neoplasms described as heterogeneous entities clinically and molecularly.Their main histological types are diffuse large B-cell lymphoma(DLBCL)or mucosaassociated lymphoma tissue.PGL has been one of the main fields of clinical research of our group in recent years.Although gastric DLBCLs are frequent,sufficient data to guide optimal care are scarce.Until today,a multidisciplinary approach has been applied,including chemotherapy,surgery,radiotherapy or a combination of these treatments.In this minireview article,we provide an overview of the clinical manifestations,diagnosis and staging of these diseases,along with their molecular pathogenesis and the most important related clinical published series.We then discuss the scientific gaps,perils and pitfalls that exist regarding the aforementioned studies,in parallel with the unmet need for future research and comment on the proper methodology for such retrospective studies.Aiming to fill this gap,we retrospectively evaluated the trends in clinical presentation,management and outcome among 165 patients with DLBCL PGL who were seen in our institutions in 1980-2014.The study cohort was divided into two subgroups,comparing the main 2 therapeutic options[cyclophosphamide doxorubicin vincristine prednisone(CHOP)vs rituximab-CHOP(R-CHOP)].A better outcome with immunochemotherapy(R-CHOP)was observed.In the next 2 mo,we will present the update of our study with the same basic conclusion.

原发性眼附属器非霍奇金淋巴瘤的特征

目的:探讨原发于眼附属器非霍奇金淋巴瘤的临床表现与病理特征。方法:对18例住院手术并经病理学检查确认为原发于眼附属器非霍奇金淋巴瘤患者的临床资料进行回顾性分析,对该病发生部位、临床及影像学表现、病理学特征进行总结。结果:全部患者均表现为眼部占位性病变,其中发生于眼眶者12例(含泪腺3例),占66.7%;发生于结膜者3例,占16.7%;发生于下睑者3例,占16.7%。全部病例中,8例患者被临床和影像学检查误诊为"炎性假瘤"。8例被诊为眼部占位性病变性质待查。经病理学检查,16例(88.9%)诊断为黏膜相关淋巴组织型的结外边缘带B细胞淋巴瘤,2例诊断为NK/T细胞性非霍奇金淋巴瘤。结论:原发于眼附属器的非霍奇金淋巴瘤主要表现为无痛性肿块,多发生于眼眶,临床和影像学确诊难度大,大多数糖皮质激素治疗有效,容易被误诊为"炎性假瘤"。黏膜相关淋巴组织B细胞淋巴瘤是最常见的病理类型。

眼附属器黏膜相关淋巴组织淋巴瘤形态及免疫表型研究

目的:探讨眼附属器黏膜相关淋巴组织淋巴瘤(mucosaassociated lymphoid tissue,MALT)形态及免疫表型特点。方法:采用光镜观察和免疫组织化学分析MALT的形态学、细胞学、免疫表型的特点。结果:眼附属器MALT患者29例33眼中,Ⅰ期23例24眼,Ⅱ期6例9眼。29例33眼均以小淋巴细胞样瘤细胞为主,瘤细胞弥漫分布,体积小,大小较均一,细胞核略呈圆形,染色深,细胞质少,染色淡;29例CD20及CD79a均(+),CD5(+)2例,CD43(+)3例;所有患者CD45RO、Cyclin D1、CD10、CD23均为(-)。结论:眼附属器MALT主要以小淋巴细胞样瘤细胞浸润为主,散在分布中心细胞样细胞及单核样细胞,部分可见浆细胞及Russel、Dutcher小体。眼附属器MALT典型的免疫表型CD20(+)、CD10(-)、CD5(-)、CD23(-)、Cyclin D1(-),少数患者CD5(+),CD5(+)可能是眼附属器MALT预后不好的一个信号。

放射治疗ⅠE期非结膜眼附属器黏膜相关淋巴组织型淋巴瘤的剂量效应和预后

目的评价放射治疗ⅠE期非结膜原发眼附属器黏膜相关淋巴组织型淋巴瘤的剂量效应和预后。方法回顾性分析2003-11至2012-03收治的影像资料完整可测量33例(42只眼)ⅠE期非结膜原发眼附属器黏膜相关淋巴组织型淋巴瘤患者资料。结果照射剂量18 Gy时与27 Gy时,依据WHO标准评价疗效,缓解率分别为31%和42.9%(P=0.258);依据RECIST标准评价疗效,缓解率分别为28.6和38.1%(P=0.355)。全组患者局部控制率100%。全组患者的5年、10年总生存(OS)率分别为96.8%和84.7%;5、10年无进展生存(PFS)率分别为89.6%和89.6%。接受≥30.6Gy组与27 Gy组,5、10年总生存(OS)率分别为100%、80%和95.7%、95.7%(P=0.8578);5、10年无进展生存(PFS)生存率分别为83.3%、83.3%和91.1%、91.1%(P=0.6497)。结论非结膜原发眼附属器黏膜相关淋巴组织型淋巴瘤对放射治疗敏感。从局部控制和长期生存的观察结果显示,27 Gy的照射剂量是足够的。探索最佳的照射剂量需要开展前瞻性大样本的剂量效应观察研究。

ⅠE期非结膜眼附属器黏膜相关淋巴组织型淋巴瘤放射治疗的临床观察

目的评价放射治疗ⅠE期非结膜原发眼附属器黏膜相关淋巴组织型淋巴瘤的剂量效应和预后。方法回顾性分析2003年11月至2012年3月收治的影像资料完整可测量的33例(42只眼)ⅠE期非结膜原发眼眶黏膜相关淋巴组织型淋巴瘤患者资料。结果当照射剂量分别为18 Gy和27 Gy时,按照WHO标准评价33例患者的缓解率分别为31%和42.9%(P=0.258),按照RECIST标准评价缓解率分别为28.6%和38.1%(P=0.355);33例患者的局部控制率为100%。33例患者的5年、10年生存率分别为96.8%和84.7%,5年、10年无病生存率分别为89.6%和89.6%。接受≥30.6 Gy和27 Gy者的5年、10年生存率分别为100%、80%和95.7%、95.7%(P=0.8578),5年、10年无病生存率分别为83.3%、83.3%和91.1%、91.1%(P=0.6497)。结论非结膜原发眼附属器黏膜相关淋巴组织型淋巴瘤对放疗敏感,27 Gy的照射剂量即可获得较好局部控制和长期生存,确定最佳照射剂量仍需要开展前瞻性大样本的剂量效应研究。

眼附属器黏膜相关性淋巴组织淋巴瘤的治疗进展

眼附属器黏膜相关性淋巴组织淋巴瘤是一类恶性程度低的B细胞非霍奇金淋巴瘤,可发生在结膜、泪腺、眼眶和眼睑等多个部位,不仅影响到眼的正常功能,而且大大降低了患者的生活质量。目前的治疗方法主要有手术切除、放疗、化疗、根除鹦鹉热衣原体(Cps)的抗生素治疗及免疫治疗等。该文对眼附属器黏膜相关性淋巴组织淋巴瘤的治疗进展作一综述。

原发性眼附属器MALT淋巴瘤的临床类型与个体化治疗

黏膜相关淋巴组织(mucosa-associated lymphoid tissue lymphomas,MALT)淋巴瘤是原发性眼附属器淋巴瘤(primary ocular adnexal lymphoma,POAL)中最常见的病理类型。目前,原发性眼附属器黏膜相关淋巴组织淋巴瘤(primary ocular adnexal mucosa-associated lymphoid tissue lymphoma,POAML)的临床类型和临床表现尚未被眼科医师熟练掌握,临床治疗亦无共识和指南。本文根据POAML起源位置,重点介绍各临床类型的早中期临床表现,以及针对各临床类型和病变范围的个体化治疗方法。

眼附属器黏膜相关淋巴组织淋巴瘤的研究进展

黏膜相关淋巴组织(MALT)淋巴瘤是非霍奇金淋巴瘤中结外边缘带淋巴瘤(MZL)的一种特殊类型。眼附属器MALT淋巴瘤是眼眶恶性肿瘤中较常见的一种,可发生在结膜、泪腺、眼睑和眼眶。近年来,国外许多学者在MALT淋巴瘤的临床特点、病理形态学、免疫表型、分子遗传学及分子生物学方面对它的发生机制在基因水平有了新的认识。就国外对眼附属器MALT淋巴瘤的分子遗传学异常在肿瘤细胞内部信号转导通路中作用的研究做一综述。

眼附属器淋巴造血组织增生性疾病临床病理分析

目的探讨眼附属器淋巴造血组织增生性疾病的临床病理学特征。方法回顾性分析210例首都医科大学附属北京同仁医院眼附属器淋巴造血组织增生性疾病患者的临床资料和临床病理资料。结果 1该组眼附属器淋巴造血组织增生性疾病中最常见的是非霍奇金淋巴瘤(100例,47.6%),其次为炎性假瘤(45例,21.4%)和良性淋巴上皮病变(Mikuliczs病,41例,19.5%)。2该组病变部分类型在发病年龄和性别上有特点,组织病理学上的表现有交叉。3该部位非霍奇金淋巴瘤中最常见的为结外黏膜相关组织边缘区B细胞淋巴瘤(74例),眼附属器的结外黏膜相关组织边缘区B细胞淋巴瘤有其不同于其他解剖部位的该肿瘤的特点。4免疫组织化学染色在该部位淋巴造血组织增生性疾病的诊断和鉴别诊断中有非常重要的作用。5眼附属器的Ig G4相关性疾病(10例)的诊断和临床病理表现有其独有的特征。结论眼附属器淋巴造血组织增生性疾病是一类比较常见的疾病,其在发病年龄、性别等临床表现和组织学类型上都具有明显的临床病理学特征,掌握这些对临床病理诊断将提供较大的帮助。

眼附属器黏膜相关淋巴组织结外边缘区淋巴瘤诊治中国专家共识(2023版)

眼附属器黏膜相关淋巴组织结外边缘区(MALT)淋巴瘤是发生在眼附属器常见的恶性肿瘤,随着人口老龄化,眼附属器MALT淋巴瘤的发生率呈上升趋势。肿瘤组织侵犯眼附属器局部,容易导致眼部外观和功能的异常,影响患者的生活质量。目前眼附属器MALT淋巴瘤尚无统一的规范化诊疗指南,为了进一步提高眼附属器MALT淋巴瘤的规范化诊断和治疗水平,中国人体健康科技促进会细胞免疫治疗专业委员会、中国医师协会眼科医师分会眼肿瘤专业委员会、中国医疗保健国际交流促进会影像医学分会、中国抗癌协会肿瘤与微生态专业委员会学会和北京癌症防治学会淋巴瘤免疫治疗专业委员会专家联合编写了首个眼附属器MALT淋巴瘤诊治中国专家共识(2023版),以期为临床提供指导。

眼附属器MALT淋巴瘤的临床分析

目的探讨[附属器包括[睑、结膜、[眶和泪腺等部位MALT淋巴瘤的临床诊断要点,影像学检查特征与治疗和预后。方法回顾性分析1994年至2005年在第二军医大学长征医院[科确诊的45例[附属器MALT淋巴瘤的临床表现、影像学检查、手术治疗及病理结果与预后情况。结果[附属器MALT淋巴瘤23例发生于[眶,10例发生于[睑,8例发生于泪腺,4例发生于结膜。B超、CT、MRI均能进行定位、定性诊断,B超能较好地显示病变内部结构和形态;CT对眶骨结构和肿瘤侵犯范围与周围组织的关系显示良好,具有定性诊断价值;MRI对软组织的分辨力强,成像参数根据不同的信号强度可提示病变的内部结构。45例均行手术治疗;其中22例辅以放疗,10例结合化疗。42例随访4～135个月,1例于手术切除后25个月转移至肺死亡,其余均存活。结论[附属器MALT淋巴瘤好发于[眶上方及[睑,影像学检查对MALT淋巴瘤的诊断及鉴别诊断有帮助。手术切除肿瘤,结合病理诊断和临床分期判断能提示较准确的病情估计,病变可长期局限,预后较好,一般不会出现全身转移和向弥漫性大细胞高度恶性淋巴瘤转化。

眼附属器黏膜相关淋巴组织边缘带淋巴瘤的PET/CT表现与临床分析

目的:描述眼附属器黏膜相关淋巴组织边缘带淋巴瘤18F-FDG PET/CT特点,并探讨最大标准摄取值(SUVmax)与相关临床指标的关系。方法:回顾性分析33例眼附属器黏膜相关淋巴组织边缘带淋巴瘤患者治疗前的18F-FDG PET/CT检查,并对SUVmax与患者基线实验室结果进行相关性分析,比较不同亚组间SUVmax差异。结果:除外手术完全切除病灶的6例患者,眼附属器黏膜相关淋巴组织边缘带淋巴瘤18 F-FDG PET/CT阳性率为92.6%,SUVmax范围为2.2~10.6,均值为5.24±2.17;SUVmax与超敏C反应蛋白(HsCRP)、血沉(ESR)呈显著正相关,而与血白蛋白(Alb)、血红蛋白(Hb)呈显著负相关;结膜组SUVmax显著低于眶周组。结论:18F-FDG PET/CT可以作为眼附属器黏膜相关淋巴组织边缘带淋巴瘤患者分期评估的方法,SUVmax与HsCRP、ESR、Alb、Hb可能反映患者肿瘤的炎症程度,并可能会成为判断疾病预后的指标。