| Background and purposeIsaacson and Wright first proposed the concept of mucosa-associated lymphoid tissue(MALT)lymphoma in a study of gastric lymphoma in 1983.The international lymphoma research group published Revised European American Lymphoma Classification(REAL)in 1994,which revised it as a new type of marginal zone B-cell lymphoma.MALT lymphoma was classified as extra-nodal marginal zone B cell lymphoma(EMZL)of mucosa-associated lymphoid tissue type(MALT)in the 2001 World Health Organization(WHO)classification,also known as MALT lymphoma.Ocular adnexal MALT lymphoma(OAML)is the most common subtype,which may account for about 38%to 76%of ocular adnexal lymphomas(OAL).OAML can occur at all ages,but the almost in the elderly.The orbit is the most frequently involved anatomical structure,accounting for 55%,and also located in the lacrimal gland,conjunctiva and eyelids.The lesions were often unilateral.OAML can be divided into primary,secondary and multiple according to the pathogenic process.The incidence of OAML has been increasing in recent years.According to the latest research,OAML is the most subtype of primary ocular adnexal lymphomas(POAL),which account for 90%.Today,there are a variety of treatment options.OAML treatments include surgery,radiotherapy,chemotherapy,immunotherapy,antibiotic therapy and interferon therapy.Among them,radiotherapy(RT)is frequently used in the treatment of local OAML,and chemotherapy is a common treatment of choice when OAML with systemic invasion.OAML is the best prognosis of all POAL,and 5-year overall survival(OS)rate is up to 90%.At present,clinical types are classified according to the primary site of the lesion and the scope of the imaging feature,which are eyelid type,conjunctival type,lacrimal gland type and orbital type.Although there are many treatment methods and the prognosis is good,the selection of the current clinical type guidance treatment model needs to be improved.Therefore,this study retrospectively analyzed the clinical medical records of the disease,focusing on summarizing the clinical stage,clinical type characteristics and treatment mode of the disease,which is helpful of the diagnosis and treatment of OAML in the future.1 Research subjectsCollected and analyzed 57 patients of OAML with complete data in The Frist Affiliated Hospital of Zhengzhou University from June 2012 to June 2018.Enrollment criteria included ophthalmic symptoms were predominant at the time of diagnosis,all were diagnosed with MALT lymphoma for the first time by disease examination or resection,pathological immunohistochemistry or gene rearrangement.Exclusion criteria included patients with lymphoproliferative diseases who cannot be diagnosed with lymphoma,other lymphoma pathological types and previous history of lymphoma was excluded.This retrospective analysis obtained informed consent of patients and was approved by the Medical Ethics Committee of the First Affiliated Hospital of Zhengzhou University.2 Research methodsWe collected statistics of 57 OAML patients including age,course of disease,eye classification,primary site,imaging results,pathological results,clinical stage,disease progression,and survival.Collect follow-up data of all patients through telephone,outpatient or inpatient review.The follow-up plan is divided into:?3 months,6 months,12 months,15 months,18 months,21 months,24 months after the end of treatment;?Follow up every 6 months after 2 years.?Follow-up content include the primary tumor changes and system evaluation.Clinical staging includes Ann Arbor staging system and AJCC-TNM clinical staging.The following prognosis and survival index includes Progression-free survival(PFS)and Overall survival(OS).Results1.A total of the 57 patients with OAML,7 patients(12.3%)presented with conjunctival OAML,11 patients(19.3%)with lacrimal gland OAML,39 patients(68.4%)with orbital OAML(13 cases of anterior orbital OAML,5 cases of posterior orbital OAML,and 21 cases of diffuse orbital OAML).This group included 33 males(57.9%)and 24 females(42.1%),age from 27 to 83 years(median age was 58 years),including conjunctival OAML 48.1 years,lacrimal gland OAML 57.5 years,and orbital OAML 60.5 years.49 patients(86.0%)were affected laterally,and 8 patients(14.0%)were bilaterally.The orbit(68.4%,39 patients)was the most frequently involved anatomical structure,followed by the lacrimal gland(19.3%,11 patients)and conjunctiva(12.3%,7 patients).2.The most characteristic presenting signs and symptoms of primary ocular adnexal lymphomas was eyelid swelling(78.9%,45 patients),a palpable masses(77.2%,44 patients),proptosis(22.8%,13 patients),movement restriction(22.8%,13 patients),ptosis(15.8%,9 patients),conjunctival swelling(mentioned as "salmon-pink patch")(14.0%,8 patients)and hypopsia(10.5%,6 patients).3.CT examination showed soft tissue shadows of medium and uniform density without bone invasion(100%,57/57).MRI examination mostly showed isosignal in T1WI and T2WI(96.0%,22/23).MRI examination mostly showed isointense in T1-and T2 weighted imaging(96.0%,22/23).B ultrasound examination showed hypoechoic and incompressible solid lesions.Color doppler flow imaging(CDFI)showed Enrich blood flow signals(77.8%,7/9)and two patients with Spot blood flow signal.Extraocular muscles were involved in 46 cases(80.7%),including 3 patients of conjunctival OAML,7 patients of lacrimal gland OAML,and 36 patients of orbital OAML,17 patients(29.8%)wrapped around the eyeball.4.HE staining showed that small lymphocytes were predominant,with deep staining,less cytoplasm,and pale staining,and germinal center-like cells were visible.Immunohistochemical staining showed that positive expressions of CD20 and CD79a,negative expressions of CyclinD1,Bcl-6,CD 10.Twenty patients of gene rearrangement suggested a monoclonal rearrangement of the IgH gene.5.Fifty-three patients with primary disease had stage IE according to Ann Arbor staging system at the time of diagnosis,three patients had stage ? disease and one patient had stage ?.There are no stage ? patients in this data.According to AJCC-TNM staging,7 patients(12.3%)had stage T1,40 patients(70.2%)had stage T2,10 patients(17.5%)had stage T3,included one patient at stage T1 had stage N1,one patient at stage T2 had stage N1 and two patients at stage T3 had stage N2.There were no T4 and M patients in this data.The median follow-up time was 52 months(range,21-93 months).During the follow-up,recurrence or progression was observed in 15 patients(26.3%,15/57)and 3 patients died(5.3%,3/57).Death was directly attributed to lymphoma in 2 of 3 patients.And 1 patient died due to other lung diseases.Survival data were available in 57 patients in this study.The 5-year OS for entire group was 91.2%and the 5-year PFS was 59.4%.When comparing AJCC/TNM stage,there was significant difference in the 5-year PFS between patients with stage T?3(14.5%)and T<3(71.1%),p<0.001.Conclusion1.Patient were often middle-aged or older(median age,58 years).And those with primary location of conjunctiva are younger.The history of the disease is long,with a median history of 1 year.Overall,OAML has a high overall survival rate,the prognosis is well,but there are still many relapses and/or progressions after treatment.2.The clinical manifestations are mainly eyelid swelling,and the main symptoms vary according to the location of the lesion.For example,the conjunctiva is visible and the characteristic "salmon-pink patch" appearance under the conjunctiva,and the orbital type may have different clinical features such as proptosis but not their clinical characteristic.3.TNM staging is based on ocular adnexal anatomical structure and lymphatic drainage relationship in evaluating severity of OAML,and has statistical significance in prognostic evaluation,and may be more suitable for OAML than Ann Arbor staging.4.According to the characteristics of clinical manifestations and imaging characteristics,OAML is subdivided into five clinical types.This clinical types of OAML can accurately summarize their clinical characteristics,which is helpful to choose the appropriate treatment options. |
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