The Classification, Clinical And Pathological Features Of The Ocular Adnexal Lymphomas

Objective:In this study,82 cases of ocular adnexal lymphomas were reclassified according to the World Health Organization classification of tumors and haematopoietic and lymphiod tissues(2001),to analyze the applicability of the new WHO classification in ocular adnexal lymphomas,to observe the incidence of different type and the clinical features and histopathological classification,to further understand the features of this disease,to establish foundation for diagnosis and individuation therapy and prognosis prediction of ocular adnexal lymphomas.Methods:The clinical and histomorphological features of 82 cases of ocular adnexal lymphomas were studied retrospectively.We analysed the immunophenotype of them with immunohistochemistry and reclassified them according to WHO classification.Results:1 Clinical features:According to the WHO classification, 77 cases of were diagnosed as ocular adnexal lymphoma.The patients included 51 males and 26 females,aged 10 to 85 years,with a mean age of 52.8 years.The mean duration between the onset of the symptoms and the time of presentation was 20 months,from 10 days to 22 years.Lymphoma involved the ocular adnexa of the left eye in 38 cases,and of the right eye in 29 cases.In 10 cases,lymphoma was bilateral.Proptosis and edema,swelling or fullness were most frequent clinical manifestation.2 Reclassification result:77 cases ocular adnexal lymphoma, including the nine subtypes,were classified as follows: there were 37 cases of MALT(48.05%),13 cases of DLBL(16.88%),9 cases of PCL(11.69%),6 cases of PTCL-U(7.79%),4 cases of MCL(5.19%),2 cases of SLL (2.60%),1 case of BL(1.30%) and 1 cases of NK/T,as well as 4 cases(5.19%) cannot classified.The result showed that the ocular adnexal lymphomas were mostly MALT type.3 Immunophenotype:In 66 cases of B-NHL,56 cases expressed CD20 and CD79a markers,all cases CD3 did not express,4 cases expressed CD43.37 cases of MALT were negative for CD10,CD23 and cyclinD1,but 3 cases were CD5 positive,34 of which showed immunoglobin light chain restriction.There were 2 cases CD10 and 8 cases BCL-6 and 4 cases MUM-1 and 2 case CD30 positive in 13 DLBL.2 cases were PC positive and2 cases were CD138 positive in 9 PCL. All the 4 cases of MCL expressed cyclinD1 and BCL-2,2 of which expressed CD5.Of 2 SLL,1 case CD23 and 1 case CD5 were positive.The one BL expressed CD10,BCL,2 and BCL-6. In 6 PTCL-U,all cases expressed CD3,CD43 markers,all cases CD20,CD79a and CD56 did not express.The only one NK/T expressed CD2,CD43,CD56 and TIA-1,as well as CD20,CD79a and CD3 were negative.Conclusions:MALT is most common type in the ocular adnexal lymphomas.The ocular adnexal lymphomas show an indolent course clinically,tending to affect the old of male. Bilateral case is not uncommon.Morphology is still foundation for diagnosis of the ocular adnexal lymphomas.The immunohistochemistry analysis plays an important role in differential diagnosis and further subtyping.A combination of both modalities and clinical feature are sufficient for arriving at an accurate diagnosis in most cases.The WHO classification is suitable for the subdivision of ocular adnexal lymphomas.