Clinical Features And Prognosis Of Primary Breast Lymphoma

Background:Primary breast lymphoma(PBL)is an extrinsic non-hodgkin's lymphoma originating in the breast,which accounts for about 0.04%-0.5%[1]of breast malignancies,and more than 70%[2]is from B cell line.The incidence of extrinsic lymphoma is on the rise in general while the incidence and prognosis of extrinsic lymphoma in different sites are different.This study focuses on the clinical features and prognostic risk factors of PBL.The specific causes and mechanisms of PBL are still unclear.Currently,there is no unified and standardized treatment scheme.Both the clinical manifestations and the specificity of auxiliary examination of PBL are poor.Histophysiology and immunohistochemistry are the gold standards for the diagnosis of PBL.PBL has a variety of pathological types and is prone to central nervous system(CNS)recurrence[3].Once CNS is involved,the prognosis is extremely poor[4,5].Objective:The clinical characteristics and prognostic factors of patients with primary breast lymphoma were analyzed to provide reference for the standardized treatment of PBL.Methods:From January 2009 to January 2019,18 patients with pathologic diagnosis of PBL were admitted to the people's hospital of Zhengzhou university.Summarize and sort out the clinical and pathological data of these patients including the patient's age,sex,tumor size,presence of ipsilateral axillary lymph node involvement,clinical stage,pathological type,with or without B symptoms(fever,night sweats,recent systemic symptom such as weight loss),serum lactate dehydrogenase,serum?2-microglobulin,international prognostic index score.The treatments on prognosis were analyzed.SPSS 20.0 software was used to statistical analysis.Quantitative data was described by median quartile spacing.Qualitative data was expressed as percentages.Survival data and prognosis were analyzed by Kalpan-Meier method.In univariate analysis,the Log-rank test was used to compare the survival curves.COX proportional risk model was used to analyze the influence of clinical factors on basic prognosis.The test level a was set at 0.05,which P<0.05 was considered statistically significant.Results:(1)Cases and clinical manifestations:Among the 18 patients,17 were female(94.4%)and 1 was male(5.6%).The median age of onset was 48 years old(27-66 years old).According to the clinical indicators of IPI score,16 patients(88.9%)were under 60 years old,and 2 patients(11.1%)were over 60 years old.Bilateral involvement in 1 case(5.6%),right involvement in 9 cases(50%),left involvement in 8 cases(44.4%).All the 18 patients presented with painless breast tumor as the first symptom,including 6(33.3%)patients with IPL involvement and 12(66.7%)patients without involvement.There were 3 cases(16.7%)with symptoms B,mainly night sweats,and 15 cases(83.3%)without symptoms B.No lactation,pregnancy,dimple sign,orange peel sign,nipple depression or other typical characteristics of breast cancer.Tumor size:a total of 19 lesions were found in 18 patients(1 bilateral patient),with a median maximum diameter of 5cm(3-12cm),a maximum diameter of<5cm in 8 patients(44.4%),and a maximum diameter of?5cm in 10 patients(55.5%).(2)Pathological types and immunohistochemistry:13 patients(72.2%)were DLBCL,1(5.6%)Peripheral t-cell lymphoma(PTCL),1(5.6%)clinical associated lymphoid tissue(MALT),1(5.6%)clinical associated lymphoid tissue(MALT),and 1(5.6%)follicular lymphoma(FL).Two cases(11.1%)were marginal B-cell lymphoma(MZBL).Among the 13 DLBCL patients,2(11.1%)were of non-germinal center B-cell(non-gcb)type,4(22.2%)were of germinal center B-cell(GCB)type,4(22.2%)were of activated type,and 3(16.7%)were of unknown cell origin.8 patients(44.4%)had Ki67?80%.(3)The clinical staging and IPI score:PBL staging is controversial.This study refer to Ann Arbor staging standard combining Wiseman and PBL diagnosis standard,proposed by Liao divides into the PBL ? E period,? E period,? period.12 patients with I E period(66.7%),5 patients with ? E period(27.8%),? period 1 case(5.6%)patients.There were 14 patients(77.8%)with an IPI score of 0-1 and 4 patients(22.2%)with an IPI score of 2-4.7 patients(38.9%)and 8 patients(44.4%)had increased LDH.(4)Treatment and prognosis:1 case(5.6%)received focal resection alone,2 cases(11.1%)received chemotherapy alone,9 cases(50%)received surgery+chemotherapy,4 cases(22.2%)received chemotherapy+radiotherapy,1 case(5.6%)received surgery+radiotherapy+chemotherapy,and 1 case(5.6%)received surgery+radiotherapy.Five patients(27.8%)received Rituximab(R)and six(33.3%)received intrathecal chemotherapy to prevent CNS recurrence.The median survival time of 18 patients was 60 months(12-110 months).During the follow-up period,12 patients(66.7%)survived,6 patients(33.3%)died,13 patients obtained CR,3 obtained PR,1 obtained PD after treatment,and 1 patient with PTCL experienced IPI breast skin recurrence during the treatment of local resection combined with R-CHOP.The results of single-factor analysis showed that IPI score?2(P=0.003)was an independent risk factor affecting survival while other indicators had no significant correlation with the poor prognosis of PBL.Conclusion:(1)PBL is rare in clinic,mainly in women,lacking typical clinical features and difficult in preoperative diagnosis.DLBCL is the most common pathological type.(2)The significance of surgery for PBL lies more in the acquisition of pathological materials rather than as a means of treatment which cannot improve the prognosis of patients.(3)IPI score(P=0.003)is an independent risk factor for poor prognosis of PBL that patients with IPI score ?2 points and IPI score 0-1 points have poor prognosis.