| Objective To retrospectively analyze the clinical characteristics,treatment and prognosis of 8 cases of primary adrenal diffuse large B-cell lymphoma(PADLBCL)in our hospital and 107 cases of PubMed database,so as to further improve the understanding of PADLBCL.Methods The clinical data of 8 patients with PADLBCL treated in the First Affiliated Hospital of Guangxi Medical University from January 2005 to January 2019 were collected.The English literature of PADLBCL republished in PubMed during the same period was reviewed.The clinical manifestations,imaging and pathological features,treatment and prognosis of PADLBCL were analyzed retrospectively.Results1.The median age of onset of PADLBCL was 48.5(46.0-60.5)years for middle-aged male patients aged 50-60 years,and 62.5(53.8-74.3)years for PubMed database.The age of onset was skewed,and the sex ratio was 3.26:1.2.The clinical manifestations of the disease were not specific.Comparing the cases in our hospital and PubMed database,low back pain(37.5% VS 54.9%)was the first symptom in some patients,accompanied by fatigue(37.5% VS 77.1%)and B symptoms(87.5% VS 65.4%).There were no adrenocortical insufficiency patients in our hospital.Two cases(25% VS 14.1%)had metastasis/recurrence of central nervous system(CNS).LDH increased in most patients(100% VS 84.0%),and Ann Arbor stage III-IV(87.5% VS 70.2%)and IPI/aa IPI score,the patients were at high/ medium and high risk(75.0% VS 78%).3.The detection rate of CT malignant tumors in 8 PADLBCL patients was 87.5%.The disease is predominant in bilateral adrenal glands.Some of the masses are larger in size and can be larger than 10 cm in diameter(37.5% VS 37.0%).PET-CT is helpful to determine whether the lesion is the primary lesion and the site of the tumor.4.CD20 was expressed in 8 PADLBCL patients,CD79a(7/7)was expressed in 7 PADLBCL patients,and CD10 and CK(0/6)were not expressed in any PADLBCL patients.The Hans classification of most patients in our hospital and PubMed database was non-GCB(75% VS 91.1%).EBERs was positive in 1 patient(1/1)and PubMed was positive in 3 patients(3/14).5.Of the 8 PADLBCL patients in our hospital,1 was treated only by surgery,6 by chemotherapy,and 1 by combination of chemotherapy and autologous hematopoietic stem cell transplantation.One patient lost follow-up after treatment,seven patients were followed up until March 22,2019.The follow-up period was 20 days to 100 months.The median total survival time was 14 months.Two patients survived(OS 29 months and 100 months,respectively).One patient was treated with chemotherapy combined with autologous hematopoietic stem cell transplantation.Four patients progressed after treatment,one relapsed and five died.Conclusions1.The heterogeneity of PADLBCL is obvious.The incidence of PADLBCL in males is significantly higher than that in females.50-60 years old is the age group with high incidence.The clinical manifestations of PADLBCL are nonspecific.Lumbago is the first symptom,and the lesions often involve bilateral adrenal glands.2.CT has a high detection rate of PADLBCL.PET-CT has important clinical value in differential diagnosis and staging of PADLBCL.Non-GCB is the most common subtype of the disease,often accompanied by poor prognosis.3.PADLBCL has high invasiveness,poor prognosis,and is prone to CNS metastasis/recurrence.The disease progresses rapidly after CNS involvement.Therefore,R-CHOP combined with HD MTX is recommended. |
PDF Full Text Request