| BackgroundLymphoma is a malignant tumor originating from lymph nodes or extradodal lymph nodes and tissues,with painless lymph node enlargement accompanied by hepatosplenomegaly as the first symptom,as well as systemic symptoms such as fever,night sweats,emaciation and pruritus,which can involve organs and tissues throughout the body.In 2015,the prevalence rate in China was 6.89 per 100,000,which is one of the common malignant tumors in China.Lymphoma classification is various,generally can be divided into two categories,Hodgkin’s lymphoma(Hodgkin’s lymphoma,HL)and non-hodgkin’s lymphoma(non-Hodgkinlymphoma,NHL),of which the highest incidence of NHL,is a highly invasive heterogeneous malignant tumor,and Diffuse large B cell lymphoma,Diffuse large B-cell lymphoma,DLBCL)from mature B lymphocytes,is the most common type of NHL in the clinical process and form highly invasive biology,It is characterized by poor prognosis,easy recurrence and poor therapeutic effect.The adrenal gland is one of the important endocrine organs of the human body,which is composed of medulla and cortex.The structure and function of the two are different and interrelated.The adrenal gland maintains the development and growth of the human body,the balance of water electrolyte and energy metabolism and the stability of blood pressure.The majority of adrenal tumors are benign tumors,and primary adrenal malignancies(adrenal cortical carcinoma,primary adrenal lymphoma)are relatively rare.When the medical effect of adrenal diseases is not obvious or there is no medical treatment,surgical means appear to be extremely important,especially the popularization of laparoscopic and Da Vinci robotic surgical system and other technologies has brought revolutionary changes to the urology department.Primary adrenal lymphoma(Primary adrenal lymphoma,PLA)is a kind of very rare high invasive malignant tumor,its etiology is unknown,atypical clinical symptoms,imaging specificity,eventually need pathologic diagnosis,because there is no uniform guidelines and standards,for clinical doctors on diagnosis and treatment of the disease has brought a lot of trouble.ObjectiveThrough the analysis in the first affiliated hospital of zhengzhou university of 41 cases of primary adrenal diffuse large B cell lymphoma(primary adrenal diffuse large B-cell lymphoma,PADLBCL)in patients with clinical data,analysis PADLBCL clinical characteristics,treatment and prognosis,to explore factors affecting their survival and prognosis,and to offer some diagnosis and treatment for the clinical diagnosis and treatment of train of thought,to optimize PADLBCL method of diagnosis and treatment,improve the prognosis of patients.MethodsReview of the first affiliated hospital of zhengzhou university 2020 between January 2010 and admitted the 41 cases clinical data of primary adrenal diffuse large B cell lymphoma,in general,such as gender,age,height,such as presence of hpi B symptoms(fever,weight loss,night sweats),presence of adrenocortical insufficiency,abdominal pain,etc.,past medical history such as hypertension,diabetes,etc.,mainly for the general physical examination data superficial with and without lymph node enlargement,laboratory data such as lactate dehydrogenase,beta 2-micro globulin,albumin,iron,protein,etc.,Imaging and pathological findings,such as tumor size and IPI score,were analyzed for demographic data,biochemical indicators,imaging,pathological findings,treatment plan,and prognostic factors.Results1.Among the 41 PADLBCL patients,23(56.0%)were over 60 years old,and 18(44.0%)were 40-60 years old.The mean age of onset was 61.7 years old,and the median age was 63 years old.Most of the patients were male,with 35 males and 16 females,and the male to female ratio was 5.8:1.0.Bilateral lesions were found in 26 patients and unilateral lesions in 15 patients.2.The clinical features were varied but non-specific,and the main manifestations were:abdominal pain in 19 patients(46.3%),symptom B(fever,weight loss,night sweats)in 19 patients(46.3%),adrenocortical dysfunction in 11 patients(26.8%),and asymptomatic in 7 patients(17.0%).3.Laboratory examination of adrenal hormone abnormalities in 11 patients showed elevated ferritin in 23 patients(56.0%),elevated lactate dehydrogenase in 21 patients(51.2%),elevated β2-microglobulin in 19 patients(46.3%),and decreased albumin in 16 patients(39.0%).The ultrasonic manifestations of PADLBCL were homogeneous and hypoechoic masses.CT plain scan showed round or oval isopdensities or mixed density shadows.There was light to moderate enhancement in both arterial and parenchymal phases,and the enhancement pattern was uneven.MRI showed low signal inhomogeneity on T1-weighted images,slightly high signal inhomogeneity on T2-weighted images,and significantly heterogeneous enhancement on enhanced scan,PET/CT examination showed adrenal malignancy with significantly enhanced glucose metabolism.4.Among the 41 patients with PADLBCL,13 patients received surgery combined with chemotherapy,14 patients received chemotherapy alone,6 patients received surgery alone,and 8 patients chose conservative observation.The 2-year OS(42.9%vs49.2%vs0.1%)of the three patients was statistically significant compared with chemotherapy alone and chemotherapy combined with surgery,respectively.Although there was no statistical difference in the 2-year OS between chemotherapy and surgery combined with chemotherapy(P=0.541>0.05),the 2-year OS of surgery combined with chemotherapy was greater than that of chemotherapy alone.5.All the 41 DLBCL patients were pathologically diagnosed as adrenal diffuse large B-cell lymphoma,of which 5 cases were of GCB subtype and 36 cases were of non-GCB subtype.6.All patients were followed up for 1-63 months,with an average follow-up time of 18.2 months.1-year OS was 70.7%,2-year OS 46.3%,and 3-year OS 15.4%.7.Univariate analysis showed tumor≥10cm,accompanied by B symptoms,high IPI score,elevated lactate dehydrogenase,low albumin,elevated β 2 microglobulin,elevated ferritin,no use of rituximab,and accompanied by adrenal insufficiency,suggesting a poor prognosis.Multivariate analysis showed that lactate dehydrogenase,β2 microglobulin and chemotherapy were independent prognostic factors of PADLBCL.Conclusion:1.Primary adrenal lymphoma is very rare,with the onset age mainly in middle and old age,bilateral adrenal involvement is more common,and clinical manifestations are diverse but lack of specificity.2.Adrenal endocrine hormone,liver and kidney function,tumor markers and ultrasound,CT,MRI,PET/CT imaging examinations are helpful for the diagnosis and prognosis of PADLBCL,but the final diagnosis requires pathological results.3.At present,adrenal resection combined with rituximab monoclonal antibody and CHOP chemotherapy regimen(cyclophosphamide+doxorubicin+vincristine+prednisone)is still an effective first-line treatment regimen for primary adrenal DLBCL.However,PADLBCL is a malignant tumor with a poor overall prognosis.4.According to univariate analysis,age,B symptoms,IPI score,lactate dehydrogenase,low albumin,β2 microglobulin,ferritin,and adrenal insufficiency were the prognostic factors of PADLBCL.Multivariate analysis showed that lactate dehydrogenase,β2 microglobulin and albumin were independent prognostic factors of PADLBCL. |
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