Clinical Characteristic And Prognostic Factors Of Primary Angiosarcoma Of The Kidney

Background and ObjectiveAngiosarcoma is a malignant tumor originated from vascular endothelial cells or differentiated into vascular endothelial cells.It can occur in any part of the body,the most common is in skin and soft tissue,especially in the head and face,and a few of them can occur in the breast,liver,thyroid gland and other solid organs.The malignant degree of angiosarcoma is high,which can often occur local lymph node metastasis and distant metastasis of liver,lung and bone.Angiosarcoma involving the kidney is usually caused by metastasis from other sites,and the primary angiosarcoma of the kidney is very rare.The prognosis of primary angiosarcoma of the kidney is very poor,and most of them have metastasis at the time of discovery.Because of its low incidence,there is still no effective treatment experience in clinical practice,and there is no standard treatment criterion at present.This study summarized the clinical features of 45 cases of primary angiosarcoma of the kidney,and made a detailed statistical comparison of the etiology,clinical manifestations,treatment methods and prognostic factors of the disease,in order to find the best treatment for the disease and further study the relationship between prognosis and clinical common factors.Subjects and MethodsPubmed,CNKI,Wanfang Date and other databases were searched for all articles and case reports on primary angiosarcoma of the kidney.The search key words include "kidney","primary" and "angiosarcoma".There is no language restriction in the search,and the reference list of related articles is searched manually.The search began in 1942 and ended in December 2018,80 patients of primary angiosarcoma of the kidney reported domestic and abroad,and 2 patients of primary angiosarcoma of the kidney diagnosed and treated in the first affiliated hospital of zhengzhou university from December 2011 to December 2018 were collected,45 cases meeting the requirements were screened out through inclusion and exclusion criteria for relevant survival analysis.The clinical features were summarized,including age,sex,tumor size,affected side,treatment,concurrent metastasis,postoperative radiotherapy and chemotherapy,and postoperative survival time.SPSS 17.0 software package was used for analysis,Kaplan-meier method was used to draw survival curve,and Log-rank test was used for univariate analysis.The independent risk factors affecting the prognosis of primary angiosarcoma of the kidney were screened by Cox risk model,P < 0.05 was statistically significant.Results(1)45 patients in this group,all patients were pathologically confirmed as primary angiosarcoma of the kidney,follow-up time is 1 to 55 months.Among the patients,38 were male and 7 were female,and the ratio of male to female was 5.4 :1.The morbidity of male(84.4%)was significantly higher than that of female(15.6%).The episode age is 24 to 95,average age are(60.1 ±14.1)years;the diameter of tumor was 2.2 to 26 cm and the average diameter was(10.7 ±7.0)cm.The tumor was located at the left side in 28(62.2%)and the right side in 17(37.8%).Asymptomatic cases accounted for 15.6%,clinical symptoms accounted for 84.4%,the most common symptoms were waist / abdominal pain(57.8%),hematuria(40.0%),and other non-specific symptoms such as weight loss,fatigue,and loss of appetite accounted for 44.4%.There were 29 patients(64.4%)with lymph node metastasis or distant metastasis;19 patients(42.2%)received radiotherapy/chemotherapy.(2)Univariate analysis showed that tumor size(P=0.001),combined metastasis(P< 0.001)were the main factors affecting the prognosis of primary angiosarcoma of the kidney,and age(P=0.647),sex(P=0.332),affected side(P=0.428)and radiotherapy or chemotherapy(P=0.345)have no relation with the prognosis of primary angiosarcoma of the kidney.(3)COX multivariate analysis showed that tumor size(RR=3.396,RR95%CI 1.125 ~ 10.254,P=0.030)and combined metastasis(RR=7.040,RR95%CI 1.76 ~ 27.900,P=0.005)were independent risk factors affecting survival time of patients with primary angiosarcoma of the kidney.ConclusionPrimary angiosarcoma of the kidney is a rare and highly malignant tumor.The morbidity of the disease is much higher in men than in women.The pathogenesis of the disease is still unclear,and most of the patients are around the age of 60.Without obvious specificity of clinical symptoms,it is difficult to distinguish from other renal cancers,and it is difficult to make a clear diagnosis before surgery,which often requires postoperative histopathological and immunohistochemical analysis.Tumor size and metastasis are the main factors affecting the prognosis of primary angiosarcoma of the kidney.At present,it is considered that radical surgery with adjuvant chemotherapy is the best choice for the treatment of primary renal adjuvant,but the effects of chemotherapy and radiotherapy still need to be further observed and studied.With the advance of molecular and genetic research on angiosarcoma,a variety of new molecular targeted drugs have emerged,which may cure the disease in the near future.