Clinical Features,Treatment And Prognostic Factors Of Primary Appendiceal Tumors

Objective: Primary appendiceal tumor is a very rare gastrointestinal tumor.By discussing the clinical features,treatment methods and prognostic factors of appendiceal tumor,we can provide reference for the diagnosis and treatment of appendiceal tumor.Methods: The clinicopathologic data of patients with primary appendicular tumor who met the inclusion and exclusion criteria were retrospectively collected from the Affiliated Hospital of Southwest Medical University from January 2013 to November 2022,in order to explore the clinical characteristics and treatment plan of the patients.Kaplan-Meier method was used for survival analysis,Logrank test was used for univariate group comparison of patients’ prognosis,and Cox regression model was used for multivariate analysis of patients’ prognosis.Results: There were 82 patients who met the inclusion and exclusion criteria,including 9 patients complicated with other malignant tumors.There were 34 males(41%)and 48 females(59%),with a median age of 63(14,80)years and an average age of 60.1 years.After excluding patients with other malignant tumors,the top ten clinical manifestations of patients with primary appendiceal tumor were abdominal pain(65 cases,89.0%),change of bowel habits(21 cases,28.8%),abdominal distension(13 cases,17.8%),vomiting(11cases,15.1%),nausea(10 cases,13.7%),abdominal mass(6 cases,8.2%),poor appetite(5 cases,6.8%),fever(3 cases,4.1%),acid reflux(2 cases,2.7%),hiccup(2 cases,2.7%).Among them,ascites was found in 22 cases(31.9%)and jelly-like substance in abdomen and pelvis was found in 17 cases(24.6%).25 cases of appendicitis and 12 cases of intestinal obstruction were confirmed by operation.There were 22 patients with high malignancy and 22 patients with metastasis.In the past history,there were 17 people with hypertension and 8people with diabetes.There are 20 smokers and 28 drinkers.Among the patients who could be followed up for preoperative examination,CEA increased(CEA> 6ng/ml)in 25 cases and CA199 increased(CA199 > 37IU/ml)in 22 cases.There were 20 patients with anemia(male<130g/L,female < 115g/L)and 26 patients with decreased serum albumin(albumin<40g/L).The median leukocyte value was 6.78(4.13,22.1)10*9/L,and the median platelet value was 232(121,613)10*9/L.The median prognostic nutrition index(PNI)was49.7(20.1,60),the mean inflammatory markers(PLR)was 190.1375,and the median inflammatory markers(NLR)was 3.68(0.22,28.77).Histopathological types of 82 patients:There were 42 low-grade mucinous tumors,2 high-grade mucinous tumors,4 mucinous adenomas/cystadenomas,3 mucinous adenomas,5 adenocarcinomas,2 signet ring cell carcinomas,2 adenocarcinomas with signet ring cell carcinomas,6 goblet cell carcinomas,5 neuroendocrine tumors,2 appendix carcinomas(not classified),9 adenomas/adenomatous polyps(6serrated adenomas,1 case villous adenoma,1 case adenomatous polyp,1 case tubular adenoma).Among the treatment methods of patients without other malignant tumors,50 cases were treated by simple surgical resection,1 case by simple chemotherapy,18 cases by operation combined with chemotherapy(including 3 cases by thermal perfusion),and 2 cases by operation combined with thermal perfusion.One case was diagnosed as poorly differentiated adenocarcinoma by colonoscopy and failed to be treated for economic reasons,and one case gave up treatment after being diagnosed as metastatic adenocarcinoma of appendix by peritoneal puncture cytology.Among the surgical methods,there were 27 cases of emergency operation,accounting for38.6% of the operation.There were 35 cases of right hemicolectomy,16 cases of appendectomy,11 cases of combined organ resection,6 cases of ileocecal resection,1 case of argon ion cauterization of appendix polyp,and 1 case of left paracolonic mass resection.Among them,10 cases were diagnosed as appendix tumor after appendectomy in other hospitals,and two-stage right hemicolectomy was performed in our hospital,and one case underwent two-stage ileocecal resection after appendectomy.The chemotherapy regimen of patients is FOLFOX regimen,and on this basis,capecitabine or bevacizumab and cetuximab are combined;Or XELOX regimen combined with Tiggio or bevacizumab,irinotecan,and paclitaxel chemotherapy alone.After excluding 9patients with appendix tumor complicated with other malignant tumors,69 patients were followed up,and 4 patients were lost.The median follow-up time was 32 months(4-106 months).Of the 69 patients who were followed up,18 patients died.In the prognosis analysis of patients,after excluding patients with appendiceal adenoma/adenomatous polyp,the cumulative survival rates of patients at 1 year,2 years,3 years and 5 years were 90.3%,88.3%,80.7% and58.3% respectively.Univariate analysis showed that gender,age,hypertension,diabetes,smoking history,drinking history,appendicitis,ileus,gelloid substances in abdominal and pelvic cavity,leukocyte,platelet,serum inflammatory markers NLR,peritoneal pseudomyxoma,adjuvant chemotherapy,and intraperitoneal thermoperfusion chemotherapy were not correlated with the prognosis of patients with appendiceal tumor(P>0.05).Serum tumor markers CEA(P=0.004),CA199(P<0.001),anemia(P<0.001),serum albumin(P<0.001),prognostic nutrition index PNI(P=0.004),serum inflammatory marker PLR(P=0.002),tumor malignancy(P=0.015),tumor metastasis(P=0.001)was associated with the prognosis of patients(P < 0.05).Multivariate Cox regression analysis showed that serum tumor markers CA199(P=0.019),anemia(P=0.021)and tumor metastasis(P=0.017)were independent factors affecting the prognosis of patients(P<0.05).Conclusion: The incidence of primary appendix tumor is low,which is an extremely rare gastrointestinal tumor.Low-grade mucinous tumor is a common pathological type of appendix tumor.The incidence population is mainly middle-aged and elderly people,with more women than men.It lacks characteristic clinical manifestations,with abdominal pain,change of defecation habits and abdominal distension as the main manifestations,and may be complicated with appendicitis and intestinal obstruction.The relationship between the treatment plan and prognosis is not clear,and further large-sample,multi-center and prospective research is needed.Serum tumor markers CEA positive,CA199 positive,anemia(male<130g/L,Female patients with<115g/L),low serum albumin(<40g/L),low prognostic nutrition index(≤ 50.1),high serum inflammatory markers(PLR)(> 188.8),high tumor malignancy,and tumor metastasis had poor prognosis.Serum tumor markers CA199,anemia,and tumor metastasis were independent prognostic factors of patients.For patients characterized by prognostic risk factors,further monitoring and intervention are required.