Clinical Diagnosis And Treatment Of 49 Cases Of Primary Appendiceal Tumor

Objective:The purpose of this study is to provide more accurate and detailed theoretical basis for clinical diagnosis and treatment of primary appendiceal tumors through the discussion of clinicopathological characteristics,diagnostic methods,treatment options and prognosis of 49 cases of primary appendiceal tumor.Methods:Through reviewing the clinical data of patients with appendiceal diseases admitted to the China-Japan Union Hospital of Jilin University from January 2014 to March 2019,49 patients,pathologically diagnosed primary appendiceal tumors and met the inclusion criteria,were included in this retrospective clinical analysis.Clinical case data mainly includes age,sex,clinical manifestation,supplementary examination(blood routine,serum tumor markers,abdominal B-ultrasound,abdominal CT,fibrocolonoscopy),surgical method,postoperative pathological results,adjuvant chemotherapy program and prognosis.Patients or their families were mainly contacted by telephone for follow-up,and others were followed up by out-patient review.Survival is calculated in months from the operation date to the date of death or the follow-up deadline(December 30,2019).The collected data were statistically analyzed by software SPSS 25.0.The Kolmogorov-Smirnov test was used to test the normality of the data,in which the data that obey the normal distribution were described in the form of " average±standard deviation",and the data that did not obey the normal distribution were described in the form of median;the proportion was expressed in percentage(%).Kaplan-Meier method was used to calculate survival rate and draw survival curve;Log-rank method was used to univariate analysis of prognostic factors,and P < 0.05 indicated that the difference was statistically significant.Results:A total of 49 cases of primary appendiceal tumor were included in this study,accounting for 1.1% of 4362 pathological specimens of appendicectomy in the same period.Among them,23 cases(46.9%)were low-grade appendiceal mucinous tumors,2 cases(4.1%)were high-grade appendiceal mucinous tumors,4 cases(8.2%)were mucinous adenocarcinomas,7 cases(14.3%)were colonic adenocarcinomas,10 cases(20.4%)were neuroendocrine tumors,2 cases(4.1%)were appendiceal adenomas,and 1 cases(2.0%)was appendiceal stromal tumors.There were 17 males(34.7%)and 32 females(65.3%)with a sex ratio of 1:1.9.The age ranged from 12 to 85 years,with an average age of 58.55±18.439 years.The first symptoms were right lower abdominal pain,right lower abdominal mass,abdominal distension and other incidental abdominal and pelvic surgery in 29 cases,7 cases,2 cases and 11 cases,accounting for 59.2%,14.3%,4.1% and 22.4% respectively.49 patients with primary appendiceal tumor were examined by blood routine examination.The leukocyte count of 27 patients(55.1%)was in the normal range,and that of 22 patients(44.9%)was increased.31 patients(63.3%)were examined by serum tumor markers.The most important related tumor markers included CEA,NSE,CA72-4 and CA19-9.14 patients were positive for any one of them,accounting for 45.2%(14/31).8 patients had increased CEA,accounting for 25.8%(8/31),6 cases had increased NSE,accounting for 19.4%(6/31),5 cases had increased CA72-4,accounting for 16.1%(5/31),and 3 cases had increased CA19-9,accounting for 9.7%(3/31).19 patients(38.8%)underwent abdominal B-mode ultrasonography,and the imaging findings were appendix thickening,mixed echo mass in the right lower abdomen,no abnormalities in 8 cases,10 cases and 1 cases,accounting for 42.1%(8/19),52.6%(10/19)and 5.3%(1/19)respectively.32 patients(65.3%)were examined by abdominal CT plain scan.The imaging findings showed that there were 12 cases,8 cases,5 cases and 7 cases respectively with thickened appendix,mass shadow of ileocecal or pelvic soft tissue,local cystic dilatation of appendix and no abnormality,accounting for 37.5%(12/32),25.0%(8/32),15.6%(5/32)and 21.9%(7/32).3 patients(6.1%)underwent abdominal CT plain scan and enhanced examination,of which 2 cases showed oval cystic density shadow in ileocecal region,slight uneven enhancement in patchy region,slight enhancement in lesion edge,and postoperative pathology of low-grade appendiceal mucinous tumor(low-grade malignancy);of which 1 case showed long tubular cystic density shadow in ileocecal region,local wall nodule,cyst wall and wall nodule were obviously strong,the right edge of the liver was locally compressed and uneven,and the postoperative pathology was low-grade appendiceal myxoma with pseudomyxoma peritonei.9 patients(18.4%)were examined by fibro colonoscopy,of which 5 cases were normal in ileocecal and appendiceal opening,3 cases were bulged mass near appendiceal opening,1 case was ulcerative lesion near appendiceal opening,biopsy pathology showed that a small number of abnormal cells were seen,and local glands were low-grade intraepithelial neoplasia.Combined with the history,physical signs and auxiliary examination(serological examination,abdominal B-ultrasound,abdominal CT plain scan+enhancement,colonoscopy),no patient in this group was diagnosed as appendiceal tumor before operation,and 20 cases,13 cases,2 cases,1 case,2 cases and 11 cases were diagnosed as acute appendicitis,right lower abdominal mass,appendiceal mucocele,periappendiceal abscess,peritoneal effusion and other abdominal and pelvic diseases,accounting for 40.8%,26.5%,4.1%,2.0%,4.1% and 22.5% respectively.Among 49 cases of primary appendiceal tumor,31 cases(63.3%)underwent simple appendectomy;6 cases(12.3%)underwent appendectomy + combined organ resection and / or tumor cell reduction;3 cases(6.1%)underwent ileocecal resection + combined organ resection and / or tumor cell reduction;8 cases(16.3%)underwent right hemicolectomy;1 case(2.0%)underwent right hemicolectomy + combined organ resection and / or tumor cell reduction.The follow-up deadline of this clinical study is December 30,2019.49 patients with primary appendiceal tumor were followed up for 6-71 months,the median follow-up time was 28.0 months(95%CI:20.4-35.6).5 patients died,and all of them were appendiceal adenocarcinoma(2 cases of mucinous-type and 3 cases of colonic-type).11 cases of appendiceal adenocarcinoma(mucinous-type and colonic-type)were followed up for the shortest 8 months and the longest 71 months,and the cumulative survival rates in 1 year,3 years and 5 years were 90.9%,40.4% and 40.4%,respectively.Univariate analysis showed that there was no significant difference in survival rate of appendiceal adenocarcinoma between extended resection(right hemicolectomy and ileocelectomy)and simple appendectomy(P = 0.5).Conclusion:1.The clinical manifestations of primary appendiceal tumor are lack of specificity,and the preoperative diagnosis is difficult.Surgery is the main diagnosis and treatment method.2.Mucinous tumors of the appendix include low-grade appendiceal mucinous neoplasm,high-grade appendiceal mucinous neoplasm and appendiceal mucinous adenocarcinoma,unified,standardized and accurate pathological diagnosis is of great significance for clinical treatment and prognosis.3.Low grade appendiceal mucinous neoplasm has a low grade of malignancy,which has a standard name and classification,and is more accurate in the diagnosis and treatment of the disease.4.The first symptom of the appendiceal neuroendocrine neoplasms is pain in the right lower abdomen,which is easy to be misdiagnosed as acute appendicitis before operation,and most of them have a good prognosis.5.The prognosis of appendiceal adenocarcinoma is worse than that of other pathological types.