Clinicopathologic Features And Prognostic Factors In Desmoid-type Fibromatosis

Objective:To study the clinicopathological features of the desmoid-type fibromatosis and to investigate the related factors that affect the prognosis of the disease.Methods:Clinical data of 112 cases of desmoid-type fibromatosis diagnosed by surgical resection from January 2005 to January 2015 in Chinese PLA General hospital were collected and summarized including the gender,age,symptoms,number of tumors,and the maximum diameter of the tumors.Immunohistochemical staining,including ?-catenin,vimentin,h-caldesmon,desmin,NF,SMA,CD 117,Dog-1 were performed to analyze the clinicopathological features and immunophenotype of the disease.All 112 patients were evaluated and followed up.Immunohistochemical staining of ?-catenin,p53,Ki-67 were performed in the follow-up period in 107 cases.A retrospective analysis of the experimental data with the follow-up information,the pathological parameters,the expression of immunohistochemistry was carried out by statistical software to investigate the factors affecting the recurrence of tumors.Results:1.A total of 112 patients between the ages of 1 to 78 years were studied.Based on the 112 patients,51 patients(46%)were male and 61(54%)were female,the average age was 27.6 years.The percentage of female patients in the 20?40 years age group(68.8%)was significantly more than that of the male patients(31.2%),but there was no significant difference in other age groups.Extra-abdominal lesions comprised 50%(56 cases)of all desmoid type fibromatoses.There were 31 cases(27.7%)interabdominal fibromatosis,and 25 cases(22.3%)in the abdominal wall.The clinical manifestations were not specific,and the masses with painless slow growth were the main manifestation(56 cases,50%).12 cases showed multicenter growth(10.7%),and there was no statistical significance to the location of the disease.The average diameter of the tumors was 8.9cm(2.8-30cm).The boundary of intraperitoneal tumor was clearer than the abdominal one.Microscopic examination showed that the tumor cells were composed of slender and uniformly long spindle cells.5 cases were accompanied by mucinous degeneration,and 66 cases(58.9%)showed an infiltrating growth,46 cases(41.1%)showed a significant growth.All the 112 cases showed vimentin expression,97 cases showed SMA expression,17 cases showed desmin expression,15 cases showed h-caldesmon expression.The expression of ?-catenin was found in 77 cases,and there was no difference of the expression between different parts.2.107 patients were followed up.The median follow-up time was 68 months(38-132 months).The total recurrence rate of 107 patients was 47.7%(51/107),the recurrence time was 2-70 months after operation,and the median recurrence time was 12 months.Univariate analysis showed that the age of onset than 20 years old(P=0.0160),tumor located outside the abdomen(P =0.007),positive margin(P=0.000),positive expression of P-catenin(P=0.013)and p53 protein positive expression(P=0.001)were the factors indicating poor prognosis.Multivariate analysis showed that positive margins(P=0.000),lesion positive margins(P=0.000)and positive expression of p53 protein(P=0.000)were independent prognostic factors for postoperative recurrence.The recurrence rate of the tumor located outside the abdomen was even higher(P=0.003).Conclusion:1.Fibromatosis is more common in women of childbearing age than men,but there is no significant difference of gender ratio in other age groups.There are many clinical manifestations of fibromatosis,but there's a lack of specificity.Tumor appearance examination and microscopic examination can be divided into aggressive or pushy manifestations.Some cases are accompanied by mucous degeneration,which need to be identified with a variety of tumors or lesions.There is no difference in the expression of?-catenin in all types of fibromatosis.2.Positive surgical margin,p53 expression are risk factors for the recurrence of the tumor.The recurrence rate of the tumor located outside the abdomen is higher,further treatment after operation is recommended.However,gender,age,tumor size,and nuclear mitosis cannot be considered as risk factors when we evaluate a prognostic impact.According to this study the expression of ?-catenin wasn't an independent prognostic risk factor.