Analysis of clinicopathologic features in 55 cases of desmoid-type fibromatosis
10.13315/j.cnki.cjcep.2018.06.011
- VernacularTitle:韧带样型纤维瘤病55例临床病理分析
- Author:
Ling-Li YAO
1
;
Zong-Ke CHEN
;
Ping GU
;
En-Yun XIE
Author Information
1. 中国科学技术大学附属第一医院(安徽省立医院)病理科
- Keywords:
desmoid-type fibromatosis;
pathological diagnosis;
differential diagnosis
- From:
Chinese Journal of Clinical and Experimental Pathology
2018;34(6):636-640
- CountryChina
- Language:Chinese
-
Abstract:
Purpose To explore the clinicopathologic fea-tures, diagnosis and differential diagnosis of desmoid-type fibro-matosis (DTF). Methods The clinicopathological data of 55 cases of DTF were retrospectively analyzed and followed up. Re-sults Among 55 cases of DTF, 18 cases were male and 37 cases were female. Median age was 33 years old (range from 6-71). 19 cases were occurred in abdominal wall, 23 cases were extra-abdomen, 13 cases in intra-abdomen and mesentery. DTF was composed of proliferating spindle fibroblasts and a number of collagen fibers. The cell showed no obvious atypia and the mitot-ic figures were rare. Immunohistochemical results showed posi-tive expression of vimentin, partial expression of SMA, desmin. The positive expression of β-catenin was located in nucleus of DTF cells. CD34, CD117, DOG1, S-100 were negatively ex-pressed. Ki-67 proliferation index was low. The effect of surgical resection was good for DTF. After 2-82 months follow-up, 17 ca-ses recurred and the remainders were disease-free survival. Con-clusion DTF is a rare intermediate tumor of soft tissue with no specificity of clinical and imaging features, which should be dif-ferentiated with a variety of benign and malignant diseases. The nuclear expression of β-catenin acts an important role in diagno-sis and differential diagnosis of DTF.
