Clinicopathological analysis of infantile/congenital fibrosarcomas with rare histological features
10.3760/cma.j.issn.0529?5807.2019.09.007
- VernacularTitle:具有少见组织学特征的婴儿型/先天性纤维肉瘤临床病理学特征
- Author:
Guangwei QI
1
;
Jia ZHENG
;
Yangyang MA
;
Yi LOU
;
Lian CHEN
Author Information
1. 浙江省杭州市儿童医院病理科 310014
- Keywords:
Infant;
Fibrosarcoma;
Diagnosis,differential
- From:
Chinese Journal of Pathology
2019;48(9):700-704
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinicopathological features, immunohistochemical (IHC) phentotype,diagnosis and differential diagnosis of infantile/congenital fibrosarcoma (IFS/CFS) with unusual histological features. Methods Five IFS/CFS at Children′s Hospital of Fudan University from March 2014 to July 2018 were analyzed for their diagnosis and differential diagnosis. Results Two cases were males, three cases were females. The clinical manifestation of IFS/CFS was a rapidly?growing and painless mass. There were no specific radiologic features. Histologically, the tumor cells are arranged in intersecting or sheet?like patterns. There were focal hemangioma?like areas in four cases. There were also focal areas of primitive asteroid, short?spindled, and oval tumor cells in three cases. IHC study showed the tumor cells diffusely expressed TLE1(2/5), Vimentin(5/5), and WT1(3/5), in a cytoplastic pattern;they focally expressed CD34(3/5), CD31(3/5), and α?SMA(2/5). Fluorescence in situ hybridization (FISH) detected break?apart positivity of ETV6 gene. Conclusions Hemangioma?like pattern, myxoid area, and TLE1 expression is very rare in IFS/CFS. Detection of ETV6 gene break?apart by FISH is very helpful in the diagnosis and differential diagnosis of IFS/CFS.
