Clinicopathological characteristics of hepatic perivascular epithelioid cell tumours
10.3760/cma.j.issn.1007-631X.2017.12.008
- VernacularTitle:肝脏血管周上皮样细胞肿瘤临床与病理学特征分析
- Author:
Yingteng MA
1
;
Danhua SHEN
Author Information
1. 100044,北京大学人民医院病理科
- Keywords:
Liver neoplasms;
Pathology,clinical;
Perivascular epithelioid cell tumor
- From:
Chinese Journal of General Surgery
2017;32(12):1007-1009
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the clinicopathological characteristics and diagnosis of hepatic perivascular epithelioid cell tumour (PEComa).Methods Retrospective analysis was made on the clinicopathological and immunohistochemical phenotype of the 9 PEComa cases.Results The average age was 39 years,male to female ratio was 1∶ 8.Tumours were grossly well-circumscribed and solid.The maximum diameter were from 1.0 cm to 9.0 cm,with the average of 3.6 cm.The tomours were of nested architecture and composed of uniform epithelioid cells with clear cytoplasm and round nuclei.A subset of tumours was dominated by spindle cells.Thick-wall blood vessels and fat cells were common.Tumour cells were positive for HMB45,MelanA,and SMA (8/8,7/7,4/4).Some of them were positive for S-100 (4/ 8).All of the 9 cases were negitive for hepatocyte (0/9).Ki-67 index of the tumours were below 10％ (9/ 9).All 9 cases were followed up from 3 months to 6 years,all were alive and recurrence free.Conclusion Hepatic PEComa is often seen in adult women with some specific histological patterns and immunophenotypes.Definite diagnosis of PEComa can be made by combining clinicopathological characteristics with a panel of immunohistochemical marks.PEComas grow slowly,often responds to surgical resection and rarely recur.
