Clinicopathologic study of primary renal hemangioblastoma
10.3760/cma.j.issn.0529-5807.2015.06.003
- VernacularTitle:原发性肾血管母细胞瘤的临床病理特征
- Author:
Xueping ZHANG
1
,
2
;
Xuan WANG
;
Jianjun WANG
;
Qin SHEN
;
Qiuyuan XIA
;
Shanshan SHI
;
Zhenfeng LU
;
Henghui MA
;
Rusong ZHANG
;
Yan HE
;
Bo YU
;
Xiaojun ZHOU
;
Qiu RAO
Author Information
1. 210002 南京大学医学院附属金陵医院 南京军区南京总医院病理科
2. 宿迁市泗阳县人民医院病理科
- Keywords:
Kidney neoplasms;
Hemangioblastoma;
Immunohistochemistry;
Diagnosis,differentail
- From:
Chinese Journal of Pathology
2015;(6):377-381
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study the clinicopathologic characteristics of primary renal hemangioblastoma.Methods The morphologic features, immunophenotype and molecular findings of 3 cases of primary renal hemangioblastoma were studied, with review of literature.Results The age of patients ranged from 43 to 57 years.There were 2 women and a man.The patients often presented with renal mass.Histologically, the tumors were surrounded by thick fibrous capsule and composed of epithelioid or spindle cells.Two cases had a prominent stromal component and the other one was rich in capillary network. Lipid vacuoles were observed in all cases.Features of hemorrhage were demonstrated in 2 cases.Capsular invasion and necrosis were seen in 1 case.Immunohistochemical study showed that the stromal cells were positive for alpha-inhibin (3/3), S-100 protein (3/3), EGFR (2/2), PAX-2 (2/2), PAX-8 (2/2) and CA9 (2/2) but negative for CKpan (2/2) and HMB45 (2/2).Focal membranous staining for CD10 (3/3) was noted.No VHL gene mutations or chromosome 3p deletion were detected in the 2 cases studied. Conclusions Renal hemangioblastoma shows distinctive morphologic appearance with a wide range of variation.The unexpected positive staining for PAX-2, PAX-8 and CD10 in renal hemangioblastoma needs to be aware.Immunohistochemical study may be helpful in differential diagnosis of these renal tumors.