原发性肾脏血管母细胞瘤临床病理特征1例

Clinicopathological features of one case with primary renal hemangioblastoma

原发肾脏血管母细胞瘤属于肾脏良性间叶源性肿瘤,发病罕见。大多数为散发病例,部分病例报道与VHL综合征相关。本文报道1例散发性原发肾脏血管母细胞瘤,镜下见瘤细胞胞浆丰富,呈上皮样形态,网状排列,血管丰富,免疫组化表达a-inhibin、VIM及NSE。同时复习整理相关文献资料,增加医师对原发肾脏血管母细胞瘤临床病理特点的了解,从而为患者选择更优更合适的治疗方案。临床疑似病例可行术前穿刺活检明确诊断,优先选择部分肾切除术,以保留更多肾功能。

Renal hemangioblastoma is a benign mesenchymal tumor of the kidney with rare onset.Most were sporadic cases and some were reported to be associated with VHL syndrome.This paper reported a case of sporadic primary renal hemangioblastoma.Microscopically,tumor cells showed abundant cytoplasm,as epithelioid morphology,and were arranged as a net with abundant blood vessels.Immunohistochemistry showed positive expressions of a-inhibin,VIM and NSE.The relevant literature was reviewed to increase doctors'understanding of the clinicopathological characteristics of primary renal hemangioblastoma,so as to choose a better and more appropriate treatment plan for patients.Preoperative biopsy can be used to confirm the diagnosis,and partial nephrectomy is preferred to preserve more renal function.