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海绵体脂肪瘤样血管外皮细胞瘤:一例报告并文献复习

Lipomatous hemangiopericytoma of the corpus spongiosum: a case report and review of the literature.

作者信息

Liu Chuang, Ding De-Gang, Shan Lei, Duan Xiao-Yu, Ji Tong-Yu, Zhou Rui-Jin, Du Tao

机构信息

Department of Urology, Henan Provincial People's Hospital Zhengzhou, China.

出版信息

Int J Clin Exp Pathol. 2019 Jan 1;12(1):212-216. eCollection 2019.

Abstract

BACKGROUND

Hemangiopericytoma (HPC) is an uncommon soft tissue tumor arising from pericytes. The urogenital system is rarely affected.

METHODS

The review of the literature used the PubMed database which was searched up to March 2015.

RESULTS

Herein, we report the first case of lipomatous HPC of the corpus spongiosum in a 37-year-old man in China. The lesion presented as a quickly growing mass. Contrast enhanced CT showed a heterogeneous fatty mass with a multifocal enhancing soft-tissue component. Microscopically, the neoplasm was composed of spindle cells, a mature fat component and collagenous stroma. The mitotic index was low at 1 to 3 mitoses per 10 high-power fields. Immunohistochemically, STAT6, Bcl-2, CD99 and CDK4 were positive; CD34 and SMA were negative. The mature adipocytes were positive for S-100. Ki-67 expression was found in approximately 5% of the tumor cells. Surprisingly, there was a diffuse and strong nuclear expression of MDM2, but, no amplification of MDM2 was demonstrated by FISH. An adequate excision of the tumor was performed.

CONCLUSION

No local recurrence or distant metastases occurred during the 18-month follow-up. In view of the unpredictable biological behavior of this tumor, a long follow-up period is mandatory.

摘要

背景

血管外皮细胞瘤(HPC)是一种起源于周细胞的罕见软组织肿瘤。泌尿生殖系统很少受累。

方法

文献回顾使用了截至2015年3月搜索的PubMed数据库。

结果

在此,我们报告中国一名37岁男性阴茎海绵体脂肪瘤样血管外皮细胞瘤的首例病例。病变表现为快速生长的肿块。增强CT显示为不均匀的脂肪肿块,伴有多灶性强化的软组织成分。显微镜下,肿瘤由梭形细胞、成熟脂肪成分和胶原性间质组成。有丝分裂指数较低,每10个高倍视野有1至3个有丝分裂象。免疫组化显示,信号转导和转录激活因子6(STAT6)、B细胞淋巴瘤-2(Bcl-2)、CD99和细胞周期蛋白依赖性激酶4(CDK4)呈阳性;CD34和平滑肌肌动蛋白(SMA)呈阴性。成熟脂肪细胞S-100呈阳性。约5%的肿瘤细胞发现有Ki-67表达。令人惊讶的是,MDM2呈弥漫性强核表达,但荧光原位杂交(FISH)未显示MDM2扩增。对肿瘤进行了充分切除。

结论

在18个月的随访期间未发生局部复发或远处转移。鉴于该肿瘤不可预测的生物学行为,必须进行长期随访。

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