膀胱血管周上皮样细胞瘤。

Perivascular Epithelioid Cell Tumor of Urinary Bladder.

机构信息

Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China.

Institute of Urology of PLA and Department of Urology, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China.

出版信息

Am J Clin Pathol. 2021 Jun 17;156(1):56-71. doi: 10.1093/ajcp/aqaa204.

DOI:10.1093/ajcp/aqaa204

PMID:33336255

Abstract

OBJECTIVES

To review the clinicopathologic features of perivascular epithelioid cell tumor (PEComa) of the urinary bladder.

METHODS

Seven cases of bladder PEComa were studied by light microscopy, immunohistochemistry, and fluorescence in situ hybridization (FISH).

RESULTS

In our 7 cases, 5 patients were female and 2 were male, with ages between 26 and 78 years. Patients presented with hematuria and recurrent abdominal discomfort as the main clinical symptoms. Microscopically, the epithelioid and spindle-shaped tumor cells with clear to granular eosinophilic cytoplasm were arranged in fascicular, acinar, or nested patterns. The tumor cells were positive for HMB45, melan-A, and SMA, but no TFE3 gene rearrangement was detected in any of the 7 samples by FISH. The analysis of all 35 cases from the literature and ours showed a patient age range from 16 to 78 years (mean age, 39 years), a male-to-female ratio of 1:1.3, maximal tumor diameters from 0.6 to 18.8 cm (mean, 4.5 cm). With a mean follow-up of 27 months, the recurrence, metastasis, and mortality rates were 10.7%, 10.7%, and 7.1%, respectively.

CONCLUSIONS

Bladder PEComa is extremely rare, remains a diagnostic challenge, and needs more attention. Strengthening the understanding of this tumor will improve diagnostic accuracy.

摘要

目的

回顾膀胱血管周上皮样细胞瘤（PEComa）的临床病理特征。

方法

通过光镜、免疫组织化学和荧光原位杂交（FISH）研究了 7 例膀胱 PEComa。

结果

在我们的 7 例病例中，5 例为女性，2 例为男性，年龄在 26 岁至 78 岁之间。患者以血尿和反复腹部不适为主要临床症状。镜下，上皮样和梭形肿瘤细胞具有透明至颗粒状嗜酸性细胞质，呈束状、腺泡状或巢状排列。肿瘤细胞 HMB45、melan-A 和 SMA 阳性，但 FISH 未检测到 7 例标本中任何一例 TFE3 基因重排。对来自文献和我们的所有 35 例病例的分析显示，患者年龄范围为 16 岁至 78 岁（平均年龄为 39 岁），男女比例为 1:1.3，最大肿瘤直径为 0.6 至 18.8 厘米（平均为 4.5 厘米）。平均随访 27 个月，复发率、转移率和死亡率分别为 10.7%、10.7%和 7.1%。