PMID- 33336255 OWN - NLM STAT- MEDLINE DCOM- 20210826 LR - 20210826 IS - 1943-7722 (Electronic) IS - 0002-9173 (Linking) VI - 156 IP - 1 DP - 2021 Jun 17 TI - Perivascular Epithelioid Cell Tumor of Urinary Bladder. PG - 56-71 LID - 10.1093/ajcp/aqaa204 [doi] AB - 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. CI - (c) American Society for Clinical Pathology, 2020. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com. FAU - Wu, You-Li AU - Wu YL AD - Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China. FAU - Lang, Lang AU - Lang L AD - Institute of Urology of PLA and Department of Urology, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China. FAU - Ma, Qiang AU - Ma Q AD - Department of Pathology, Daping Hospital, Third Military Medical University (Army Medical University), Chongqing, China. FAU - Wu, Feng AU - Wu F AD - Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China. FAU - Zhang, Yu AU - Zhang Y AD - Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China. FAU - Chen, Wei AU - Chen W AD - Department of Radiology, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China. FAU - Yan, Xiao-Chu AU - Yan XC AD - Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China. FAU - Duan, Guang-Jie AU - Duan GJ AUID- ORCID: 0000-0002-5564-4394 AD - Institute of Pathology and Southwest Cancer Center, Southwest Hospital, Third Military Medical University (Army Medical University), Chongqing, China. LA - eng PT - Journal Article PT - Review PL - England TA - Am J Clin Pathol JT - American journal of clinical pathology JID - 0370470 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Male MH - Middle Aged MH - Perivascular Epithelioid Cell Neoplasms/*pathology MH - Urinary Bladder Neoplasms/*pathology OTO - NOTNLM OT - Bladder OT - Diagnosis OT - FISH OT - PEComa OT - Perivascular epithelioid cell tumor OT - Prognosis OT - TFE3 OT - Treatment EDAT- 2020/12/19 06:00 MHDA- 2021/08/27 06:00 CRDT- 2020/12/18 05:54 PHST- 2020/12/19 06:00 [pubmed] PHST- 2021/08/27 06:00 [medline] PHST- 2020/12/18 05:54 [entrez] AID - 6041275 [pii] AID - 10.1093/ajcp/aqaa204 [doi] PST - ppublish SO - Am J Clin Pathol. 2021 Jun 17;156(1):56-71. doi: 10.1093/ajcp/aqaa204.