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Published in: BMC Urology 1/2023

Open Access 01-12-2023 | Cystectomy | Case Report

Transurethral en bloc resection of a bladder perivascular epithelioid cell tumor (PEComa): a case report

Authors: Shi-Ping Zeng, Yi-Fei Sun, Jun-Bing Ye, Ke Zeng, Xiao-Bin Li

Published in: BMC Urology | Issue 1/2023

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Abstract

Background

Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor with distinct histologic and immunologic features. PEComas that originate in the bladder are extremely rare clinically, with only 35 cases reported in the English literature thus far. Here, we report a case of bladder PEComa resection by transurethral en bloc resection of bladder tumor (ERBT).

Case presentation

A 66-year-old female with a history of poorly controlled type 2 diabetes with associated complications of frequent urinary tract infections presented to our hospital for a routine physical examination. Outpatient ultrasound examination revealed a strong echogenic mass of approximately 1.5 × 1.3 × 1.3 cm in size on the posterior wall of the bladder. The enhanced computed tomography and enhanced magnetic resonance imaging after admission both suggested a well-defined isolated nodular mass on the posterior wall of the bladder with significant enhancement on the enhanced scan. The tumor was successfully and completely resected by ERBT. Postoperative pathological examination and immunohistochemical results confirmed the mass was a bladder PEComa. No tumor recurrence was observed in the six-month postoperative follow-up.

Conclusion

Bladder PEComa is an extremely rare mesenchymal tumor of the urinary system. When imaging and cystoscopy reveal a nodular mass with an abundant blood supply in the bladder, PEComa should be included in the differential diagnosis of bladder tumors. Surgical resection is currently the primary option for the treatment of bladder PEComa. For a solitary, pedunculated, narrow-based, small-sized bladder PEComa, resection of the tumor by ERBT was a safe and feasible approach in our patient and may be considered for similar cases in the future.
Literature
1.
go back to reference Wu Y, Lang, Ma, Wu Z, et al. Perivascular epithelioid cell tumor of urinary bladder. Am J Clin Pathol. 2021;156(1):56–71.CrossRefPubMed Wu Y, Lang, Ma, Wu Z, et al. Perivascular epithelioid cell tumor of urinary bladder. Am J Clin Pathol. 2021;156(1):56–71.CrossRefPubMed
2.
3.
go back to reference Sarti R, Balzarro, Cerruto, Ma B, et al. Bladder perivascular epithelioid cell tumor (PEComa): a case report and literature review. Urol J. 2015;82(2):120–3.CrossRef Sarti R, Balzarro, Cerruto, Ma B, et al. Bladder perivascular epithelioid cell tumor (PEComa): a case report and literature review. Urol J. 2015;82(2):120–3.CrossRef
4.
go back to reference Xuesong H. Weiguo. Bladder Perivascular Epithelioid Cell Tumor: dynamic CT and MRI presentation of 2 cases with 2-year follow-up and review of the literature. Clin Genitourin Canc. 2019;17(5):e916–e22.CrossRef Xuesong H. Weiguo. Bladder Perivascular Epithelioid Cell Tumor: dynamic CT and MRI presentation of 2 cases with 2-year follow-up and review of the literature. Clin Genitourin Canc. 2019;17(5):e916–e22.CrossRef
5.
go back to reference Martignoni P, Zampini, Brunelli, Segala Z, et al. PEComas of the kidney and of the genitourinary tract. Semin Diagn Pathol. 2015;32(2):140–59.CrossRefPubMed Martignoni P, Zampini, Brunelli, Segala Z, et al. PEComas of the kidney and of the genitourinary tract. Semin Diagn Pathol. 2015;32(2):140–59.CrossRefPubMed
6.
go back to reference Creti R, Severini, Baldoni B, Di C, et al. Primary Perivascular Epithelioid Cell Tumor (PEComa) of the bladder: a Case Report with 2-Years of Follow-Up and Review of current literature. Clin Genitourin Canc. 2017;15(1):e79–e81.CrossRef Creti R, Severini, Baldoni B, Di C, et al. Primary Perivascular Epithelioid Cell Tumor (PEComa) of the bladder: a Case Report with 2-Years of Follow-Up and Review of current literature. Clin Genitourin Canc. 2017;15(1):e79–e81.CrossRef
7.
go back to reference Tarplin et al. Osterberg, Ec, Robinson, Bd, Herman,. Perivascular epithelioid cell tumour of the bladder. BMJ Case Rep. 2014;10(205500):2014-205500. Tarplin et al. Osterberg, Ec, Robinson, Bd, Herman,. Perivascular epithelioid cell tumour of the bladder. BMJ Case Rep. 2014;10(205500):2014-205500.
8.
go back to reference Hamza, Guo. Cc. Perivascular Epithelioid Cell Tumor of the urinary bladder: a systematic review. Int J Surg Pathol. 2020;28(4):393–400.CrossRefPubMed Hamza, Guo. Cc. Perivascular Epithelioid Cell Tumor of the urinary bladder: a systematic review. Int J Surg Pathol. 2020;28(4):393–400.CrossRefPubMed
9.
go back to reference Tee J. Chambers, Strutton. Bladder Perivascular Epithelioid Cell Tumour and Tuberous Sclerosis Complex: a Rare Association. BMJ Case Rep. 2021;14(5):2021–41635.CrossRef Tee J. Chambers, Strutton. Bladder Perivascular Epithelioid Cell Tumour and Tuberous Sclerosis Complex: a Rare Association. BMJ Case Rep. 2021;14(5):2021–41635.CrossRef
10.
go back to reference Kalkan S. Etlik, Uçmaklı. A bladder Wall Angiomyolipoma as a Manifestation of Tuberous Sclerosis: First Case Report. Case Rep Urol. 2013;398328(10):398328. Kalkan S. Etlik, Uçmaklı. A bladder Wall Angiomyolipoma as a Manifestation of Tuberous Sclerosis: First Case Report. Case Rep Urol. 2013;398328(10):398328.
11.
go back to reference Tian, Li G. Bladder PEComa: a case report and literature review. Radiol Case Rep. 2019;14(10):1293–6.CrossRef Tian, Li G. Bladder PEComa: a case report and literature review. Radiol Case Rep. 2019;14(10):1293–6.CrossRef
12.
go back to reference Folpe A, Kwiatkowski D. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010;41(1):1–15.CrossRefPubMed Folpe A, Kwiatkowski D. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010;41(1):1–15.CrossRefPubMed
13.
go back to reference Vannucchi M. Salvi, Montironi, Raspollini, Mr. TFE3 Gene Rearrangement in Perivascular Epithelioid Cell Neoplasm (PEComa) of the Genitourinary Tract. Clin Genitourin Canc. 2020;18(6):e692–e7.CrossRef Vannucchi M. Salvi, Montironi, Raspollini, Mr. TFE3 Gene Rearrangement in Perivascular Epithelioid Cell Neoplasm (PEComa) of the Genitourinary Tract. Clin Genitourin Canc. 2020;18(6):e692–e7.CrossRef
14.
go back to reference Folpe A, Mentzel, Lehr, Ha F, et al. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 2005;29(12):1558–75.CrossRefPubMed Folpe A, Mentzel, Lehr, Ha F, et al. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 2005;29(12):1558–75.CrossRefPubMed
15.
go back to reference Russell C, Buethe, Dd, Dickinson S, et al. Perivascular epithelioid cell tumor (PEComa) of the urinary bladder associated with Xp11 translocation. Ann Clin Lab Sci. 2014;44(1):91–8.PubMed Russell C, Buethe, Dd, Dickinson S, et al. Perivascular epithelioid cell tumor (PEComa) of the urinary bladder associated with Xp11 translocation. Ann Clin Lab Sci. 2014;44(1):91–8.PubMed
16.
go back to reference Williamson S, Bunde P, Lopez-Beltran M, et al. Malignant perivascular epithelioid cell neoplasm (PEComa) of the urinary bladder with TFE3 gene rearrangement: clinicopathologic, immunohistochemical, and molecular features. Am J Surg Pathol. 2013;37(10):1619–26.CrossRefPubMed Williamson S, Bunde P, Lopez-Beltran M, et al. Malignant perivascular epithelioid cell neoplasm (PEComa) of the urinary bladder with TFE3 gene rearrangement: clinicopathologic, immunohistochemical, and molecular features. Am J Surg Pathol. 2013;37(10):1619–26.CrossRefPubMed
17.
go back to reference Chen X, Yeong C, Kte L, et al. TFE3-Expressing Epithelioid Rich Perivascular Epithelioid Cell Neoplasm (PEComa) of the bladder with unusual Benign Course. Ann Clin Lab Sci. 2018;48(1):110–5.PubMed Chen X, Yeong C, Kte L, et al. TFE3-Expressing Epithelioid Rich Perivascular Epithelioid Cell Neoplasm (PEComa) of the bladder with unusual Benign Course. Ann Clin Lab Sci. 2018;48(1):110–5.PubMed
18.
go back to reference Chan A, Chan C, Chiu Y, et al. Primary perivascular epithelioid cell tumour (PEComa) of the urinary bladder. Pathology. 2011;43(7):746–9.CrossRefPubMed Chan A, Chan C, Chiu Y, et al. Primary perivascular epithelioid cell tumour (PEComa) of the urinary bladder. Pathology. 2011;43(7):746–9.CrossRefPubMed
19.
go back to reference Abou G, Nasr. Shahait, Khairallah. Bladder perivascular epithelioid cell tumours. BMJ Case Rep. 2013;200153(10):2013–0153. Abou G, Nasr. Shahait, Khairallah. Bladder perivascular epithelioid cell tumours. BMJ Case Rep. 2013;200153(10):2013–0153.
20.
go back to reference Gallioli D, Fontana, Territo, Rodriguez-Faba Ó, et al. En Bloc Versus Conventional Transurethral resection of bladder tumors: a single-center prospective randomized noninferiority trial. Eur Urol Oncol. 2022;10(05):001. Gallioli D, Fontana, Territo, Rodriguez-Faba Ó, et al. En Bloc Versus Conventional Transurethral resection of bladder tumors: a single-center prospective randomized noninferiority trial. Eur Urol Oncol. 2022;10(05):001.
21.
go back to reference Croghan S, Compton, Manecksha, Rp C, et al. En bloc transurethral resection of bladder tumors: a review of current techniques. Cuaj Can Urol Assoc. 2022;16(5):E287–E93. Croghan S, Compton, Manecksha, Rp C, et al. En bloc transurethral resection of bladder tumors: a review of current techniques. Cuaj Can Urol Assoc. 2022;16(5):E287–E93.
22.
go back to reference Palleschi P, Al, Evangelista, Silvestri R, et al. Bone metastases from bladder perivascular epithelioid cell tumor - an unusual localization of a rare tumor: a case report. J Med Case Rep. 2014;8(227):1752–947. Palleschi P, Al, Evangelista, Silvestri R, et al. Bone metastases from bladder perivascular epithelioid cell tumor - an unusual localization of a rare tumor: a case report. J Med Case Rep. 2014;8(227):1752–947.
23.
go back to reference Parfitt A Jr, Wehrli B, et al. Primary PEComa of the bladder treated with primary excision and adjuvant interferon-alpha immunotherapy: a case report. BMC Urol. 2006;6(20):1471–2490. Parfitt A Jr, Wehrli B, et al. Primary PEComa of the bladder treated with primary excision and adjuvant interferon-alpha immunotherapy: a case report. BMC Urol. 2006;6(20):1471–2490.
Metadata
Title
Transurethral en bloc resection of a bladder perivascular epithelioid cell tumor (PEComa): a case report
Authors
Shi-Ping Zeng
Yi-Fei Sun
Jun-Bing Ye
Ke Zeng
Xiao-Bin Li
Publication date
01-12-2023
Publisher
BioMed Central
Keyword
Cystectomy
Published in
BMC Urology / Issue 1/2023
Electronic ISSN: 1471-2490
DOI
https://doi.org/10.1186/s12894-023-01198-6

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