Bladder PEComa: A case report and literature review

Tian, Chuan; Li, Zhenghui; Gao, Dongsheng

doi:10.1016/j.radcr.2019.07.012

Cited by 7 publications

(10 citation statements)

References 15 publications

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“…The solid part of the tumor was weakly enhanced on the delayed phase, the liquid part was not enhanced, and the marginal part was circumferentially enhanced [4,9]. On MRI, the mass showed isosignal on T1WI and heterogeneous high signal on T2WI [4,9].…”

Section: Discussionmentioning

confidence: 98%

“…On the CT enhancement scan, the mass showed obvious uneven enhancement on the arterial phase and a rapid decrease on the venous phase. The solid part of the tumor was weakly enhanced on the delayed phase, the liquid part was not enhanced, and the marginal part was circumferentially enhanced [4,9]. On MRI, the mass showed isosignal on T1WI and heterogeneous high signal on T2WI [4,9].…”

Section: Discussionmentioning

confidence: 98%

“…The imaging features of bladder PEComa are not speci c. Ultrasonography often shows a well-de ned ovoid, nodular, hypoechoic, or isoechoic solid mass; CDFI shows abundant blood ow signals within and around the lesion [1]. The unenhanced CT scan shows a nodular, irregular, well-de ned solid or cystic mass with homogeneous or heterogeneous density, and necrosis within the mass [1,9]. On the CT enhancement scan, the mass showed obvious uneven enhancement on the arterial phase and a rapid decrease on the venous phase.…”

Section: Discussionmentioning

confidence: 99%

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

Zeng

Sun

et al. 2022

Preprint

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Background: Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor with distinct histologic and immunologic features. PEComa originating in the bladder is extremely rare clinically, with only 35 cases reported in the English literature so far. We report the first case of bladder PEComa resection by transurethral en bloc resection of bladder tumor (ERBT). Case presentation: A 66-year-old woman presented to our hospital with an ultrasound finding of a slightly strong echogenic mass of about 1.5×1.3×1.3 cm in size on the posterior wall of the bladder. The patient did not have any clinical symptoms during the disease. 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 that the mass was bladder PEComa. No tumor recurrence was observed in the six-month postoperative follow-up. Conclusions: Bladder PEComa is an extremely rare mesenchymal tumor of the urinary system. When imaging and cystoscopy revealed 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 main option for the treatment of bladder PEComa. For solitary, with pedicle, narrow-basal, small-sized bladder PEComa, resection of the tumor by ERBT is safe and feasible.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

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

Zeng

Sun

et al. 2022

Preprint

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“…As with PEComa in other sites, PEComa of the urinary system also occurs in young and middle-aged patients. However, unlike PEComa of soft tissue, skin, and bone, PEComa of the urinary system is more common in men (man: woman = 2:1), while PEComa of other sites is more common in women (man: woman = 1:7) ( 7 , 8 ). Previous research suggests that patients with urinary system PEComa often present with nonspecific symptoms, with small, asymptomatic tumors frequently being discovered incidentally.…”

Section: Discussionmentioning

confidence: 99%

Case report: Urachal perivascular epithelioid cell tumor

Liu,

Liang,

Lyu

et al. 2024

Front. Oncol.

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BackgroundUrachal tumors are rare in clinical practice, among which urachal adenocarcinoma is the most common. In this study, we report a rare case of urachal perivascular epithelioid cell tumor to improve our understanding of the disease.Case presentationA 26-year-old male patient was hospitalized for lower abdominal pain. The US showed a hypoechoic mass measuring 26mm × 18mm in the superior aspect of the bladder. MRI showed an irregular mass located anterior to the bladder roof, near the midline. The tumor exhibited hypointense on T1WI and heterogeneous hyperintense on T2WI. Additionally, contrast-enhanced T1-weighted imaging revealed obvious ring enhancement of the tumor. The patient underwent surgical resection of the urachal tumor, with subsequent pathological examination revealing a diagnosis of urachal PEComa. Following surgery, the patient underwent regular follow-up assessments, with no evidence of recurrence or metastasis observed after three and a half years.ConclusionsUrachal PEComa is a rare mesenchymal tumor that presents challenges in diagnosis through imaging and clinical symptoms. Definitive diagnosis relies on pathological and immunohistochemical analysis. Due to the rarity of urachal PEComa, prognosis assessment necessitates long-term follow-up and evaluation of more cases.

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“…PEComas are known to occur in the genitourinary tract 9-14 including the urinary bladder; however, there are only a few reported cases of primary PEComa of the urinary bladder in the literature. 15-39 Despite improvements in diagnostic modalities, PEComa remains a diagnostic challenge for radiologists as well as pathologists. Additionally, due to the limited number of cases, prognostic assessment is also difficult.…”

Section: Introductionmentioning

confidence: 99%

Perivascular Epithelioid Cell Tumor of the Urinary Bladder: A Systematic Review

Hamza

Guo

2019

Int J Surg Pathol

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Perivascular epithelioid cell tumor (PEComa) of the urinary bladder is a rare neoplasm showing distinct melanocytic and smooth muscle differentiation. We aimed to review the clinicopathologic features of bladder PEComa using all the available cases in the literature, along with 2 new cases from our database. The patients included 15 females and 15 males with a mean age of 39.2 ± 15.3 years. Painless hematuria was the most common clinical presentation. The tumors were usually well circumscribed with a mean tumor size of 4.4 ± 2.7 cm. Bladder PEComas demonstrated nests, trabeculae, or sheets of epithelioid cells with intermixed spindled cells and numerous thin-walled vessels. Immunohistochemical studies showed that the tumors were positive for HMB45 (27/27), cathepsin (4/4), SMA (20/22), and caldesmon (3/3) and were negative for pan cytokeratin (0/18) and EMA (0/4). Molecular studies revealed that PEComa was associated with the TFE3 (n = 3) and EWSR1 (n = 1) gene rearrangements. Treatment included partial cystectomy (n = 18), transurethral resection (n = 8), and radical cystectomy (n = 4). Twenty patients had no evidence of disease during a mean follow-up time of 19.4 ± 17.2 months. Two patients had recurrence, and 1 patient died of metastatic disease. In conclusion, bladder PEComas demonstrate distinct morphologic and immunohistochemical features. Although most tumors follow a benign course, a small subset may develop metastasis and cause death.

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Bladder PEComa: A case report and literature review

Cited by 7 publications

References 15 publications

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

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

Case report: Urachal perivascular epithelioid cell tumor

Perivascular Epithelioid Cell Tumor of the Urinary Bladder: A Systematic Review

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