Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Bladder: A Case Report With 2-Years of Follow-Up and Review of Current Literature

Creti, S.; Romagnoli, Daniele; Severini, E.; Baldoni, Cristina; Bondi, A; Campli, Alfonso Di; Dicuio, Mauro; Gentile, Giorgio; Dipietro, Rosario; Saltutti, C.; Schiavina, Riccardo; Brunocilla, Eugenio; Buli, P.

doi:10.1016/j.clgc.2016.08.010

Cited by 7 publications

(5 citation statements)

References 11 publications

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“…Only 20 previous cases of bladder PEComas have been reported in the English literature, and these 2 were the twenty-first and twenty-second. [5][6][7] The clinicopathologic and imaging features of all published cases of this kind of lesion are summarized in Table 1. To our knowledge, this series was the first that reported the imaging features of bladder PEComas.…”

Section: Discussionmentioning

confidence: 99%

Bladder Perivascular Epithelioid Cell Tumor: Dynamic CT and MRI Presentation of 2 Cases With 2-year Follow-up and Review of the Literature

Xiang-ke

Guo

Zhang

2019

Clinical Genitourinary Cancer

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Section: Discussionmentioning

confidence: 99%

Bladder Perivascular Epithelioid Cell Tumor: Dynamic CT and MRI Presentation of 2 Cases With 2-year Follow-up and Review of the Literature

Xiang-ke

Guo

Zhang

2019

Clinical Genitourinary Cancer

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“…[ 3 ] Furthermore, we see variable expression of multiple melanocytic and myoid markers in bladder PEComa such as Melan-A , CD117 , S100 , CD31 , and CD34. [ 4 ] Finally, bladder PEComa might be associated with transcription factor binding to IGHM enhancer 3-rearrangement, which is not necessarily associated with poor outcome. [ 5 ]…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, they show to be round-shaped having yellowish to reddish-brown color [ Figure 2 ]. [ 4 6 17 ] The lesions are also reported to be strongly vascularized. [ 8 18 ] Finally, on cystoscopy, extrinsic mass effect on the bladder is seen in extraluminal PEComas.…”

Section: Discussionmentioning

confidence: 99%

Bladder perivascular epithelioid cell neoplasm: Review on clinical features of this rare tumor

Adriaenssens,

Lamoury,

Tilborghs

et al. 2024

Urology Annals

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Perivascular epithelioid cell neoplasm (PEComa) is a rare mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. They can arise in various localizations such as the bladder. A total of 36 case reports regarding bladder PEComa have been described in the literature. Eleven reviews regarding this tumor have been published in literature so far primarily focusing on anatomic pathology. Through these reviews, it is known that in bladder PEComa, the melanocytic marker Human Melanoma Black-45 is expressed in 100% of cases whereas variable expression can be seen in multiple other melanocytic and myoid markers such as smooth muscle actin, Melan-A, CD117, S100, CD31, and CD34. Since current reviews mainly emphasize anatomic pathology, we perform a review focusing on the clinical aspects of PEComa at the level of the clinician. A manual electronic search of the PubMed/Medline and Web of Science Core Collection databases was conducted. Search was done on (perivascular epithelioid cell neoplasms [MeSH terms]) AND (Bladder). All case reports and reviews were encompassed until March 15, 2023, to identify studies that assessed bladder PEComa. The age of presentation is relatively low with a median age of 37 years. There is a female predominance with a female/male ratio of 1.5. The tumor shows no preference in anatomical localization within the bladder. Even involvement of the bladder neck, proximal urethra, and distal ureter has been described. The clinical presentation consists in the majority of patients of symptoms related to the urinary tract such as hematuria, dysuria, passage of urine sediment, frequency, and urgency. Other symptoms include abdominal discomfort and dysmenorrhea. In clinical examination, an abdominal mass can be found based on the size and location of the tumor. Further examination usually encompasses cystoscopy due to the hematuria and radiological investigations such as ultrasound (US), computed tomography, and magnetic resonance imaging. These radiological investigations reveal a heterogeneous solid mass with clear borders. In our center, we performed a transvaginal US additionally in a patient with bladder PEComa, which was the only investigation in our patient that concluded the mass was located in the Retzius space. For treatment, transurethral resection of the bladder tumor and partial cystectomy were both described in equal numbers. The choice of treatment depends on the localization and size of the tumor. Follow-up consists of imaging, but clear guidelines on this matter are lacking. Bladder PEComa is a rare condition and usually presents itself with nonspecific symptoms. Radiological investigations will reveal the tumor, but the final diagnosis is based on cytological and immunohistochemical features. Since bladder PEComa is an entity with uncertain malignant potential, it is important to include this entity in the differential diagnosis when a patient presents with lower abdominal discomfort and lower urinary tract symptoms in combination with a mass in the pelvic region.

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“…Geographic areas of necrosis were seen in only one case described in the literature. 22 The overlying urothelium is usually tan-pink, smooth and glistening, and grossly unremarkable. Microscopically, the tumor is composed of nests, trabeculae, or sheets of epithelioid cells intermixed with areas of more spindled cells.…”

Section: Discussionmentioning

confidence: 99%

“…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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Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Bladder: A Case Report With 2-Years of Follow-Up and Review of Current Literature

Cited by 7 publications

References 11 publications

Bladder Perivascular Epithelioid Cell Tumor: Dynamic CT and MRI Presentation of 2 Cases With 2-year Follow-up and Review of the Literature

Bladder Perivascular Epithelioid Cell Tumor: Dynamic CT and MRI Presentation of 2 Cases With 2-year Follow-up and Review of the Literature

Bladder perivascular epithelioid cell neoplasm: Review on clinical features of this rare tumor

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

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