Perivascular epithelioid cell tumour of the bladder

Tarplin, Sarah; Osterberg, E. Charles; Robinson, Brian D.; Herman, Michael; Rosoff, James S.

doi:10.1136/bcr-2014-205500

Cited by 7 publications

(4 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Even involvement of the bladder neck, proximal urethra, and distal ureter has been described. [ 8 9 10 ] A wide overview of those characteristics is presented in Table 1 .…”

Section: Discussionmentioning

confidence: 99%

“…In four of those cases, the subsequent partial cystectomy was performed short after TURBT failed to remove the bladder PEComa completely. [ 10 12 22 23 ] In the fifth case, 6 months after TURBT, an infiltrative mass invaded the entire thickness of the bladder wall and a metastatic nodule in the sigmoid colon was found. Therefore, radical cystectomy was carried out and the nodule in the sigmoid colon was synchronously resected.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

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

Adriaenssens,

Lamoury,

Tilborghs

et al. 2024

Urology Annals

View full text Add to dashboard Cite

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.

show abstract

“…Even involvement of the bladder neck, proximal urethra, and distal ureter has been described. [ 8 9 10 ] A wide overview of those characteristics is presented in Table 1 .…”

Section: Discussionmentioning

confidence: 99%

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

View full text Add to dashboard Cite

show abstract

“…The PEComa family includes angiomyolipomas, lymphangioleiomyosarcomas, clear-cell sugar tumors, and other perivascular epithelioid cell-derived tumors [3,4]. PEComa has been reported in many organs and tissues of the body, such as the lung, liver, pancreas, breast, uterus, vulva, ovary, broad ligament, heart, skull base, and soft tissues [6,7]. In the urinary system, PEComa is most commonly found in the kidney, while PEComa originating in the bladder is extremely rare, with only 35 cases reported in the English literature so far.…”

Section: Discussionmentioning

confidence: 99%

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

Zeng

Sun

et al. 2022

Preprint

View full text Add to dashboard Cite

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.

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

Perivascular epithelioid cell tumour of the bladder

Cited by 7 publications

References 18 publications

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

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

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

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

Contact Info

Product

Resources

About