Bladder Perivascular Epithelioid Cell Tumor: A Novel Rare Neoplasm

Kyrou, D.; Staios, Dimitris; Papatsoris, Athanasios

doi:10.1159/000332199

Cited by 11 publications

(6 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…First, cells of melanotic neoplasms are characterized by large and conspicuous nucleoli. Second, strong S-100 protein expression is usually detected in most melanotic tumors but only in a minority of PEComas [19]. Third, the majority of PEComas also express muscle markers, whereas most melanotic tumors are negative for actin.…”

Section: Discussionmentioning

confidence: 99%

Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: a case report and review of the literature

Liu

Chen

et al. 2012

Diagn Pathol

View full text Add to dashboard Cite

Perivascular epithelioid cell neoplasms (PEComas) of the urinary bladder are extremely rare and the published cases were comprised predominantly of middle-aged patients. Herein, the authors present the first urinary bladder PEComa occurring in an adolescent. This 16-year-old Chinese girl present with a 3-year history of abdominal discomfort and a solid mass was documented in the urinary bladder by ultrasonography. Two years later, at the age of 18, the patient underwent transurethral resection of the bladder tumor. Microscopically, the tumor was composed of spindled cells mixed with epithelioid cells. Immunohistochemically, the tumor were strongly positive for HMB45, smooth muscle actin, muscle-specific actin, and H-caldesmon. Fluorescence in situ hybridization analysis revealed no evidence of EWSR1 gene rearrangement. The patient had been in a good status without evidence of recurrence 13 months after surgery. Urinary bladder PEComa is an extremely rare neoplasm and seems occur predominantly in middle-aged patients. However, this peculiar lesion can develop in pediatric population and therefore it should be rigorously distinguished from their mimickers.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1870004378817301

show abstract

Section: Discussionmentioning

confidence: 99%

Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: a case report and review of the literature

Liu

Chen

et al. 2012

Diagn Pathol

View full text Add to dashboard Cite

show abstract

“…Imaging findings are usually nonspecific but frequently show hypervascular, solid or solid-cystic masses with heterogeneous hyperintense signals on the T2-weighted images. 21,24 Imaging studies can delineate the extent of disease preoperatively that helps the surgical planning. Surgical resection is the mainstay of treatment in the management of PEComas.…”

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

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

“…3 In recent years there have also been at least 8 reported cases of perivascular epithelioid cell tumour (PEComa) of the bladder. 4 Chondroid bladder tumours are very rare, with only 2 reported cases of soft tissue chondroma, 1,2 and 5 of chondrosarcoma. 5 This diverse group of mesenchymal bladder tumours includes benign and malignant entities, highlighting the importance of their early and accurate diagnosis.…”

Section: Discussionmentioning

confidence: 99%