Perivascular Epithelioid Cell Tumor of the Urinary Bladder: A Systematic Review. (June 2020)

• Record Type: Journal Article
• Authors: Hamza, Ameer; Guo, Charles C.
• Is Part Of: International journal of surgical pathology. Volume 28:Number 4(2020)
• Page Start: 393
• Page End: 400
• Publication Date: 2020-06
• Subjects: urinary bladder -- perivascular epithelioid cell neoplasm -- TFE3 gene -- HMB45; Pathology, Surgical -- Periodicals; 617.07
• DOI: 10.1177/1066896919895810
• Languages: English
• ISSNs: 1066-8969

Abstract

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.