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

Lijuan Yin1, Hong Bu1, Min Chen1, Jianqun Yu2, Hua Zhuang3, Jie Chen1 and Hongying Zhang1*

Author Affiliations

1 Department of Pathology, West China Hospital, Sichuan University, Guoxuexiang 37, 610041, Chengdu, Sichuan, China

2 Department of Radiology, West China Hospital, Sichuan University, 610041, Chengdu, Sichuan, China

3 Department of Ultrasound, West China Hospital, Sichuan University, 610041, Chengdu, Sichuan, China

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Diagnostic Pathology 2012, 7:183  doi:10.1186/1746-1596-7-183

Published: 31 December 2012


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.

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Perivascular epithelioid cell neoplasms; Urinary bladder; Adolescent