Perivascular epithelioid cell tumour (PEcoma) of the bladder. Report of two cases and review of the literatura

Abstract

Description: We present two cases of bladder PEComa:1- A 43-year-old man with haematuria and urinary frequency was diagnosed with a subcentimeter, non-papillary, exophytic tuberous lesion via cystoscopy, which was completely removed by transurethral resection. 2- A 36-year-old woman was incidentally diagnosed with a bladder lesion during a gynaecological ultrasound, having had symptoms of overactive bladder previously. Cystoscopy revealed a 2 cm solid lesion near the right ureteral orifice, compatible with bladder PEComa pT2G3, with a healthy base in re-resection. Partial cystectomy was performed.

Relevance: PEComa are a family of mesenchymal tumours composed of perivascular epithelioid cells that exhibit melanocytic and smooth muscle markers and can appear in almost any organ of the body. Appearance in bladder is extremely rare, with very few cases reported in the literature. Diagnosis is anatomopathological, so it is important to distinguish from other bladder tumours.

Clinical implications: PEComa may be associated with Tuberous Sclerosis and, although most are benign, malignant lesions have been reported. They do not usually present specific clinical or radiological features, being the definite diagnosis histological.

Conclusions: Bladder PEComa are a rare entity that should be considered in the differential diagnosis of other bladder tumours.