Tumor de células epitelioides perivasculares (PEComa) vesical: presentación de dos casos y revisión de la literatura
Cargando...
Fecha
2025-08-13
Título de la revista
ISSN de la revista
Título del volumen
Editor
Sociedad Mexicana de Urologia. Colegio de Profesionistas A.C.
Resumen
Descripción: presentamos dos casos de PEComa vesical: hombre de 43 años que consulta por hematuria y polaquiuria y es diagnosticado mediante cistoscopia de una lesión tuberosa excrecente subcentimétrica no papilar que se reseca completamente mediante resección transuretral. Mujer de 36 años con hallazgo de lesión vesical durante ecografía ginecológica, con clínica de vejiga hiperactiva. En cistoscopia se objetiva lesión sólida de 2 cm junto al orificio ureteral derecho, siendo diagnóstica de PEComa vesical pT2G3, con base sana en re-resección. Se decide realizar cistectomía parcial. Relevancia: los PEComa son una familia de tumores mesenquimales formados por células epitelioides perivasculares que muestran diferenciación melanocítica y de músculo liso y pueden localizarse en casi cualquier parte del cuerpo. Su localización vesical es extremadamente rara, habiendo muy pocos casos en la literatura. El diagnóstico es anatomo-patológico y es importante el diagnóstico diferencial con otros tumores vesicales. Implicaciones clínicas: pueden asociarse a la esclerosis tuberosa y, aunque la mayoría son benignos, se han descrito lesiones malignas. Debido a que no muestran características clínicas ni radiológicas específicas, su diagnóstico es histológico. Conclusiones: los PEComa vesicales son una entidad poco frecuente a tener en cuenta en el diagnóstico diferencial con otros tumores vesicales.
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.
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.
Palabras clave
Neoplasias de células epitelioides perivasculares
Neoplasias de vejiga urinaria
Angiomiolipoma
Perivascular epithelioid cell neoplasms
Urinary bladder neoplasms
Angiomyolipoma
Neoplasias de vejiga urinaria
Angiomiolipoma
Perivascular epithelioid cell neoplasms
Urinary bladder neoplasms
Angiomyolipoma
Descripción
Materias
Cita
Gutiérrez-Zurimendi, G., Álvarez-Hornia Pérez, E., Maldonado de Sasia, A., Gartzia Rivero, A., Mosteiro González, L., & Urresola Olabarrieta, A. (2025). Tumor de células epitelioides perivasculares (PEComa) vesical: presentación de dos casos y revisión de la literatura. Revista Mexicana de Urologia, 85(3). https://doi.org/10.48193/1QCY1J81