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Published on: December 1, 2013
Primary bladder perivascular epithelioid cell tumour in an early adolescent
Francesca Gigola1,2, Charlotte Mussini3, Geraldine Héry4
1Paediatric Surgery Unit, Université Paris-Saclay, Assistance Publique-Hôpitaux de Paris, Hopital Bicetre, Le Kremlin-Bicetre, Île-de-France, France francesca.gigola@unifi.it.
Insights
Primary bladder perivascular epithelioid cell tumours (PEComas) are rare. This study highlights a PEComa case in an adolescent male with unusual symptoms, emphasizing PEComa diagnosis in pediatric patients.
Area of Science:
- Urology
- Oncology
- Pathology
Background:
- Primary bladder perivascular epithelioid cell tumours (PEComas) are rare neoplasms.
- PEComas typically affect individuals in their third or fourth decade of life.
- Diagnosis relies on radiological and histopathological findings, with surgical excision as the primary treatment.
Purpose of the Study:
- To report a rare case of primary bladder PEComa in an adolescent male.
- To highlight the importance of considering PEComa in pediatric patients with specific symptoms.
- To discuss the diagnostic and clinical implications of this rare tumor presentation.
Main Methods:
- Case report presentation.
- Review of clinical presentation, radiological imaging, and histopathological findings.
- Discussion of diagnostic considerations and treatment approaches.
Main Results:
- A primary bladder PEComa was diagnosed in an early adolescent male.
- The patient presented with macroscopic haematuria and a hypervascular bladder mass.
- This presentation is atypical given the typical age demographic for PEComas.
Conclusions:
- Primary bladder PEComa can occur in pediatric patients.
- Macroscopic haematuria and hypervascular bladder mass are key indicators for considering PEComa.
- Early consideration of PEComa is crucial for timely diagnosis and management in younger populations.
Abstract:
Primary bladder perivascular epithelioid cell tumours (PEComas) are rare tumours that typically present in the third or fourth decade of life. Radiological and histopathological findings play a crucial role in establishing the diagnosis, and treatment is primarily based on surgical excision. We present the case of a primary bladder PEComa in an early adolescent male presenting with macroscopic haematuria and a hypervascular bladder mass. These peculiar symptoms should prompt consideration of the rare diagnosis of PEComa, even in paediatric patients.
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