Multilocular cystic renal tumor in children: radiologic-pathologic correlation

Area of Science:

• Pediatric Nephrology
• Surgical Pathology
• Oncology

Background:

• Multilocular cystic renal tumor encompasses two distinct entities: cystic nephroma and cystic partially differentiated nephroblastoma (CPDN).
• These lesions are histologically different but macroscopically indistinguishable, affecting different age groups (children for CPDN, adult women for cystic nephroma).
• While cystic nephroma is benign, CPDN can recur locally, necessitating accurate diagnosis despite similar presentation.

Purpose of the Study:

• To review the distinct histological features, clinical presentations, and management of multilocular cystic renal tumors.
• To highlight the differential diagnosis of pediatric cystic renal masses.
• To emphasize the importance of surgical management for these tumors.

Main Methods:

• Histopathological review of multilocular cystic renal tumors.
• Analysis of clinical data including age, sex, and outcomes.
• Comparison with other pediatric cystic renal masses.

Main Results:

• Cystic nephroma lacks blastemal elements, while CPDN contains embryonal cells in its septa.
• Multilocular cystic tumors are primarily diagnosed in early childhood (CPDN) or adult women (cystic nephroma).
• Nephrectomy is curative for both, but CPDN has a risk of local recurrence.

Conclusions:

• Cystic nephroma and CPDN, though distinct, share radiological features and are managed similarly via nephrectomy.
• Accurate pathological diagnosis is crucial for understanding prognosis and potential recurrence, especially for CPDN.
• Differential diagnosis with other pediatric cystic renal masses is essential for appropriate treatment stratification.