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Multicystic congenital mesoblastic nephroma
1Servicio de Patología, Hospital de Niños Superiora Sor María Ludovica, 1900 La Plata, Argentina.
International Journal of Surgical Pathology
|April 3, 2002
Summary
This report details a rare congenital mesoblastic nephroma cellular variant in a neonate, presenting as a multicystic kidney tumor. The patient recovered well after surgery, highlighting the importance of accurate diagnosis for this pediatric kidney tumor.
Area of Science:
- Pediatric Oncology
- Nephropathology
- Surgical Pathology
Background:
- Congenital mesoblastic nephroma (CMN) is a rare kidney tumor in neonates.
- Cellular variants of CMN can present diagnostic challenges.
- Multicystic presentation is uncommon for CMN.
Observation:
- A 1-week-old neonate presented with a multicystic kidney tumor.
- The tumor exhibited extensive pseudocystic cavitation and infiltration of renal tissue.
- Histologically, the tumor comprised stellate and spindle cells positive for vimentin and smooth muscle actin.
Findings:
- The tumor was diagnosed as a congenital mesoblastic nephroma, cellular variant.
- Immunohistochemistry confirmed tumor cell expression of vimentin and smooth muscle actin.
- The patient remained disease-free 16 years post-resection.
Implications:
- This case underscores the importance of considering CMN cellular variant in neonates with multicystic kidney masses.
- Accurate differential diagnosis is crucial, distinguishing it from other pediatric cystic renal tumors.
- Long-term survival is achievable with appropriate surgical management.