Congenital mesoblastic nephroma: a single-centre series

Area of Science:

• Pediatric Oncology
• Nephrology
• Surgical Pathology

Background:

• Congenital mesoblastic nephroma (CMN) is a rare renal tumor in infants.
• Historically, chemotherapy has not been considered a primary treatment for CMN.
• Recurrent or metastatic CMN carries a significant mortality rate.

Purpose of the Study:

• To review the clinical characteristics, treatment, and outcomes of congenital mesoblastic nephroma.
• To evaluate the role of adjuvant therapies in managing recurrent disease.

Main Methods:

• Retrospective case review of prospectively collected data.
• Analysis of demographics, histopathology, treatment modalities, and patient follow-up.
• Inclusion of nine patients diagnosed with congenital mesoblastic nephroma.

Main Results:

• Six males and three females presented with a median age of one month.
• The cellular subtype was most common (n=5), with two patients having mixed/classical subtypes.
• Three recurrences (two local, one pulmonary) were observed and treated with multimodal therapy; eight patients achieved disease-free survival.

Conclusions:

• Surgery is the primary treatment for congenital mesoblastic nephroma.
• Chemotherapy and radiotherapy are valuable for unresectable tumors or recurrent disease.
• The tyrosine kinase pathway presents a potential target for future therapeutic strategies in CMN.