Choroid Plexus Carcinomas With TP53 Germline Mutations: Management and Outcome

Area of Science:

• Pediatric Oncology
• Cancer Genetics
• Tumor Biology

Background:

• Choroid plexus carcinomas (CPCs) are rare pediatric neoplasms.
• CPCs are frequently linked to Li-Fraumeni syndrome (LFS), characterized by TP53 germline mutations.

Purpose of the Study:

• To analyze management strategies for CPCs.
• To investigate the impact of TP53 germline mutations on treatment outcomes.
• To evaluate the role of surgery and radiotherapy in CPC patient survival.

Main Methods:

• Retrospective analysis of 12 CPC cases.
• Assessment of surgical resection extent and radiotherapy use.
• TP53 germline mutation analysis.
• Kaplan-Meier curves and log-rank tests for progression-free survival (PFS) evaluation.

Main Results:

• TP53 germline mutations were identified in 8 out of 12 CPC patients.
• Patients with TP53 germline mutations who underwent gross total resection (GTR) without radiotherapy demonstrated significantly improved survival (P=0.012).
• Patients without TP53 germline mutations also showed a survival advantage with GTR, though not statistically significant (P=0.063).

Conclusions:

• Radiotherapy may not be beneficial for CPC patients with TP53 germline mutations.
• Gross total resection is a critical factor for favorable outcomes in CPC.
• Findings support tailored therapeutic strategies based on TP53 mutation status.