Treatment of Choroid Plexus Carcinoma in the Pediatric Population
Maximal safe surgical resection followed by adjuvant therapy is the recommended treatment for choroid plexus carcinoma in pediatric patients, with gross total resection significantly improving overall survival.
Diagnosis and Initial Management
- Diagnosis requires comprehensive staging workup with MRI of brain and spine
- CSF cytology should be obtained (not earlier than 2-3 weeks after surgery) to assess for dissemination 1
- Molecular and genetic testing should be performed to identify potential genetic alterations 1
- Postoperative MRI should be performed to evaluate the extent of resection 1
Surgical Management
- Maximal safe surgical resection is the cornerstone of treatment 2
- Gross total resection (GTR) is strongly associated with improved overall survival (OS) and should be the primary goal 2
- A second-look surgery should be considered when residual tumor is demonstrated on postoperative MRI and GTR is achievable 1
- The surgical approach must be carefully planned due to the highly vascular nature of these tumors and their location within the ventricular system
Adjuvant Therapy
Radiation Therapy
- Postoperative conformal radiotherapy with doses up to 59.4 Gy is recommended for children older than 18 months 1
- For children between 12-18 months, a reduced dose of 54 Gy is recommended 1
- In case of CSF or spinal dissemination, craniospinal irradiation (CSI) of 36 Gy is recommended with a boost up to 45-54 Gy on focal lesions 1
- Proton beam therapy should be considered to reduce late toxicity, especially in younger patients 1
Chemotherapy
- Chemotherapy alone is recommended for children less than 12 months old 1
- For children between 12-18 months, chemotherapy may be used in combination with reduced-dose radiation therapy 1
- No standardized chemotherapy regimen has been established specifically for choroid plexus carcinoma, but protocols designed for malignant brain tumors in infants have shown efficacy 3
Treatment Algorithm Based on Age
Children <12 months:
- Maximal safe surgical resection
- Chemotherapy alone
- Avoid radiation therapy due to neurocognitive risks
Children 12-18 months:
- Maximal safe surgical resection
- Reduced-dose radiation therapy (54 Gy)
- Consider adding chemotherapy
Children >18 months:
- Maximal safe surgical resection
- Full-dose conformal radiotherapy (up to 59.4 Gy)
- Consider chemotherapy for high-risk features (incomplete resection, disseminated disease)
Special Considerations
- Patients with Li-Fraumeni syndrome (germline TP53 mutations) may develop choroid plexus carcinoma and require special consideration regarding radiation therapy due to increased risk of secondary malignancies 1
- For patients with disseminated disease, craniospinal irradiation plus focal boost is recommended 1
- Regular surveillance with MRI is essential to monitor for recurrence, starting 2-6 weeks post-radiation, then every 2-3 months for the first year 1
Prognostic Factors
- Extent of resection is the most significant prognostic factor, with GTR associated with significantly better survival 2
- Age at diagnosis (younger patients typically have worse outcomes)
- Presence of metastatic disease at diagnosis
- Molecular characteristics (TP53 status)
Pitfalls and Caveats
- Delaying radiation therapy in very young children must be balanced against the risk of tumor progression
- Aggressive surgical resection must be weighed against potential neurological morbidity
- Long-term surveillance is essential as late recurrences can occur
- Neurocognitive assessment should be performed regularly to monitor for treatment-related toxicities 1
- Consider genetic counseling as choroid plexus carcinoma may be associated with cancer predisposition syndromes 1
Despite the rarity of choroid plexus carcinoma, the evidence strongly supports maximal safe surgical resection as the primary treatment modality, followed by age-appropriate adjuvant therapy to optimize survival outcomes while minimizing long-term morbidity.