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Carcinoma of the choroid plexus: a pediatric experience
J Y Pierga1, C Kalifa, M J Terrier-Lacombe
1Department of Pediatrics, Institut Gustave Roussy, Villejuif, France.
Insights
Carcinoma of the choroid plexus (CCP) is a rare pediatric brain tumor. Complete surgical resection is key for survival, and chemotherapy may be a viable alternative to radiotherapy in young children.
Area of Science:
- Pediatric neuro-oncology
- Central nervous system tumors
- Rare pediatric malignancies
Background:
- Carcinoma of the choroid plexus (CCP) is a rare central nervous system tumor.
- CCP occurs more frequently in children under 3 years of age.
Purpose of the Study:
- To report on 10 pediatric cases of Carcinoma of the choroid plexus.
- To evaluate prognostic factors and treatment outcomes for pediatric CCP.
Main Methods:
- Retrospective case series of 10 children diagnosed with CCP.
- Analysis of surgical resection extent, adjuvant therapies (radiotherapy, chemotherapy), and patient outcomes.
- Comparison with existing literature data.
Main Results:
- Six out of 10 patients achieved complete remission.
- Complete surgical resection was the major prognostic factor for survival.
- Chemotherapy alone was effective in some patients, suggesting it as an alternative to radiotherapy.
Conclusions:
- Complete tumor resection is critical for improving survival in pediatric CCP.
- Radiotherapy should be used cautiously in very young children due to potential adverse effects.
- Postoperative chemotherapy protocols for infant brain tumors may be effective for CCP treatment.
Abstract:
Carcinoma of the choroid plexus (CCP) is a rare tumor of the central nervous system which occurs more frequently in patients under 3 years of age. We report 10 cases of CCP in children. There were 5 males and 5 females, aged 5 to 84 months at diagnosis (median 32 months). Eight CCP were located in the lateral ventricle and 2 in the fourth ventricle. All patients underwent initial surgery, with complete resection in 5 cases. Postoperative treatment included radiotherapy alone in 4 patients, chemotherapy alone in 4, both in 1 patient, and no further treatment in one patient. Six patients remain in first continuous complete remission with a follow-up of 12 to 156 months (mean 34 months). Five of these six patients had a complete surgical resection of their tumor and 4 of them received chemotherapy alone. A comparison with the series in the literature suggests that total resection of the tumors is the major prognostic factor for survival. Recourse to additional treatments to prevent local relapses and CNS dissemination seems necessary. In our opinion, radiotherapy should be avoided as far as possible because of its deleterious effect in very young children. As some of the long term survivors did not receive radiation treatment, we conclude that CCP can be treated post operatively using chemotherapy protocols designed for infants with malignant brain tumors.