A Meta-Analysis of Choroid Plexus Papilloma (CPP) and Choroid Plexus Carcinoma (CPC)

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Abstract 885 Poster Session I, Saturday, 5/1 (poster 278)

Background: Choroid plexus tumors (CPPs and CPCs) are rare and their prognosis is uncertain. As they comprise only 2-3% of all brain tumors in children, prospective randomized trials are missing. Consequently, adequate data to provide rationale treatment recommendations is lacking. Surgical excision is the primary mode of therapy, although the extend of surgery remains an issue of debate, in particular for choroid plexus carcinomas. The role of radiotherapy (XRT) and chemotherapy (CT) is an open question.

Methods: Information obtained from the literature was used to create a database. Sixty-two of 297 publications between 1954 and 1997 provided information on 524 individual patients. Follow-up data was available for 226 patients. The data were analyzed using FOX-Pro and SPSS.

Results: The overall projected survival (Kaplan Meier) of 109 choroid plexus-papilloma (CPP) was 87%, 78% and 72% after 1, 5 and 10 years, respectively. For the 136 choroid plexus carcinoma (CPC) patients survival was 75%, 45%, 38%, respectively (p=0.0001, log rank test). Gender did not alter outcome, age did. For CPC patients, children ≥4 years did better than those <4 years of age (p=0.029), while for CPP patients outcome was best for those <40 years of age (p=0.0003). Complete resection correlated with good outcome: for those with CPC, 1-year survival after complete resection was 76% as compared to 40%, 35% and 20% after subtotal resection, partial resection or biopsy only (p < 0.001). Similarly, CPP patients with complete resection had a 10 year survival rate of 77% versus 50% after incomplete resection (p=0.007). The influence of XRT could not be evaluated in CPP, because the number of radiated patients was too low. Even in CPC this question could only addressed in one subgroup: survival of CPC patients after gross total resection was significantly better if followed by XRT. The 10-year survival rate for those with XRT (n=24) was 50% versus 15% for those (n=24) without XRT (p=0.016). Finally, the effects of CT showed a trend towards being beneficial for CPC patients; 62% survival for those receiving CT versus 46% for those with no adjuvant CT (p=0.53).

Conclusion: Despite the limitations of a meta-analysis, we believe that our data support the recommendation of gross total surgical resection for all patients with choroid plexus tumors. In addition, adjuvant XRT should be considered for patients with CPC. The role of chemotherapy should be further explored in prospective trials.

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Sagidi, M., Coppes, M., Egeler, R. et al. A Meta-Analysis of Choroid Plexus Papilloma (CPP) and Choroid Plexus Carcinoma (CPC). Pediatr Res 45, 152 (1999) doi:10.1203/00006450-199904020-00902

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