Choroid plexus carcinoma in infants: report of two cases and review of the literature

Abstract

Choroid plexus carcinoma (CPC) is a rare malignant brain tumour which occurs predominantly in childhood. We present the cases of two infants with CPC. One, a 6-month-old boy with the tumour in the right lateral ventricle, who died of a postoperative intracranial haemorrhage and severe gastrointestinal bleeding, and the other, a 9-month-old boy with the tumour in the fourth ventricle, who has been well without recurrence for 12 months after total removal in combination with chemotherapy using cisplatin and VP-16 and local radiotherapy. In the 54 CPC cases in children under 2 years of age including our 2 cases in which the clinical results were described in the literature since 1983, tumour location (lateral ventricle, p = 0.0225), surgery (gross total resection, p = 0.0447), and chemotherapy (yes, p = 0.0010) were revealed to be significant positive prognostic factors by the univariate analysis using the log rank test, and surgery (gross total removal, p = 0.0259) and chemotherapy (yes, p = 0.0016) were independent, significant positive prognostic factors in the multivariate analysis using the Cox proportional hazard regression model. Although there is a risk in doing a statistical analysis of other people's reports, these results suggest that, at present, the gross total removal of the tumour with intensive chemotherapy is the best choice of initial treatment for young children with CPC, and that radiotherapy should be considered for patients after 24 months of age and/or should be performed locally.