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Current management of choroid plexus carcinoma in children
S K St Clair1, R P Humphreys, P K Pillay
1Division of Neurosurgery, Hospital for Sick Children, Toronto, Canada.
Insights
Choroid plexus carcinomas in children are aggressive tumors. Neoadjuvant chemotherapy can reduce tumor size and vascularity, potentially improving surgical outcomes and prognosis.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Cancer Research
Background:
- Choroid plexus carcinomas are rare, aggressive brain tumors in children.
- These tumors are often highly vascular and difficult to resect completely.
- Prognosis for long-term survival is typically poor.
Purpose of the Study:
- To review presentation, diagnosis, treatment, and outcomes of pediatric choroid plexus carcinoma.
- To evaluate the role of neoadjuvant chemotherapy in managing these tumors.
- To assess the impact of chemotherapy-induced tumor reduction on surgical resection and prognosis.
Main Methods:
- Retrospective review of 11 pediatric choroid plexus carcinoma cases over 33 years.
- Analysis of tumor presentation, radiographic findings, and histopathology.
- Evaluation of treatment strategies including surgery and adjuvant chemotherapy.
Main Results:
- Choroid plexus carcinomas represented 39% of all choroid plexus tumors in the study cohort.
- Neoadjuvant chemotherapy in four recent cases reduced tumor volume and vascularity.
- This reduction facilitated safer, more complete tumor removal in a second-stage surgery.
Conclusions:
- While not curative, neoadjuvant chemotherapy appears to improve surgical resectability of choroid plexus carcinoma.
- Further follow-up is needed to determine if this approach significantly improves long-term survival.
- Gross total tumor removal is a critical initial step toward better management and prognosis.
Abstract:
Eleven children with choroid plexus carcinoma are reviewed with respect to presentation, radiographic diagnosis, treatment and pathologic histology. These cases accumulated over a 33-year period and represent 39% of all of our choroid plexus tumors (n = 28). Characteristically, choroid plexus carcinomas are associated with a poor prognosis for long-term survival. Unfortunately, efforts at total resection are hindered in part by the extreme vascularity of the tumor which itself may be of considerable volume. We have recently treated four children who had biopsy or minimal resection of their tumor after which adjuvant chemotherapy was given. Chemotherapy is not curative but it does cause a reduction in tumor volume and, more importantly, has tended to reduce the tumor vascularity. This allows for a second stage operation which is more safely performed and typically allows for more complete tumor removal. At this point, our length of follow-up is insufficient to conclude if total removal in this manner significantly increases survival but it would seem that consistent gross total removal may be an initial step toward better overall management and prognosis in this tumor type.