Initial Management at Presentation
- Control of hydrocephalus: Emergent management of hydrocephalus may be required if the child is lethargic from elevated ICP related to hydrocephalus. EVD placement may be required prior to definitive resection of the tumor.
- Embolization: Preoperative embolization is useful in reducing the amount of blood loss during the surgical resection. However, since 75% of these tumors are diagnosed in children younger than 2 years of age, there are technical difficulties linked to embolization, and the risks vs. benefits might differ depending on the experience of the interventional team.
- Surgical resection: For choroid plexus papillomas, total surgical resection leads to a cure in a large percentage of cases and therefore should be the aim. For choroid plexus carcinomas, gross total resection also leads to better outcomes.
Choroid plexus carcinoma
- Chemotherapy: The role of adjuvant chemotherapy is controversial. It can be used in young children when deferring radiation treatment. Use of a combination of ifosfamide, carboplatin, and etoposide (ICE) has been described (111).
- Radiotherapy: As many as 44% of carcinomas will have CSF seeding. Radiotherapy is indicated with a positive neuraxis staging, a WHO grade III, and positive margins. However, radiation treatment will be deferred in children younger than 3 or 4 years of age (111).
- Scheduled follow-up: Typically, early follow-up is planned for 4–6 weeks after surgery.
- Postoperative imaging important: The first postoperative imaging should be done within 72 hours to make sure there is no residual tumor or to assess its size if tumor remains. Follow-up imaging will then depend on the pathology of the tumor and on the adjuvant therapies (typically every 3 months for choroid plexus carcinomas; and every 6 months, then every year for choroid plexus papillomas).
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