Management of Supratentorial Choroid Plexus Tumors in Children
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).