Adjuvant Therapies for Supratentorial Choroid Plexus Tumors in Children

Chemotherapy

Choroid plexus carcinoma

• Children younger than 4 years of age: Different regimens have been used to treat patients with choroid plexus carcinomas, and chemotherapy is the sole adjuvant therapy for children with choroid plexus carcinoma who are younger than 3 or 4 years. Chemotherapy allows the deferral of radiation therapy. Overall, however, the response rate is poor (63).
• Treatment regimens: Treatment regimens include etoposide (shown in a meta-analysis to have the highest response rate (10)) and mannitol or cisplatin; vinblastine, cisplatin, and bleomycin; cisplatin and vincristine; vincristine and CCNU (10, 33).
• Unproven: No randomized controlled trial has been conducted due to the small number of cases.  Chemotherapy is therefore recommended to treat children with hypervascular tumors and with tumors that were not completely resected (93).
• Preoperative chemotherapy: In some series, preoperative chemotherapy has been shown to decrease the size of the tumor (77). A single case report shows a 25% reduction in tumor mass after preoperative chemotherapy (71).

Radiation

Choroid plexus carcinoma

• Children older than 4 years of age: Radiation has been used to treat patients with choroid plexus carcinomas who are older than 4 years of age (92). However, the benefit is controversial (24, 66, 80). The general consensus is that radiation should only be used to treat children older than 3 or 4 years, since the effects of global radiation on children younger than 4 years have been shown to be more deleterious than beneficial.
• Craniospinal: Irradiation of the whole craniospinal axis is advocated because of the propensity of the tumor for seeding.