Intra-Arterial Chemotherapy for Newly Diagnosed, Residual, or Recurrent Atypical Choroid Plexus Papilloma and Choroid Plexus Carcinoma Prior to Second-Look Surgery

Inclusion Criteria:

1. Subjects with a histologically confirmed diagnosis of ACPP or CPC that is newlydiagnosed, residual or recurrent.

2. Subjects must have a Karnofsky or Lansky Performance Score ≥ 60 % assessed withintwo weeks prior to enrollment. Karnofsky is used for patients ≥ 16 years and Lanskyfor those < 16.

3. Subjects must have normal organ and marrow function documented within 14 days ofenrollment and within 7 days of the start of treatment as noted below:

4. Absolute neutrophil count ≥ 1,000/μL

5. Platelets ≥ 100,000/μL (transfusion independent, defined as not receivingplatelet transfusions within a 7-day period prior to enrollment)

6. Hemoglobin ≥ 8 g/dL (may receive PRBC transfusions)

7. Total bilirubin < 1.5 times upper limit of normal for age

8. AST (SGOT)/ALT(SGPT) < 2.5 X institutional upper limit of normal for age

9. Creatinine clearance or radioisotope GFR ≥ 70 ml/min/1.73m2 or a serumcreatinine WNL for age as determined using the Schwartz formula.36

10. Sodium, Potassium, Calcium and Magnesium < 1.5x institutional ULN

11. Albumin ≥ 3 g/dL

12. Subjects who are receiving dexamethasone must be on a stable or decreasing dose forat least 1 week prior to enrollment.

13. Subjects with neurological deficits should have deficits that are stable for aminimum of 1 week prior to enrollment.

14. If the subject has any of the following therapies, must be at least:

• 4 weeks post-focal RT (radiation therapy), 3 months post-CSI (craniospinalirradiation)

• 4 weeks post-myelosuppressive chemotherapy (if post-nitrosoureas, must have 6weeks therapy)

• 4 weeks post-monoclonal antibodies

• 1 week post-targeted therapy

7. If subject has received any previous treatment, all treatment related toxicitiesshould have recovered to < grade 2

8. Subject or parent must sign a written informed consent document according toinstitutional guidelines.

Exclusion Criteria:

1. Females who are pregnant or lactating.

2. Subjects with any clinically significant unrelated systemic illness (seriousinfections or significant cardiac, pulmonary, hepatic or other organ dysfunction)likely to interfere with the study procedures or results.

3. Subjects who are receiving any other anticancer or investigational agents.

4. Subjects with uncontrolled seizures.

5. Subjects receiving enzyme inducing anticonvulsants.

6. Subjects with other factors that increase the risk of QT prolongation or arrhythmicevents (e.g., heart failure, hypokalemia, family history of long QT intervalsyndrome) including heart failure that meets New York Heart Association (NYHA) classII or above.

7. Subjects who have had an allogenic bone marrow transplant < 6 months prior toenrollment or an autologous bone marrow/stem cell transplant < 3 months prior toenrollment.

8. Subjects with multifocal disease or disease that has been disseminated will not beeligible for this study. They will undergo systemic chemotherapy and their diseasewill be further evaluated prior to be eligible for 2nd look surgery.

9. This study will only enroll subjects with ACPP or CPC and will not enroll subjectswith choroid plexus papilloma (CPP). ACPP or CPC subjects with symptomatichydrocephalus will not be eligible for this study. These subjects will have to betreated for their hydrocephalus and be re-evaluated according to our eligibilitycriteria in order to be enrolled.