Abstract
BACKGROUND
Children diagnosed with Choroid Plexus Carcinoma (CPC) in the first year of life usually have a very poor outcome partly because radiotherapy cannot be used as a part of treatment. We report 3 infants diagnosed with CPC in the first year of life including one patient who has Li-Fraumeni Syndrome who were successfully treated solely with chemotherapy. CASE
REPORTS
Patient 1: diagnosed at age 1.5 months with a non-metastatic CPC in the 3rd ventricle. Following gross total tumor resection (GTR) he was treated on COG protocol POG 9923/34 using Vincristine, Etoposide, Cyclophosphamide and Cisplatin (VECC). He remains disease free 4 years post completion of therapy. Patient 2: diagnosed at age 8 months with a non-metastatic left lateral ventricle CPC. Following GTR he was treated on protocol POG 9932/34. He remains disease free 6 years post completion of therapy. Patient 3: Patient had a strong family history of cancer and was diagnosed with an adrenocortical neoplasm at birth. She was diagnosed at age 6 months with a right lateral ventricle CPC disseminated through the brainstem and spinal cord. After a GTR of the intracranial tumor she was treated as per the Head Start 2 (Regimen A) protocol using VECC and High Dose Methotrexate followed by triple autologous stem cell transplant (Carboplatin/Thiotepa conditioning). She remains disease free 8 months post completion of therapy. All patients experienced significant bacterial sepsis episodes, malnutrition and ototoxicity.
CONCLUSION
Long term tumor control of CPC is possible using chemotherapy only protocols in very young infants.