LINC-05. Successful response to bevacizumab/irinotecan/temozolomide in a progressive choroid plexus carcinoma: A case report

Choroid plexus carcinomas (CPC) are a rare type of aggressive pediatric brain tumors with poor survival rates, and no standard curative therapy after relapse. We report the case of a 1-year-oldmale, with a right lateral ventricular CPC and disseminated leptomeningeal disease. First line therapy was an initial surgery aborted due to hemorrhage and a second near total resection. After the second surgery six cycles of ICE regimen were applied. The MRI after primary therapy showed progression with a new lesion located on the optic pathway and leptomeningeal disease. At this point a second line therapy consisting of Bebacizumab 10 mg/kg and Irinotecan 125 mg/kg every 2 weeks and Temozolamide 150 mg/m2 every 4 weeks was given. He received up to 24 cycles. His imaging demonstrated 80% primary tumor reduction and improvement of leptomeningeal disease. This treatment gave him the time to turn 3 years old to receive 3D conformational craniospinal radiotherapy as follows: spine 24Gy, overdose 27 Gy to tumor bed and cranial for a total dose 51 Gy. The patient is now 19 months from the end of treatment with stable disease. He is clinically well, with good performance status (Lansky 100%) and attending school. The relevance to present this case is to highlight a safe and effective treatment for a relapsed CPC since there is not a curative therapy for these children.