Atypical Choroid Plexus Papilloma Treated With Single Agent Bevacizumab
A 40-year-old woman was referred to us for salvage treatment. She was diagnosed in February 2005 with a 6.5 cm right occipital tumor with small millimetric parenchymal metastases and drop metastases throughout the neuraxis. She presented with clinical evidence of increased intra-crania pressure (ICP), mass effect with extensive vasogenic edema requiring urgent surgery. She underwent gross tumor resection and pathology reported an atypical choroid plexus papilloma. Her staging work up was obtained following her recovery including brain and full spine contrast enhanced magnetic resonance imaging (MRI) scans. This revealed small drop metastases in the posterior fossa and along the spinal cord. The patient received radiation therapy to the neuraxis (the CSI dose was 36 Gy and the total dose to the primary tumor bed or surgical cavity was 54 Gy). She remained clinically stable for two years, and was able to perform activities of daily living. Repeated contrast enhanced MRI scans of the brain and full spine reported stable appearance in size and enhancement of all drop metastases in the posterior fossa and the spinal canal.\nAt relapse, she presented with continuous headaches, neck stiffness, altered mental status, and severe ataxia. Imaging revealed metastasis to bilateral cerebello-pontine (CP) angles and intraparenchymal supratentorial lesions (left frontal, right thalamic, and right temporal). She received 2 cycles of nitrosourea without response and worsening symptoms. Hence this was changed to ifosfamide, carboplatin and etoposide based combination. Radiological and clinical stabilization were attained following three cycles, but second progression was documented after the fifth cycle. Further surgeries and performed for rapidly growing and symptomatic right CP angle metastasis and dorsal brain stem metastasis. Post-operative imaging revealed decrease size of the tumor. With remaining residual disease, the patient was referred to us for salvage treatment.\nIn this setting of previous craniospinal irradiation, significant chemotherapy toxicity, and only short-lived stabilization of the disease, bevacizumab was offered as single agent treatment in an attempt to spare the patient further toxicities. The first treatment was injected after 8 weeks from last surgery.\nThe patient received bevacizumab, 5 mg/kg on day 1 and day 15 of each cycle. Cycles were repeated every 4 weeks. Contrast enhanced MRI scans were obtained after the initial two treatments and every 2 cycles thereafter or sooner if required to assess new or worsening symptoms.\nThe patient was taken off steroids, which was started at first relapse, shortly after starting bevacizumab and did not receive steroids later during the course of her disease. Her symptoms immediately stabilized following the first cycle of bevacizumab and progressively improved. The headaches resolved, the mental status returned to normal, and the ataxia improved very slowly but her cervical pain and stiffness did not. She achieved radiological stabilization with significant decrease of the vasogenic edema, and minimal decrease in the size and enhancement of all metastatic lesions ( and ). This response continued for over 5 months of therapy. A total of five cycles of bevacizumab were administered (10 treatments). An elective cerebello-pontine angle metastasis resection was opted to relieve her debilitating neck stiffness.\nThe postoperative course was complicated by respiratory failure requiring tracheostomy, although there was no evidence of disease progression. The patient died form pulmonary infection, in turn complicated by severe sepsis.