Bevacizumab (BEV) use in disseminated choroid plexus papilloma (DCCP).

Abstract

e13039 Background: Choroid plexus papilloma (CCP) is a rare primary neuro-ectodermal tumor of the choroid plexus, representing less than 1% of intracranial tumors in adults. The most common location in adults is the 4th ventricle and treatment is gross total resection (GTR). Leptomeningeal disease (LMD) can rarely occur, even when the tumor retains benign histology. Because of the rarity of DCCP, there is no standard treatment, with less than 20 published cases. There have no previous descriptions of the use of BEV therapy in DCCP. Methods: We present 2 patients (pts) with DCCP, who received gross total resection, radiation and chemotherapy with continued progression, who received treatment with BEV therapy. Results: Pt 1 presented with a 4th ventricular mass and evidence of LMD. GTR of the posterior fossa lesion revealed an atypical CCP. The pt received craniospinal radiation with concurrent temozolomide (TMZ) therapy. After 4 years, there was leptomeningeal disease progression and the pt developed progressive radicular pain and weakness, unresponsive to typical treatments. He was initiated on TMZ and BEV therapy, but the TMZ was discontinued due to thrombocytopenia and the pt was continued on BEV alone. While the MRI showed stable disease at, the pt had complete remission of pain and some improvement of ataxia. When BEV was held due to toxicity, the pain returned until BEV was restarted. Carboplatin was added to BEV with progression of LMD at 21 months. Pt 2 is a 58-year-old man who present with a 4th ventricular mass, shown to be CCP after gross total resection. The pt received radiotherapy for diffuse sacral recurrence. After three years of surveillance, the pt received lomustine and 6-thioguanine for disease progression, limited to 5 cycles by thrombocytopenia. After 5 years, the pt noted continued progressive radicular pain in his lower extremities. The pt completed a planned course of 10 doses of bevacizumab and had improvement in pain control and stable imaging at 11 months. Conclusions: While bevacizumab resulted in stable imaging when used in DCCP, there was significant improvement in debilitating radicular symptoms. The complications of BEV therapy must be considered with the potential benefit of symptomatic relief. Prospective studies are needed.