Variations of disseminated choroid plexus papilloma: 2 case reports and a review of the literature

Abstract

Background Choroid plexus papillomas are typically considered benign lesions, but histology is not always predictive of their behavior. These tumors can metastasize anywhere along the neuraxis and may be intraventricular, subarachnoid, or intraparenchymal. We present 2 cases that illustrate the wide diversity with which choroid plexus papillomas can disseminate. Case Descriptions The patient described in case 1 had a primary fourth ventricular choroid plexus papilloma that produced diffuse cystic subarachnoid and leptomeningeal lesions. Patient 2 also had a primary fourth ventricular tumor but with subsequent suprasellar and spinal drop metastases. Patient 1 was treated with temozolomide, resulting in regression of symptoms including headache and dizziness. Patient 2 has been treated with several modalities, including radiation therapy and chemotherapy, with slowing of symptom progression. Conclusions Variations of choroid plexus papilloma dissemination include intraventricular, subarachnoid, and leptomeningeal nodules or cystic lesions, and intraparenchymal locations. There is no consensus on the most effective treatment for choroid plexus papilloma metastases; surgical resection, chemotherapy, and radiation therapy may all yield benefits. The prognosis for patients with disseminated choroid plexus papilloma can range from prolonged stable disease and symptoms to death within months.