Atypical choroid plexus papillomas can metastasize in the form of leptomeningeal seeding. Postoperative chemotherapy is the recommended first-line treatment when gross-total removal is not achieved or in cases of disseminated disease. Here the authors report on 2 children with atypical choroid plexus papillomas and MRI findings of diffuse leptomeningeal enhancement at diagnosis, later presenting with spontaneous resolution of the leptomeningeal involvement after removal of the primary lesions. Observations in this report expand our knowledge about the natural history and biological behavior of these tumors and highlight the role of close neuroimaging surveillance in the management of atypical choroid plexus papillomas in cases with MRI evidence of diffuse leptomeningeal enhancement at presentation.
Keywords: ACPP = atypical CPP; CPC = choroid plexus carcinoma; CPP = choroid plexus papilloma; CPT = choroid plexus tumor; CSF = cerebrospinal fluid; atypical choroid plexus papilloma; brain tumor; leptomeningeal dissemination; oncology; pediatric.