{Reference Type}: Case Reports
{Title}: Leptomeningeal Dissemination of Low-Grade Neuroepithelial Tumor with FGFR1_TACC1 Fusion with Clinical and Radiographic Response to Pazopanib and Topotecan.
{Author}: Nelson AT;Bendel A;Skrypek M;Patel S;Tabori U;McDonald W;Schultz KAP;
{Journal}: Pediatr Neurosurg
{Volume}: 0
{Issue}: 0
{Year}: Nov 2021 8
{Factor}: 1.165
{DOI}: 10.1159/000519889
{Abstract}: <B>BACKGROUND: </B>Low-grade neuroepithelial tumors are a heterogeneous group of central nervous system tumors that are generally indolent in nature but in rare instances can progress to include leptomeningeal dissemination.<BR><B>METHODS: </B>We present a case of a patient with a low-grade neuroepithelial tumor of indeterminate type with symptomatic leptomeningeal dissemination despite 3 chemotherapy regimens and radiotherapy. Somatic targetable mutation testing showed an FGFR1_TACC1 fusion. Therapy with pazopanib/topotecan was initiated, and disease stabilization was achieved. He received pazopanib/topotecan for a total of 2 years and is now >2 years from completion of treatment and continues to do well with no evidence of disease.<BR><B>CONCLUSIONS: </B>This case highlights the utility of targetable mutation testing in therapeutic decision-making and the novel use of systemic pazopanib/topotecan therapy for refractory low-grade neuroepithelial tumor within the context of this clinical situation and specific mutation profile.