Open AccessLeptomeningeal Dissemination of Low-Grade Neuroepithelial Tumor with FGFR1_TACC1 Fusion with Clinical and Radiographic Response to Pazopanib and Topotecan
Author(s) -
Alexander T Nelson,
Anne Bendel,
Maggie Skrypek,
Sachin Patel,
Uri Tabori,
William C. McDonald,
Kris Ann P. Schultz
Publication year - 2021
Publication title -
pediatric neurosurgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.385
H-Index - 72
eISSN - 1423-0305
pISSN - 1016-2291
DOI - 10.1159/000519889
Subject(s) - pazopanib , medicine , topotecan , oncology , context (archaeology) , radiation therapy , refractory (planetary science) , neuroepithelial cell , chemotherapy , surgery , radiology , cancer , sunitinib , paleontology , physics , astrobiology , biology , genetics , stem cell , neural stem cell
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. Case Presentation: 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. Discussion: 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.