Tumor Reduction with Pazopanib in a Patient withRecurrent Lumbar Chordoma

hordomas are rare malignancies of bone origin that occur in the axial skeleton, typically the skull base andlumbar/sacral regions. Although often classified as low-grade neoplasms, its locally infiltrative behavior may result in significantmorbidity and mortality. Optimal surgical resection may be curative, but up to 50% of the cases relapse within 5 years, andcurrently there are no systemic treatments approved in this setting. A large proportion of these tumors express stem-cell factorreceptor (c-KIT) and platelet-derived growth factor receptors (PDGFRs), providing a rationale for the use of tyrosine-kinaseinhibitors (TKIs).Case report. A 27-year-old male presented with recurrent chordoma of the lumbar spine 4 years after initialdiagnosis. Salvage therapies in the interval included repeat resections and radiation therapy. He ultimately developed multifocalrecurrence not amenable to complete excision or reirradiation. A comprehensive genomic profiling assay was performed andrevealed nondrugable alterations. Decision was made to proceed with systemic treatment with pazopanib 800 mg/day, resulting intumor reduction (−23.1% reduction in size) and prolonged disease control.Conclusion. For this patient with a multiple recurrentchordoma and limited treatment options, pazopanib resulted in sustained clinical benefit following initial tumor reduction