Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma

الملخص EN

Introduction.

Chordomas are rare malignancies of bone origin that occur in the axial skeleton, typically the skull base and lumbar/sacral regions.

Although often classified as low-grade neoplasms, its locally infiltrative behavior may result in significant morbidity and mortality.

Optimal surgical resection may be curative, but up to 50% of the cases relapse within 5 years, and currently there are no systemic treatments approved in this setting.

A large proportion of these tumors express stem-cell factor receptor (c-KIT) and platelet-derived growth factor receptors (PDGFRs), providing a rationale for the use of tyrosine-kinase inhibitors (TKIs).

Case report.

A 27-year-old male presented with recurrent chordoma of the lumbar spine 4 years after initial diagnosis.

Salvage therapies in the interval included repeat resections and radiation therapy.

He ultimately developed multifocal recurrence not amenable to complete excision or reirradiation.

A comprehensive genomic profiling assay was performed and revealed nondrugable alterations.

Decision was made to proceed with systemic treatment with pazopanib 800 mg/day, resulting in tumor reduction (−23.1% reduction in size) and prolonged disease control.

Conclusion.

For this patient with a multiple recurrent chordoma and limited treatment options, pazopanib resulted in sustained clinical benefit following initial tumor reduction.