Case Reports in Oncological Medicine (Jan 2018)

Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma

  • Maurício Fernando Silva Almeida Ribeiro,
  • Micelange Carvalho de Sousa,
  • Samir Abdallah Hanna,
  • Marcos Vinicius Calfat Maldaun,
  • Ceci Obara Kurimori,
  • Luiz Guilherme Cernaglia Aureliano de Lima,
  • Romulo Loss Mattedi,
  • Rodrigo Ramella Munhoz

DOI
https://doi.org/10.1155/2018/4290131
Journal volume & issue
Vol. 2018

Abstract

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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.