| Autor: |
Fernando Silva Almeida Ribeiro, Maurício, Carvalho de Sousa, Micelange, Abdallah Hanna, Samir, Vinicius Calfat Maldaun, Marcos, Obara Kurimori, Ceci, Guilherme Cernaglia Aureliano de Lima, Luiz, Loss Mattedi, Romulo, Ramella Munhoz, Rodrigo |
| Zdroj: |
Case Reports in Oncological Medicine; 2018, Vol. 2018 Issue: 1 |
| Abstrakt: |
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. |
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