Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma

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Tipo de produção
article
Data de publicação
2018
Título da Revista
ISSN da Revista
Título do Volume
Editora
HINDAWI LTD
Autores
RIBEIRO, Mauricio Fernando Silva Almeida
SOUSA, Micelange Carvalho de
HANNA, Samir Abdallah
MALDAUN, Marcos Vinicius Calfat
KURIMORI, Ceci Obara
Citação
CASE REPORTS IN ONCOLOGICAL MEDICINE, article ID 4290131, 7p, 2018
Projetos de Pesquisa
Unidades Organizacionais
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Resumo
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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Referências
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