Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma
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 r...
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| Format: | Article |
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Wiley
2018-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2018/4290131 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Maurício Fernando Silva Almeida Ribeiro |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-3bc5e182a9d74ba1be1ee7505b0b6394 |
| institution | Kabale University |
| issn | 2090-6706 2090-6714 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-3bc5e182a9d74ba1be1ee7505b0b63942025-02-03T00:59:27ZengWileyCase Reports in Oncological Medicine2090-67062090-67142018-01-01201810.1155/2018/42901314290131Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar ChordomaMaurício Fernando Silva Almeida Ribeiro0Micelange Carvalho de Sousa1Samir Abdallah Hanna2Marcos Vinicius Calfat Maldaun3Ceci Obara Kurimori4Luiz Guilherme Cernaglia Aureliano de Lima5Romulo Loss Mattedi6Rodrigo Ramella Munhoz7Hospital Sírio Libanês, São Paulo, SP, BrazilHospital Sírio Libanês, São Paulo, SP, BrazilHospital Sírio Libanês, São Paulo, SP, BrazilHospital Sírio Libanês, São Paulo, SP, BrazilHospital Sírio Libanês, São Paulo, SP, BrazilHospital Sírio Libanês, São Paulo, SP, BrazilHospital Sírio Libanês, São Paulo, SP, BrazilHospital Sírio Libanês, São Paulo, SP, BrazilIntroduction. 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.http://dx.doi.org/10.1155/2018/4290131 |
| spellingShingle | 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 Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma Case Reports in Oncological Medicine |
| title | Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma |
| title_full | Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma |
| title_fullStr | Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma |
| title_full_unstemmed | Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma |
| title_short | Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma |
| title_sort | tumor reduction with pazopanib in a patient with recurrent lumbar chordoma |
| url | http://dx.doi.org/10.1155/2018/4290131 |
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