Tumor Reduction with Pazopanib in a Patient with Recurrent Lumbar Chordoma

• Published in: Case reports in oncological medicine Vol. 2018; no. 2018; pp. 1 - 7
• Main Authors: Munhoz, Rodrigo Ramella, de Lima, Luiz Guilherme Cernaglia Aureliano, Kurimori, Ceci O., Maldaun, Marcos Vinicius Calfat, Hanna, Samir Abdallah, de Sousa, Micelange Carvalho, Ribeiro, Maurício Fernando Silva Almeida, Mattedi, Romulo Loss
• Format: Journal Article
• Language: English
• Published: Cairo, Egypt Hindawi Publishing Corporation 01.01.2018; Hindawi; John Wiley & Sons, Inc; Hindawi Limited
• Subjects: Antimitotic agents; Antineoplastic agents; Cancer therapies; Case Report; Case reports; Development and progression; Disease control; Growth factors; Kinases; Medical research; Metastasis; Mortality; NMR; Nuclear magnetic resonance; Oncology; Patients; Platelet-derived growth factor; Radiation therapy; Radiotherapy; Spinal cord; Targeted cancer therapy; Transcription factors; Tumors; Tumors, Embryonal
• ISSN: 2090-6706; 2090-6714
• DOI: 10.1155/2018/4290131

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.