Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review

Launay, Simon; Chetaille, Bruno; Medina, F. Sanchiz; Perrot, Delphine; Nazarian, Serge; Guiramand, Jérôme; Moureau-Zabotto, Laurence; Bertucci, François

doi:10.1186/1471-2407-11-423

Cited by 50 publications

(40 citation statements)

References 17 publications

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“…Shalaby et al (62) demonstrated that ErbB1 inhibitor tyrosine (AG1487) markedly inhibited proliferation of the chordoma cell line U-CH1 in vitro and diminished ErbB1 phosphorylation, and these data implicated aberrant ErbB1 signaling in the pathogenesis of chordomas. Furthermore, four clinical cases highlighted the antitumor activity of ErbB1 inhibition in advanced chordomas (68)(69)(70)(71). These studies support the conclusion that ErbB1 could be a potential target for improving treatment of osteosarcomas.…”

Section: Erbb Receptors In Chordomamentioning

confidence: 53%

ErbB Receptors as Prognostic and Therapeutic Drug Targets in Bone and Soft Tissue Sarcomas

Wang¹,

Yang²,

Fu³

et al. 2014

Cancer Investigation

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ErbB receptors have been intensely studied to understand their importance in cancer biology and as therapeutic targets, and many ErbB inhibitors are now used in the clinical setting. A large number of studies have been conducted to examine the expression of ErbB family members in bone and soft tissue sarcomas, including osteosarcomas, synovial sarcomas, Ewing sarcomas, rhabdomyosarcomas, and so on. Nevertheless, the clinical implications of ErbB receptors remain elusive. To illustrate the potential of ErbB family members as prognostic and therapeutic drug targets in bone and soft tissue sarcomas, we summarized the molecular evidence and observations from clinical and basic trials.

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Section: Erbb Receptors In Chordomamentioning

confidence: 53%

ErbB Receptors as Prognostic and Therapeutic Drug Targets in Bone and Soft Tissue Sarcomas

Wang¹,

Yang²,

Fu³

et al. 2014

Cancer Investigation

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“…[44] Two cases of treatment with an alternative EGFR inhibitor, erlotinib, also showed significant response. [4281] The successes seen with these EGFR inhibitors suggest that targeting EGFR in chordoma is an effective method of treatment that should be incorporated into the arsenal of therapies available for chordoma patients.…”

Section: Molecular Geneticsmentioning

confidence: 99%

Converging paths to progress for skull base chordoma: Review of current therapy and future molecular targets

Maio

Kong²,

Yip³

et al. 2013

Surg Neurol Int

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Background:Chordomas of the skull base are rare locally aggressive neoplasms with a predilection for encapsulating critical neurovascular structures, bony destruction and irregular growth patterns, and from which patients succumb to recurrence and treatment failures.Methods:A review of the medical literature is performed, using standard search engines and identifying articles related to skull base chordomas, surgery, radiation therapy, chemotherapy, molecular genetics, and prospective trials.Results:A synthesis of the literature is presented, including sections on pathology, treatment, molecular genetics, challenges, and future directions.Conclusion:Beyond an understanding of the current treatment paradigms for skull base chordomas, the reader gains insight into the collaborative approach applied to orphan diseases, of which chordomas is a prime exemplar.

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“…Most of these drugs have shown modest activity in case reports and case series, administered either as monotherapy or combined with other agents [7–13]. A dedicated phase 2 study of lapatinib in EGFR-positive chordoma resulted in a progression free survival (PFS) of 8 months [14].…”

Section: Introductionmentioning

confidence: 99%

Efficacy of pazopanib and sunitinib in advanced axial chordoma: a single reference centre case series

2016

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BackgroundChordomas are rare malignant tumours of the axial skeleton and skull base supposed to arise from cellular remnants of the notochord. These tumours have the potential to metastasize (30–40 %), usually in the later course of the disease. However, the greatest morbidity is usually a result of loco-regional recurrence with infiltration and destruction of surrounding bone and soft tissue. Patients with unresectable or metastatic chordoma are faced with a poor prognosis since cytotoxic chemotherapy or other systemic therapies have not proven their efficacy yet. However, several molecularly targeted drugs have been proposed as potentially beneficial, including tyrosine kinase inhibitors (TKIs) directed at vascular endothelial growth factor receptor (VEGFR), like pazopanib and sunitinib.Case presentationFive patients with unresectable or metastatic chordoma were treated with VEGFR inhibitors pazopanib or sunitinib in the Leiden University Medical Centre (LUMC) between 2008 and 2015. Two out of four patients treated with pazopanib derived clinical benefit and disease remained stable for respectively 14 and 15 months. The one patient treated with sunitinib achieved a partial response according to RECIST 1.1 which lasted for a total of 27 months. No serious adverse events were observed.ConclusionThese results on the use of pazopanib and sunitinib in chordoma are promising, with an objective response on sunitinib and a median progression free interval of 8.5 months (range 3–15 months), comparable to that of imatinib, in the pazopanib subgroup. However further research is needed to assess the definite role of VEGFR inhibitors in chordoma.

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Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review

Cited by 50 publications

References 17 publications

ErbB Receptors as Prognostic and Therapeutic Drug Targets in Bone and Soft Tissue Sarcomas

ErbB Receptors as Prognostic and Therapeutic Drug Targets in Bone and Soft Tissue Sarcomas

Converging paths to progress for skull base chordoma: Review of current therapy and future molecular targets

Efficacy of pazopanib and sunitinib in advanced axial chordoma: a single reference centre case series

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