Abstract

BackgroundChordoma is a rare, slow growing and locally aggressive mesenchymal neoplasm with uncommon distant metastases. It is a chemo-resistant disease with surgery and radiotherapy being the mainstay in treatment of localized disease. In advanced disease imatinib has a role. We report a case of metastatic sacral chordoma with symptomatic and radiological response to erlotinib post-progression on imatinib.Case presentationA 48-year-old male with a sacral chordoma underwent partial sacrectomy followed by post-operative radiotherapy. Upon recurrence he received palliative radiotherapy to hemipelvis and was offered therapy with imatinib. However, the disease was refractory to imatinib and he was started on treatment with erlotinib—showing a partial response on imaging at two months. He is currently doing well at 13 months since start of erlotinib.ConclusionsAs seen in previously reported cases, erlotinib is a therapeutic option in advanced chordoma, even in imatinib refractory cases and thus warrants exploration of its therapeutic role in prospective clinical trials.

Highlights

  • Chordoma is a rare, slow growing and locally aggressive mesenchymal neoplasm with uncommon distant metastases

  • As seen in previously reported cases, erlotinib is a therapeutic option in advanced chordoma, even in imatinib refractory cases and warrants exploration of its therapeutic role in prospective clinical trials

  • The biological rationale of using imatinib comes from its inhibition of PDGFRβ which is expressed on chordomas

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Summary

Introduction

Slow growing and locally aggressive mesenchymal neoplasm with uncommon distant metastases. We present a case of metastatic sacral chordoma that has shown response to erlotinib after having progressed on imatinib.

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