Background: Chordomas are rare tumors of the skull base and the spine. Treatment is difficult since conventional radiotherapy or chemotherapy have only limited effects.
Patient and methods: A patient with a sacral chordoma and pulmonary metastases received initial surgery and radiotherapy for a local recurrence. After 11 months another local recurrence as well as a progression of the pulmonary metastases was documented. As tumor biopsies revealed the expression of the EGF receptor an individual treatment approach with a combination of cetuximab and gefitinib was performed.
Results: Under the treatment with cetuximab/gefitinib the local recurrence and the pulmonary metastases showed a partial response over the follow-up period of 9 months. To date no treatment failure was observed.
Conclusions: The inhibition of the EGF pathway seems to be an effective measure in the treatment of a chordoma. Further follow-up will have to prove the long-term efficiency.