Chordoma is a rare slow-growing neoplasm arising from the embryonic notochordal remnants inside the bones of the skull base or spine, most commonly in the clivus or sacrum. It usually recurs after surgery and may metastasize.
A 54 years old male with abruptly installed headaches, followed by ophthalmoplegia in the right eye, was diagnosed with a large midline tumor with heterogeneous enhancement invading the clivus and the sellar wall; histologically it was a chordoma. After transsphenoidal incomplete surgery and gamma knife radiosurgery, the headaches and the oculomotor nerve paresis receded, the pituitary function was normal but the tumor re-grew (MRI, T1 sagittal view Figs 1 a, b). He underwent a second transsphenoidal surgery, then fractionated proton radiotherapy (RT, total dose 54 Gy in the tumor area + 17 Gy as a boost in the shrinking field) (Fig. 1c, MRI T2 sagittal view before RT). The tumor was reduced and did not re-grow in the next 2.5 years; 8 months after RT a progressive paresis of the right oculomotor nerves III and IV occurred, along with a partial paresis of the right arm and leg, and subsequent panhypopituitarism, likely due to RT side-effects on the surrounding brain tissue.
Figure 1 a, b.
Clivus chordoma before second surgery, MRI T1 sagittal view, c. MRI T2 sagittal view, before proton radiotherapy