[Therapeutic management of craniocervical chordoma]

Chordomas are rare neoplasms of notochordal origin that arise along the vertebral axis. In the cervicofacial area, they show a marked proclivity for the sphenooccipital region. These slow-growing and infiltrating tumors are often discovered because of neglected symptoms related to the ENT field such as nasal obstruction, snoring, dyspnea or dysphagia in the case of anterior development, serous otitis media, cervical pain, or even palsy of the X, XI, or XII cranial nerves when the tumor develops toward the foramen jugulare or the foramen magnum. Prognosis is usually poor because of local malignancy, proximity to critical central nervous system structures, and volume of the tumor. Surgery is the preferred treatment for these extradural tumors, but most authors recommend postoperative irradiation because of surgical spillage or residual tumor. However, conventional irradiation is limited by the sensitivity of surrounding structures, which results in a poor rate of local control. We present 9 cases of histologically proven diagnosis of chordoma treated from 1984 to 1994 at our institution. Prognosis and therapeutic modalities are discussed. Therapeutic improvement might be brought the protontherapie, which ensures a better local control, and therefore may transform the prognosis of the disease.