Large secondary chordoma of maxilla : metastasis or surgical pathway recurrence ? : presentation of a case and review of literature

Abstract EN

Chordomas are rare malignant tumors arising from embryonic remnants of notochord.

The usual location is the axial skeleton, with the commonest sites being, clivus and sacrococcygeal region.

Naso maxillary region is a very rare site for chordomas.

We present a case of chordoma in Cranio Maxillofacial Surgery of the clivus treated with surgery and radiotherapy ten years previously, who reported with an extensive secondary lesion in the maxilla and nasal cavity.

Surgical management of this case is demonstrated.

The question of whether the maxillary lesion represents a metastasis or a surgical pathway recurrence is discussed.

The factors such as the amount of normal tissue between the original tumor and the secondary lesion, presence or absence of recognizable lymphatic or perineural pathway of spread, presence or absence of other metastatic lesions are important in distinguishing marginal recurrences from surgical pathway recurrences.

Modifications of surgical, chemotherapy (2) and radiotherapy regimens are considered in the light of the aforementioned factors.

Intruduction : The spectrum and clinicopathological features of nasopharyngeal tumors of bone and cartilage are similar to those of other sites in the upper erodigestive tract, except for chordoma, which typically presents in the nasopharynx.