ABSTRACT
Chordomas are rare tumors which arise from the embryological remnants of the notochord. These tumors can potentially arise from any region within the craniospinal axis and often clinically present as a diagnostic challenge. Chordomas are rare in patients younger than 40 years of age. The most common primary cancers that metastasize to the jaw bones are the ones originating from the breast, lung, kidney adrenal, colo-rectum, or prostate. Mandibular metastasis from a primary chordoma is an extremely rare occurrence with only five prior reports, three originating from primaries in the sacrococcygeal region, one from a lumbar spine primary and the other from a primary arising from the spheno-occipital region. A literature review did not reveal any prior reports of mandibular metastasis at presentation from a clival chordoma. We possibly report the first case of such an unusual clinical scenario in a 7-year-old male child and further discuss the evaluation and management of these rare tumors