Abstract

Patients presenting with neck complaints, such as pain or stiffness, are not uncommon in the Emergency Department. Complaints of neck instability, however, are unusual. We report the case of a 30-year-old woman who presented with multiple neck complaints that included having a “wobbly” sensation of her neck on flexion, feeling as if it were unstable. Our patient indeed had atlanto-occipital instability secondary to a locally destructive tumor of the cranial base, known as a clival chordoma. Chordomas are rare and unique bony tumors that arise along the neural axis and are thought to originate from the nucleus pulposus. The tumors are slow growing; locally invasive; and cause a variety of neurologic, musculoskeletal, cranial, and neck complaints. We describe this unique case and its presentations in an attempt to increase the sensitivity of physicians in early detection of this rare and lethal tumor.

Chordomas are very rare tumors that are thought to originate from the embryologic remnants of the notochord, the nucleus pulposus. Arising along the neural axis, chordomas most often arise from the sacrococcygeal area and the clivus region in the cranial base. ¹ The localized invasiveness of these tumors results in a variety of musculoskeletal and neurologic impairments and/or complaints at the time of clinical presentation. ^2–7 In this case report, we describe a case of clival chordoma that at presentation had gone undiagnosed and therefore untreated for an extended period of time. The tumor had grown large enough to erode most of vertebral bodies C1 and C2, causing atlanto-occipital instability.