ICD-10 CODE D16.4
The Clinical Syndrome
Clival chordoma is a rare neoplasm that arises from embryological remnants of the notochord along the spinal axis. Clival chordomas are usually benign, although aggressive clival chordomas have been reported. Comprising one-third of central nervous system chordomas, clival chordomas tend to be slow growing and produce symptoms by compression of the adjacent brainstem and cranial nerves. In spite of this, the long-term outcome of patients diagnosed with clival chordoma remains poor because of the location of these tumors and their tendency to recur regardless of the treatment method chosen. Clival chordomas can occur at any age, further complicating the diagnosis. These uncommon tumors occur slightly more often in men. Early diagnosis of clival chordoma is important to avoid acute neurological disasters; however, because of the slow growth of these tumors, the average time from onset of symptoms to diagnosis averages 2 years.
Signs and Symptoms
Headache is the most common presenting complaint in patients with clival chordoma. Other common symptoms associated with clival chordoma reflect the propensity of this tumor to compress adjacent neural structures, causing facial pain, facial numbness, facial paresthesias, and diplopia ( Table 21.1 ). Ataxia, dysphagia, visual disturbance, hoarseness, and extremity weakness also commonly occur.
Findings on neurological examination (e.g., cranial nerve deficits, pyramidal tract dysfunction, hemiparesis, hyperreflexia, clonus, a positive Babinski sign, and cerebellar signs, including ataxia) also reflect compression of neural structures by this slow-growing tumor ( Fig. 21.1 ). Occasionally, papilledema and optic nerve atrophy are identified.