Clival Chordoma Syndrome

Clival Chordoma 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.

What are the Symptoms of Clival Chordoma

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.

Ataxia, dysphagia, visual disturbance, hoarseness, and extremity weakness also commonly occur.

Common Symptoms Associated With Clival Chordoma

Headache

Facial numbness

Facial pain

Facial paresthesias

Diplopia

Dysarthria

Dysphagia

Ataxia

Extremity weakness

Hoarseness

Visual disturbance

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.

Occasionally, papilledema and optic nerve atrophy are identified.

How is Clival Chordoma diagnosed?

Magnetic resonance imaging (MRI) of the brain and brainstem should be performed in all patients thought to have clival chordoma. MRI of the brain provides the best information regarding the cranial vault and its contents.

MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease.

Magnetic resonance angiography (MRA) may be helpful in identifying aneurysms responsible for neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice.

Clinical laboratory tests consisting of a complete blood cell count, automated chemistry profile, and erythrocyte sedimentation rate are indicated to rule out infection, temporal arteritis, and other malignancies that may mimic clival chordoma.

Endoscopy of the nasopharynx and hypopharynx with special attention to the piriform sinuses also is indicated to rule out occult malignancy.

Differential Diagnosis

Clival chordoma is generally a straightforward clinical diagnosis in retrospect. Given that the time between the onset of neurological signs and symptoms and definitive diagnosis is an average of 2 years, a high index of clinical suspicion is necessary to avoid misdiagnosis.

Obtaining a targeted history and performing a careful physical examination are essential. Diseases of the eye, ears, nose, throat, and teeth may mimic trigeminal neuralgia or may coexist and confuse the diagnosis.

Tumors of the nasopharynx and hypopharynx, including the tonsillar fossa and piriform sinus, may mimic the pain of clival chordoma, as may tumors at the cerebellopontine angle. Occasionally, demyelinating disease may produce a clinical syndrome identical to clival chordoma.

The jaw claudication associated with temporal arteritis also sometimes confuses the clinical picture, as does trigeminal neuralgia.

Treatment

Treatment of clival chordoma requires surgery, radiation therapy, or both. Although clival chordomas are almost always benign and rarely metastasize, the critical location of clival chordomas relative to adjacent neural structures makes both forms of treatment challenging.

Often, complete tumor resection is impossible because of the location, and postoperative radiation therapy, gamma knife stereotactic surgery, and implantation of radioactive seeds may be required.

Complications

Because of the slow-growing nature of clival chordomas, delayed diagnosis is an ever-present possibility complicating an already difficult treatment regimen.

Further confusing the clinical presentation of this tumor of embryological origin is the fact that many of the clinical syndromes that mimic the signs and symptoms of clival chordoma are also difficult to diagnose.

Clinical Pearls

Clival chordoma is a rare neoplasm that is usually benign, although aggressive clival chordomas have been reported. Clival chordomas tend to be slow growing and produce symptoms by compression of the adjacent brainstem and cranial nerves.

In spite of this fact, 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.