Neck pain: common complaint, uncommon diagnosis--symptomatic clival chordoma.

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.

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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.

Discussion

Chordomas are bony tumors that are thought to arise from the remnants of the notochord, an embryologic structure that precedes the development of the axial skeleton. (2) Remnants of the notochord are found in the nucleus pulposus and in the clival bone marrow. Chordomas are rare tumors, occurring with an incidence of 0.2 to 0.5 per 100,000 population, constituting 1% of intracranial tumors and 3% of all primary bone tumors. (2), (5) There is a slight male preponderance, and the average age of incidence is between the third and fifth decades of life, occurring more often in the latter. Chordomas arise most often in the sacrococcygeal area and occur second most frequently in the base of the skull. In children, sacrococcygeal chordomas are the most common. Due to the nature of this case, we have limited our discussion to chordomas of the cranial base (clival chordomas).

Although most chordomas are histologically benign and rarely metastasize, they should be considered clinically malignant due to their critical location to vital structures and their locally aggressive nature. Chordomas can be identified with special stains for such components as epithelial membrane antigen, [alpha]-fetoprotein, and vimentin. (2) Three histologic subtypes, typical, atypical, and chondroid, have been described. Prognosis is directly related to the histologic subtype. Tumors with chondroid elements portend a better outcome, with the atypical subtype carrying the worst outcome. Unfortunately, atypical histology is more common in younger children, with a more aggressive nature and a higher incidence of metastasis. (3) Chordomas are slow growing and present insidiously. Most patients experience symptoms for an extended period of time before diagnosis, averaging 2.5 to 4.4 years. (1), (5) Symptomatic presentation is dependent on the location of the tumor and is represented by compression or traction of neighboring structures and distal effects of hydrocephalus and vascular compromise. Symptoms are often vague and fluctuate for years before becoming more focal and rapidly progressive. (1) Common presenting complaints include headache (occipitocervical); diplopia; and symptoms attributable to involvement of the other cranial nerves such as ptosis, facial paresis, hearing loss, vertigo, and visual loss. Patients also complain of mass effects of the tumor including dysphagia and nasal obstruction. In-depth investigation is warranted in anyone complaining of a wobbly or unstable neck, because this could be a clinical sign of major destruction of the upper cervical spine. Transient symptoms due to vascular involvement include paralysis, paresthesias, and visual loss. (1), (2), (5) Physical examination findings commonly include cranial nerve palsies such as visual field deficits and extraocular muscle dysfunction. Up to half of the patients may have a palpable retropharyngeal mass. Less common signs are gait disturbances and sensory deficits. Patients with occipital headaches aggravated by motion may hold their head still and in a fixed position, resembling the clinical symptoms of torticollis. (1)

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Radiologic diagnosis uses multiple imaging modalities. Plain films can reveal areas of bony destruction and calcification and are a good screening tool. Computed tomographic scanning with bone windows reveals areas of bone destruction and foci of calcification. Magnetic resonance imaging has been vital in delineating the extent of the tumor and structures involved. These tumors enhance well with gadolinium, are isointense with T1-weighted images, and show a bright hyperintensity with T2-weighted images. (1), (2)

The mainstay of treatment for chordomas is surgery. Total resection of the tumor offers a theoretical cure. Unfortunately, most patients present when the tumor is too large and has already compromised vital structures, making total resection impossible. In most cases, the best surgical treatment that can be offered is partial resection. Postoperative radiation has been used with mixed results, with the best results seen from proton beam radiotherapy. The prognosis without treatment is extremely dim, ranging from 0.6 to 2.2 years. Even with surgery, prognosis is poor, with average survival times ranging from 5 to 7 years. (2)

Conclusions

The clinical presentation of patients with chordomas can often be misleading and confusing, because patients can present with a variety of symptoms that can fluctuate over time. Although rare, it should be considered in the differential diagnosis of patients presenting with occipital headaches and complaints attributable to cranial nerve compression and/or gait disturbances. Patients complaining of neck instability or wobbly neck, as in this case, should have lateral cervical spine films thoroughly reviewed for evidence of soft tissue masses or bony destruction, as early detection is the goal. Plain radiography is a good screening tool and may prevent a delay in the diagnosis of this deadly tumor. Once diagnosis has been established, it should not be forgotten that recurrence is the rule, even with total resection (although complete removal has a longer recurrence-free survival rate). With an increased index of suspicion, clival chordomas may be diagnosed early in their course, which in turn favors a decreased morbidity. (4), (6), (7)

As to your method of work, I have a single bit of advice, which I give with the earnest conviction of its paramount influence in any success which may have attended my efforts in life--Take no thought for the morrow. Live neither in the past nor in the future, but let each day's work absorb your entire energies, and satisfy your widest ambition.

--William Osler

From the Division of Emergency Medicine, Department of Surgery, University of Texas Health Science Center at San Antonio, San Antonio, TX.

Reprint requests to Charles P. Davis, MD, PhD, Division of Emergency Medicine, Department of Surgery, University of Texas Health Science Center at San Antonio, 7703 Floyd Curl Drive, San Antonio, TX 78284-7702. Email: [e-mail]

Accepted April 28, 2003.

Copyright [c] 2004 by The Southern Medical Association 0038-4348/04/9701-0083

References

(1.) Menezes AH, Traynelis VC. Tumors of the craniovertebral junction, in Youmans JR (ed): Neurological Surgery: A Comprehensive Reference Guide to the Diagnosis and Management of Neurosurgical Problems. Philadelphia, W.B. Saunders, 1996, Vol 04, ed 4, pp 3041-3072.

(2.) Sekhar LN, Gay E, Wight D. Chordomas and Chondrosarcomas of the Cranial Base, in Wilkins RH, Rengachary SS (eds). Neurosurgery. McGraw-Hill Health Professions Division, 1996, vol 02, ed 2, pp 1529-1542.

(3.) Borba LA, Al-Mefty O, Mrak RE, et al. Cranial chordomas in children and adolescents. J Neurosurg 1996;84:584-591.

(4.) Maira G, Pallini R, Anile C, et al. Surgical treatment of clival chordomas: The transsphenoidal approach revisited. J Neurosurg 1996;85:784-792.

(5.) Mizerny BR, Kost KM. Chordoma of the cranial base: The McGill experience. J Otolaryngol 1995;24:14-19.

(6.) Tai PT, Craighead P, Bagdon F. Optimization of radiotherapy for patients with cranial chordoma: A review of dose-response ratios for photon techniques. Cancer 1995;75:749-756.

(7.) Swearingen B, Joseph M, Cheney M, et al. A modified transfacial approach to the clivus. Neurosurgery 1995;36:101-105.

RELATED ARTICLE: Key Points

* Clival chordoma should be suspected in any patient with occipitocervical headaches, a "wobbly" neck, dysphagia, and/or cranial nerve deficits.

* Careful evaluation of radiologic films for soft tissue masses is essential to early diagnosis.

* Even though total resection of this tumor is the goal, recurrence of this destructive tumor is the rule.

* Chordomas are histologically benign with rare metastasis but should be considered malignant due to their locally aggressive/destructive nature in close proximity to vital structures.

RELATED ARTICLE: Case Report

A 30-year-old Hispanic woman presented to the Emergency Department with a chief complaint of a wobbly neck on flexion and a 2-year history of neck stiffness. She stated that her neck discomfort began 2 years previously, when she began to experience decreased range of motion and occasional pain on attempting to rotate her head to the right. She also reported that 1 year previously she began to experience moderate pain on rotation of her neck to the left, during which acetaminophen was used to alleviate her pain. The patient also described intermittent dysphagia when attempting to swallow large bites of solid food. One previous episode of tongue deviation to the left, for an unspecified amount of time, was noted that resulted in spontaneous resolution. The patient attributed these symptoms to the stress of raising her children and did not seek medical attention for a period of approximately 2 years, coinciding with the onset of earliest symptoms. She reported that 3 months before this presentation a physician in Mexico evaluated her complaints. On review of her radiographs, he suggested to the patient that she see an undescribed "specialist." The patient did not comply and did not seek further evaluation until she felt her symptoms had worsened to include the "wobbly" sensation she was currently experiencing with the pain. On interview, she denied any paresthesias or distal pain on neck movement. The patient also denied any weakness in her neck or extremities and/or decreased sensation. No history of chronic headache or constitutional symptoms of fever, nausea, or vomiting were described by the patient. The predominant physical findings were marked decreased passive cervical range of motion in all dimensions with diffuse muscle spasm. Our patient did report some mild numbness in her distal fingers bilaterally; however, no overt neurologic deficits were evident. No obvious neck masses or tracheal deviation were noted.

Lateral cervical spine radiographs revealed an indistinguishable cloudy region, a poorly visualized vertebral body C1 and odontoid process, and a partially visualized C2 (Fig. 1). In this case, this could have been secondary to the technique of radiographic methods, although a lytic/destructive lesion could not be ruled out. All visualized vertebral bodies were appropriately aligned, with no evidence of fracture or subluxation. An anterior soft tissue mass impinging on the oropharynx was also visualized on lateral cervical spine plain film (Fig. 1). Computed tomographic scan revealed massive bone erosion including the clivus and the majority of C1 and C2 with beginning erosion of C3 (Fig. 2).

A soft tissue density appearing to include the dens, retropharyngeal space, and the pharynx was noted. Due to the patient's cervical spine instability, she was immediately placed in a cervical collar. Magnetic resonance imaging revealed a large mass protruding from the brainstem through the former clival area (Fig. 3), resulting in significant compression of the brainstem and proximal cervical spinal cord (Fig. 3). The clival mass extended into the anterior neck, manifesting as a large bulge in the oropharynx, consistent with a clival chordoma. The patient was discharged with a follow-up appointment and operative date.

The patient's initial surgery involved oromaxillofacial surgery and neurosurgery. Oromaxillofacial surgery began by implementing a diverting tracheostomy followed by arch bars. Transoral and transmaxillary access was obtained by a LeFort Type I osteotomy with complete palatal split. The neurosurgical team then performed transoral resection and debulking. Postoperatively, the patient was placed in a halo and exhibited no neurologic deficits. The patient's hospital course between operations was unremarkable except for Pseudomonas-positive blood cultures, which were appropriately treated. The patient consequently underwent multiple surgical interventions, including posterior laminectomy, further tumor debulking, and metal stabilizers and harvested bone grafts to augment cervical stability. The chordoma was grossly resected in the midline down to the dura. However, significant portions of the tumor remained laterally due to the extensive nature of the tumor, as was expected. After these multiple operations, the patient was discharged without complication.

Ricardo Alvarado, BS, John Gomez, MD, Samuel G. Morale, MD, FACEP, and Charles P. Davis, MD, PHD