Chordoma In A Rare Site

Author

Abdulrahim H. Zwayed

Yemen-Sana’a – PO Box 4598

Email: aralmaliky@yahoo.com

Abstract
Chordomas are rare tumours arising from notochord cell nests. They arise throughout the craniospinal axis but occur predominantly at the ends of the axial skeleton in: the basioccipit and sacrococcygeal regions. Here we report a case of 4th cervical spine involvement, and discuss the clinical picture, radiological findings and surgical procedure.

Keywords
Chordoma, Cervical spine, Vertebral Chordoma, Spinal axis.

Introduction
Chordomas are rare neoplasms – as primary intracranial neoplasms, they only constitute 0.2% of all CNS tumours; however, they constitute 2-4% of all primary bone neoplasms. Chordomas generally occur in 3 locations, which are, in descending order of frequency, the sacrum, intracranially at the clivus, and along the spinal axis. So for this case the chordoma located in the cervical spine is regarded as a rare place. Chordomas arise from embryonic notochordal remnants along the length of the neuraxis at developmentally active sites. We briefly present this case of chordoma, discussing the symptoms and signs, the radiological features and management including excision and replacement of the defect with bone graft. The patient was discharged well without any significant complaints.

Case report
A 37-year-old lady presented at our neurosurgical outpatient clinic with three month’s history of continuous neck pain mostly at anterior aspect, and to less extent at the posterior region, radiated to both upper limbs, on the left more than the right with gradual weakness of the left upper arm since the last 6 weeks. Her complaint was associated with painful neck movement, but no other significant complaints (no hoarseness, or dysphagia, or any pharyngeal bleeding).

On examination: The patient was conscious but with mild hemiplegic gait.

The vital signs were normal apart from low-grade fever.

Babinski`s and Hoffmann signs were present bilaterally.

Pain and temperature were reduced from C4 dermatome and below but vibration and joint position senses were normal.

On spinal examination, there was tenderness in the neck at cervical region but no swelling or other abnormal signs.

Sphincters within control Plain x-ray showed bone destruction of fourth cervical spine body.

Contrast-enhanced sagittal computed tomography (CT) scan images demonstrate an area of decreased attenuation within vertebrae, with destructive or lytic lesions in the bone and spinal cord compression at that level.

The MRI Scan revealed a mass in the fourth cervical spine body compressing the spinal cord antero-posteriorly and laterally on the left side.

The patient was admitted to the Neurosurgical unit and prepared for operation on the next day.

The operation was done in supine position and neck extension with the aid of T.V. screening.

A left cervical oblique incision is applied at the anterior triangle, muscle splitting, and a mass from the cervical spine body was totally excised, with bone graft at the defect area (methyacrylate).

The patient was kept in the intensive care unit for about 48 hours for possible respiratory problems then referred to the neurosurgical unit, with encouraging physiotherapy.

The patient stayed in the hospital for up to 8 days where stitches was removed then discharged with good state (i.e. full functional and able to work but slight neurological deficits).

Complete recovery was noticed after 3 months.

Grossly, the mass was an oval shape of about 4 x 5 cm. in size, soft, gelatinous, with a little pit lobulated and was gray-white in color on its outer surface.

On cut section, the tumour was homogeneous in color and consistency, and there was no clear-cut capsule.

Then the patient was sent for deep X-ray therapy with around 20 sessions to prevent recurrence if any. She was followed for three years and no signs of recurrences were documented.

Discussion
Chordomas are thought to arise from primitive notochordal remnants along the axial skeleton. During development, the notochord is surrounded by the developing vertebral column. In adults, remnants of the notochord are present as the nucleus pulposus of the intervertebral discs.

Notochordal remnants that are extradural are most common at the sacrococcygeal region but can be found at any site along the length of the axial skeleton. The distribution of tumours matches the distribution of notochordal remnants.^(1,2,10)

Even though chordomas usually are slow-growing tumours, they are locally aggressive with a tendency to infiltrate into adjacent tissues and organs. Local recurrence results in tissue destruction and generally is the cause of death. Metastases are recognized but are uncommon.⁽¹¹⁾

When considering all locations, the male-to-female ratio is 2:1. However, skull base tumours, as a subgroup, tend to have a more equal sex distribution. A number of reports indicate that chordomas are seen in all age groups, with the peak incidence varying by site. Intracranial chordomas present in a much younger age group than their spinal counterparts because the relevant anatomy of the clival region produces earlier symptomatology. In one series of chordomas reviewed, the average age at diagnosis of all patients with chordomas was 56 years, with an age range of 27-80 years. ⁽¹³⁾

The clinical presentation is entirely dependent on the location of the chordoma. At the sacrum, common presenting symptoms are back and/or lower extremity pain. About one half of patients with Chordoma have autonomic symptoms, particularly rectal dysfunction or urinary incontinence. About one half of patients with chordomas have a palpable sacral mass.^(1,13)

With intracranial tumours, the most common presenting symptoms are diplopia and headache. Neurological signs also occur in over one half of the patients, primarily as cranial nerve palsies. Palsies of cranial nerve VI, the sensory branch of V, and the sensory branch of III are the most common.^(2,4,8)

Patients with tumours located along lower vertebrae may present with pain, bladder dysfunction, or lower extremity weakness. Patients with tumours located along cervical vertebrae present with hoarseness, dysphagia, and, occasionally, pharyngeal bleeding. Other rare or unique symptoms have been reported but are the exception. The time span from the onset of symptoms to diagnosis averages 10 months.^(4,7)

Plain films (x-ray) may be useful to demonstrate the amount of bone involvement.^(10,12)

CT scan or MRI studies are indicated to evaluate the extent of the tumour and to identify the tissues that the chordoma has infiltrated. Knowledge of the extent of the tumour is important in planning the optimal surgical approach.^(10,12,14)

With CT scans, chordomas at any site appear as single or multiple areas of decreased attenuation within the clivus, vertebrae, or sacrum. Fingers of low density radiate throughout the mass and into the adjacent tissues. If the chordoma has a significant chondroid component, focal regions of hyperdensity may be present. The lesions are expansile with destructive or lytic lesions in the bone. ^(1,10,14)

On MRI, the appearance of a chordoma is similar to the appearance on CT scan, with better resolution of the soft-tissue component, resulting in better anatomical definition. Chordomas are hyperintense on T2 images and hypointense on T1 images. ^(10,14)

Biopsies of chordomas are useful only when other bone lesions remain in the differential diagnosis after imaging studies are performed. In this instance, tissue diagnosis by biopsy can enable optimal planning for surgical resection of the tumour. Fine needle aspiration (FNA) is the preferred method for establishing the preoperative morphologic diagnosis of chordoma and has been reported to lower local recurrence rates when compared with open biopsy. ⁽⁵⁾

Surgical therapy for these tumours is indicated as they continuously grow, albeit slowly, and erode bone and adjacent soft tissue, causing marked destruction of surrounding tissues. ^(1,8,13)

Surgical resection remains the primary mode of treatment for both diagnostic and therapeutic purposes. ^(1,8,9)

The prognosis of chordomas generally depends on the extent and completeness of the tumour excision. ^(1,8)

Microscopically, chordomas are composed of uniform cells with small oval or round eccentric nuclei and dense chromatin. The hallmark microscopic features of chordomas are the numerous, variably sized vacuoles located in the tumour cell cytoplasm, the physaliphorous cells. Some tumour cells may have more solid or eosinophilic cytoplasm. Mitoses, foci of pleomorphic cells, or focal hemorrhage rarely can be seen but are not prominent features. Fibrous tissue surrounds the neoplasm and extends projections into the tumour, usually without forming a true capsule.^(3,5,9)

A chondroid variant of chordoma is well recognised. In these tumours, a significant cartilaginous component is present with features of either chondrosarcomas or chordomas. Some authors believe these entities are separate and that studies with both immunoperoxidase staining and electron microscopy can distinguish them. Also, patients with this variant were once thought to have a slightly better prognosis; however, recent large studies have shown this variant to be of no prognostic significance. ^(3,9,12)

Immunohistochemically, the tumour cells label with cytokeratins and epithelial membrane antigen (EMA). Both chordomas and the embryologic notochord are S-100 positive, whereas most carcinomas are negative. This difference in S-100 positivity can be helpful in the differentiation of metastatic carcinomas from chordomas in instances in which the histologic pattern is similar. Positivity for cytokeratins and EMA can be helpful in distinguishing the chondroid variant of chordoma from chondrosarcoma. ^(3,5,9)

Clinical trials are underway to study the effectiveness of imatinib mesylate in the treatment of chordoma. This drug has been shown to have antitumour activity in chordomas; however, research is ongoing and surgery remains the standard treatment for chordomas. Adjuvant radiation therapy is used in cases where incomplete resection is suspected. Chemotherapy has not been shown to be effective. ⁽⁶⁾

Radical resections of tumours with clean margins are associated with a longer disease-free interval. If subtotal excision is the only option (generally due to location and proximity to delicate anatomy), the addition of radiation therapy can lengthen the interval to recurrence. In cases in which radiation therapy is utilised without surgical resection, an average of only 50% for 10-year local control is seen for skull-based and cervical spine tumours. ^(1,13,15)

Rehabilitation may be necessary in the case of sacral surgeries, depending on the extent of damage to the spinal cord and the level of presurgical functioning. ^(2,13)

Frequent follow-up is required because of the high rate of recurrence of these tumours. Tumour recurrence identified early is easier to treat. The average interval to recurrence is 3.8 years for radically resected tumours, 2.1 years for subtotal resection followed by radiation therapy, and 8 months for subtotal excision without adjuvant therapy. The interval of follow-up, including repeat MRI or CT scans, depends on the completeness of the resection. Because residual tumour drastically shortens the recurrence time, patients with known or suspected residual tumour need to be evaluated more frequently. ^(1,12)

Complications occur at a higher rate after radical resections than with subtotal resections and depend somewhat on the location of the tumour. ^(1,2,14)

Morbidity from surgery can be very mild or severe following tumour resection. With the resection of sacrococcygeal chordomas, bowel and bladder dysfunction are the most frequent complications. ^{(2, 5)}

The 5-year survival rate is estimated to be 51%, and the 10-year survival is estimated to be 35%. Factors that may improve prognosis are young age, complete resection, and the addition of radiation therapy in incompletely resected tumours. ^(5,14)

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