Cervical spine chordoma with retropharyngeal extension. The authors present a case of an extensive upper cervical chordoma treated by en bloc resection, reconstruction, and long. About 300 people are diagnosed with chordoma in the united states each. The evidence for this is the location of the tumors along the neuraxis, the similar immunohistochemical staining patterns, and the demonstration that notochordal cells are preferentially left behind in the clivus and sacrococcygeal regions when the remainder of the. In four of these families duplication of the brachyury gene was found to be responsible for causing chordoma. Sagittal t2weighted mri showing hyperintense huge cervical mass with an intimate. Upper cervical spine chordoma tumors are extremely rare. Chordomas of the base of the skull and upper cervical spine.
However, achieving a favorable cancerous result with free margins in cervical chordomas is very difficult due to a number of anatomical factors including 1 the. Chordomas grow slowly, gradually extending into the bone and soft tissue around them. The stephan harris chordoma centers growing patient database offers extensive opportunities for analysis and discovery. In addition, ongoing clinical trials test new therapies that enable our researchers to better understand and treat chordoma, which offers additional hope for cure. Chordoma is a kind of cancer that grows in the bones of your skull and spine. Since most chordomas are stage ib at diagnosis, margin free excision means resection of paravertebral muscle, nerve root, vertebral artery, even dura or retropharyngeal mucosa. The wider the tumorfree margins the lower the chances of recurrence. Pdf upper cervical spine chordoma of c2c3 researchgate. Ongoing communication with the patient, family, and the healthcare team about the diagnosis, surgical intervention required, and the plan of care is essential. One of the new advances in chordoma treatment is immunotherapy. In one study, the 10year tumor free survival rate for sacral chordoma was 46%.
The symptoms become evident by the compression of the chordoma to the adjacent structures. Pdf we report an extremely rare case of cervical chordoma presenting with impaired voice. I have deficits caused by craniotomies and treatments making it difficult to speak clearly, in addition to other side effects. Isolated cervical extraosseous intradural chordoma attached to the. Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Sphenooccipital the clival region is the second most common location, accounting for 3035% of cases 2,3. Pdf chordoma is a rare lowgrade malignant neoplasm derived from the remnants of. Upper cervical spine chordoma of c2c3 article pdf available in european spine journal 183. Atlantoaxial chordoma in two patients with occipital. Raoulanddiss15 seem to becredited for the first case of lumbar chordoma reported. Cervical chordoma, arising in the cervical spine, grows progressively into the soft tissue of the neck. Chordoma is a rare slowgrowing neoplasm thought to arise from cellular remnants of the notochord. Chordoma arising from the cervical spine is rare and the traditional longterm prognosis is typically poor. Surgical management of chordomas of the cervical spine article pdf available in journal of neurosurgery spine 65.
Chordoma begins in cells that once made up a collection of cells in the developing embryo that go on to become the disks of the spine. To the individual afflicted with this diagnosis, aggressive neurosurgical and radiotherapy treatments are warranted. However, this method is still challenging for the cervical spine because of the proximity of the tumor to the vertebral. Total en bloc spondylectomy with a wide margin is generally accepted to be the most appropriate management for thoracic and lumbar malignant tumors. En bloc resection of multilevel cervical chordoma with c2 involvement. Upper cervical tumors often are accidentally discovered during investigation of cervical radiculopathy.
As chordomas are slow growing and locally invasive with high recurrence rates, initial recommendations include complete surgical resection. Pdf surgical management of chordomas of the cervical spine. Chondroid chordomas appear to have a more indolent clinical course. I have recurred from skull to cervical spine and latest the lung in 20. Cervical spinal chordoma with chondromatous component in a dog. Chordoma is a relatively rare tumor that primarily occurs in the axial skeleton. A chordoma is a rare type of cancerous tumor that can occur anywhere along the spine, from the base of the skull to the tailbone. En bloc resection of multilevel cervical chordoma with c2. Pdf cervical chordoma with retropharyngeal extension. In russia, the annual incidence of chordoma is approximately 1 in one million. Chordoma is a rare bone neoplasm 14% of bone malignancies, which originates from the embryonic notochord. Among head and neck sites, the majority arise in the skull base with a small minority arising along the cervical spine. Approximately 6% of chordomas arise from the cervical spine as a slowly growing tumor, which makes the tumor symptomatic at relatively later stages 3,4.