All cell lines in the repository are extensively characterized to ensure that they are valid models of chordoma. Chordoma periphericum is a primary soft tissue chordoma and is very rare. One patient presented with a palpable neck mass and dysphagia, while the other presented with posterior neck pain. Thus, about 300 patients are diagnosed with chordoma each year in the united states and about 700 in all of europe. This is due to the tendency of chordomas to involve multiple. Even less radical resection case has ever been reported for atlantoaxial chordoma 11. Surgery for a chordoma weill cornell brain and spine center. Therefore complete removal may not be recommended, as it could cause damage to those nerves. The chordoma foundation works to accelerate the development of new treatments for chordoma, while helping chordoma patients get the best care possible. Some parts of the tumor are surrounded by fibrous tissue with clear diathermied edges.
Chordomas are rare, malignant bone tumors of the skullbase and axial skeleton. Mri is a useful adjunct to ct and is superior to ct in delineating the exact soft tissue extent of chordomas, as was clearly shown in the later part of the management of this case. Chordoma is rarely observed in the cervical spine, taking up only 6% of all chordomas 3. Chordoma arising from the cervical spine is rare and the traditional longterm prognosis is typically poor. These guidelines will help chordoma patients and their physicians make informed decisions that reflect the very latest standard of care. Chordoma, lesion derived from the notochord, represents about 4% of the primary malignant bone tumours. Chordomas of the cervical spine may cause variable neurological symptoms. Nov 27, 2017 the mainstay of treatment for chordoma is a combination of surgery with radiation and chemotherapy. Mri results of 11 patients with chordoma of the cervical spine in an. In most cases, the enneking stage is iblow grade malignant, extracompartmental. Chordoma is a rare form of primary bone cancer, known to affect only 1 individual per 800,000 people every year1. In the cervical spine they represent 5% of all chordomas. Brachyury may distinguish chordoma from histologic mimics, including clear cell renal cell carcinoma, chondrosarcoma, chordoid meningioma am j surg pathol 2009.
For patients with cervical spine chordoma, pain on neck flexion or rotation is most common. Pdf epidural chordoma of the cervical spine with secondary. Although the biological behavior of the chordomas is highly variable, most conventional chordomas are slow growing. Chordoma articles case reports symptoms treatment, argentina. Chordoma is a rare type of bone cancer that happens most often in the bones of the spine or the skull. Oct 26, 2018 chordoma of the spine causes symptoms like. The removal of a chordoma in the skull base and cervical spine neck, for example, may impact nerves that affect the eyes, face, and swallowing.
It is a tumor with lobules and vacuolated, moderately atypical, neoplastic cells. Synchronous infrasellar and cervical chondroid chordoma. Chordomas are uncommon malignant tumors of the axial skeleton that account for 1% of intracranial tumors and 4% of all primary bone tumors. Sometimes, chordoma has dumbbell shape which could be diagnosed as neuromas. The clinic radiologic features, anatomopathologic aspects, diagnostic difficulties, therapeutic methods and prognostic factors are discussed through a case report of a cervical spine chordoma with. Chordoma nord national organization for rare disorders. They also have a tendency for local recurrence since enbloc resection is rarely possible in these locations.
Adult patients presenting with neck pain, paresthesias or limb paresis are further investigated. Chordomas are challenging tumours which usually occur in the axial skeleton, and are particularly difficult to manage when they are situated in the clivus and upper cervical spine due to the proximity of eloquent structures. Sometimes it may be helpful to combine photon radiation and particle therapy. Total en bloc spondylectomy with a wide margin is generally accepted to be the most appropriate management for thoracic and lumbar malignant tumors. Chordoma is the only embryonic neoplasm that presents in the later decades of life, most typically in the fifth through seventh decades. Chordoma is a low grade malignant tumors accounting for about 1% to 4% of all malignant bone tumors. It is predominantly found in the clivus 50%, followed by the sacral spine 15%. It accounts for 14 % of all primary skeletal tumors, and its incidence rate is inferior to 1. Pdf chordoma is a rare lowgrade malignant neoplasm derived from the remnants of the embryonic notochord. In the cervical spine few cases have been reported of en bloc excision, due to anatomical constraints. The peculiar anatomical complexity of the cervical region includes the dens, the vertebral arteries, the transverse processes around and the nerve roots. Clinically, the initial symptom arises when the chordomas have already been progressed to a larger size with paravertebral or spinal canal extension and encapsulation of one or both vertebral arteries vas and nerve roots 15, 29. Those located in the sphenooccipital region most commonly occur in patients 2040 years of age, whereas sacrococcygeal chordomas are typically seen in a slightly older age group peak around 50 years 10.
Only rarely is this tumors seen in cervical spine accounting for 6% of all chordoma 1. Computed tomography and magnetic resonance imaging features of. Even if all of the visible chordoma is removed, microscopic cells may remain. This article focuses on chordoma tumors that affect the bones in the spine. Ongoing communication with the patient, family, and the healthcare team about the diagnosis, surgical intervention required, and the plan of care is essential. Expert recommendations for the diagnosis and treatment of. Feb 17, 2009 in the cervical spine few cases have been reported of en bloc excision, due to anatomical constraints. The reason as to how chordoma develops is that, there is a cartilage like structure in the fetus when the baby is developing which facilitates formation of the spine. They originate from embryonic remnants of the primitive notochord earliest fetal axial skeleton, extending from the rathkes pouch to the tip of the coccyx. Management of a cervical chordoma involving c7t1 sciencedirect. A case of c5 vertebral chordoma in a 73yearold patient with.
At any given time, fewer than one in 100,000 people are living with chordoma. Myxoid liposarcoma negative for ema and cytokeratins. Approximately 300 people in the united states are diagnosed with chordoma each. Oct 22, 2015 chordoma is a rare, lowgrade, primary malignant skeletal tumor. It accounts for 14% of all primary skeletal tumors and its incidence rate is inferior to 0. Chordomas most frequently arise in the spine or the skull, and the different locations of these tumour lead to varying symptoms. Chordomas can occur at any age but are usually seen in adults 3070 years. It is necessary to combine ct and mri examinations for patients with.
In contrast, t2wfse images missed 34 that were depicted on merge images. The 5year survival rate for patients with vertebral chordomas is approximately 50%, and with effective treatment the diseasefree interval at 5 years is 30 to 50% 3,12. In addition, the merge images only missed one lesion that was shown on the t2wfse images. Jan 28, 2010 chordoma is a rare, lowgrade, primary malignant bone tumour arising from primitive notochord remnants of the axial skeleton. Cervical chordoma present a high rate of recurrence given their location and growing in a highly risky anatomical region with complex anatomy. Additionally, they can be difficult to distinguish radiographically and pathologically from chondrosarcoma. First clinical practice guidelines developed for chordoma. A chordoma can develop anywhere along the spine from the base of the skull to the tailbone coccyx. Chordoma is a malignant tumor that usually involves the axial skeleton, and its origin is related to notochord remnants. Chordomas of the base of the skull and upper cervical spine. Mar 11, 2019 chordoma is diagnosed in just one person per million per year.
Thoracic and lumbar chordoma patients classically have local mechanical pain. Pdf chordomas are rare low to intermediate grade malignant tumours derived from the notochordal remnants. Chordomas of the base of the skull and upper cervical. A schematic description of the anatomy of the clivus and its surrounding structures is provided based on the modular classification of the surgical corridors used in endoscopic skull base surgery. Jul 12, 2016 clinically, patients with chordoma most often present with pain. Surgical management of chordomas of the cervical spine in.
The authors believe that, in most cases, en bloc resection of cervical chordoma is not feasible. Like the fse sequence, the merge sequence has a short acquisition time and the whole length of the cervical spinal cord can be scanned within a reasonable time usually 56 minutes. Upper cervical spine chordoma tumors are extremely rare. Chordomas grow slowly, gradually extending into the bone and soft tissue around them.
One hundred patients irradiated by a 3d conformal technique combining photon and proton beams. Radiation oncologychordoma wikibooks, open books for an. Myelopathy may be present if there is spinal cord compression. 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. Nov 10, 2016 a number of reports indicate that chordomas are seen in all age groups, with the peak incidence varying by site. They often recur after treatment, and in about 40 percent of cases the cancer spreads metastasizes to other areas of the body.
Jan 22, 2010 the majority of chordomas occur between the fifth and seventh decades of life and are thus extremely rare in children, which account for less than 5% of all spinal chordoma cases. 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. Chordomas are very rare bone malignant tumours that have had a shortage of effective treatments for a long time. The chordoma foundation maintains and supports repositories of validated chordoma cell lines and makes them available to researchers and companies interested in performing invitro experiments. Neuroscience volume 3 number 1 january 1996 cervical chordoma case report as characteristic. Lesions of the cervical spine are rare, comprising between 3 and 7% of all. The most common locations for a chordoma are at the triangular bone near the base of the spine sacrum, the coccyx, and the clivus, which is a bone in the base of the skull. Webmd explains what causes this cancer, and why it can be tricky to treat. Chordoma is a rare and slowgrowing type of bone cancer that may develop in the skull base andor at any level of the spinal columncervical, thoracic, lumbar, sacral, or coccyx tailbone. Intracranial chordomas present in a much younger age group than their spinal counterparts because the relevant anatomy of the clival region produces earlier symptomatology.
For spinal chordomas, the lower spinal level means the longer survival and the mortality rate of lumbar chordoma is better than that for thoracic or cervical lesions. Chordoma is the most common malignant primary bone cancer in. Chordoma is characteristically slowgrowing, locally invasive neoplasm. Chordomas of the cervical spine occur earlier than skull base. Large notochordal rest only evidence of destructive growth can identify a chordoma. Cervical spinal chordoma with chondromatous component in a dog. Dec 19, 2018 although surgery and radiation have long been considered the mainstays of chordoma treatment, this is the first time that expertdeveloped standards have been codified for the care of chordoma patients. However, this method is still challenging for the cervical spine because of the proximity of the tumor to the vertebral arteries and neural. Chordoma is a rare cancer that affects bones in the spine and skull. In 12 cases, the chordoma was localized in the cervical spine. Chordoma is 4th most frequent primary malignant tumor most patients are 40 to 70 years of age physaliferous cell is the most representative cell present in this tumor most common in sacrococcygeal and sphenooccipital region occurs along central axis of sacrum and spine 20% have calcifications clinical data. Chordomas are rare bony tumors, usually involving the skull base and sacrococcygeal spine, while least presented in cervical and thoracic spine.
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