eMedicine Specialties > Radiology > Brain/Spine

Chordoma

Paule Peretti, MD, Neuroradiologist, Radiological Department, Sainte Marguerite Hospital, France
Hervé Brunel, MD, Consulting Staff, Department of Neuroradiology, Montpellier of Pr Bonafé, France; Frédéric P Borrione, MD, Assistant Professor of Orthopedic Surgery, University of Marseilles; President, Center for Evaluation of Osteoarthritis Diseases, Marseilles, France; Guillaume Gorincour, MD, Staff Physician, Department of Neuroradiology, University Hospital of Marseilles, France

Updated: Dec 23, 2008

Introduction

Morphology of chordoma. Chordoma of the upper part of the clivus with posterior extension to the pontine cistern. Contrast-enhanced sagittal T1-weighted spin-echo image. The bone appears expanded in this early form.

Differential diagnosis between chordoma and invasive pituitary adenoma. Sagittal contrast-enhanced T1-weighted magnetic resonance image. 6a. Clivus chordoma with posterior extension into pontine cistern and compression of brainstem. The tumor appears lobulated and enhances heterogeneously, while the pituitary gland shows more marked enhancement, suggesting that the tumor does not arise from it. 6b. Invasive pituitary adenoma. The signal of the mass in the sphenoid is not homogeneous. No posterior extension is observed; extension is mostly into the sphenoid sinus. The pituitary gland is not visible.

Background

Chordomas are tumors originating from embryonic remnants of the primitive notochord. Because chordomas lie in bone, they are usually extradural and induce bone destruction.^1,2

Related eMedicine topics:
Chordoma (from Neurosurgery)
Chordoma (from Orthopedic Surgery)
Histology of Bone
Non-neoplastic Conditions Simulating Bone Tumors
Disorders of Bone Mineralization

Pathophysiology

Histologically, chordomas appear as embryonic notochord under both light and electron microscopy. Macroscopically, chordomas form a soft, white, multilobulated mass delineated by a fibrous pseudocapsule that, because of adjacent tissue compression, has the appearance of a true capsule.

Fluid and gelatinous mucoid substance (associated with recent and old hemorrhage) and necrotic areas are found within the tumor; in some patients, calcification and sequestered bone fragments are seen as well. The variety of these components may explain the signal heterogeneity observed on magnetic resonance imaging (MRI) scans. Incomplete delineation of the tumor and microscopic, distal extension of tumor cells may explain the frequency of recurrences. Microscopically, chordomas are composed of characteristic mucoid, fluid-containing, translucent cells of variable size with a large, intracytoplasmic vacuole, and they are rich in mucin and glycogen (physaliphorous cells). The physaliphorous cells are organized into lobules separated by thick, fibrous sheets.

In addition to conventional chordomas, chondroid chordomas, which are composed of cartilaginous hyaline tissue, are identified. These chondroid forms have shorter T1- and T2-weighted MRI signals because of low water content. Patients with chondroid chordomas appear to have a different prognosis, but the clinical and histologic characteristics of these tumors remain controversial.

Differentiating chordomas from chondrosarcomas using both radiologic and histologic criteria can be difficult. Immunohistochemical studies using cytokeratin antibodies and epithelial membrane antigen (negative in chondrosarcomas, positive in chordomas) can make the distinction. Chondroid forms can represent low-grade chondrosarcomas, which also is controversial. Metastatic epithelial neoplasms should be considered in the differential diagnosis as well.

Metastatic spread of chordomas is observed in 7-14% of patients with lymph node, pulmonary, bone, cerebral, or abdominal visceral involvement, predominantly from massive tumors. In true malignant forms of chordomas there occasionally are areas of typical chordoma, as well as undifferentiated areas, most often suggestive of fibrosarcoma; the prognosis is poor.

Frequency

International

Chordomas are rare tumors with an estimated incidence of 0.51 cases per million. Clival chordomas represent less than 0.2% of all intracranial tumors.

Mortality/Morbidity

Clival chordomas are benign tumors; but because of their critical location, local invasion, recurrence, and occasional metastatic spread, their prognosis is similar to that of malignant tumors.

For sacrococcygeal chordomas, the average survival rate from the onset of symptoms is usually 5.7 years. Metastasis is rare, but when it occurs, it is most often to the lymph nodes, lungs, and liver. In 5-40% of patients with spinal and sacrococcygeal chordoma, metastatic lesions have been reported.³ Recurrence after curative resection is frequent and may result in a slow but relentless and fatal progression of the disease as a result of invasion of local pelvic structures. This is one of the most difficult tumors for the surgeon to treat. Infection is a frequent complication.

Race

No racial predilection exists for this tumor.

Sex

The male-to-female ratio is 2:1.

Age

Chordomas are found primarily in adults and occur rarely in patients younger than 30 years.

Anatomy

Chordomas can be found in any part of the axial skeleton, with a predominance in the sacrococcygeal (50%) or clival (35%) areas; however, the cervical, thoracic, and lumbar vertebrae also can be involved (15%).

Presentation

• Clival chordomas
  • The progressive growth of chordomas results in compression and invasion of important neighboring structures.
  • Skull base chordomas cause a variety of clinical signs, including headache and cranial nerve deficits.
  • The cranial nerve involved most often is the CN VI abducens.
  • Other signs include dysphagia, facial pain, facial paresis, visual loss, hearing loss, and ataxia.
• Spinal chordomas
  • Symptoms of spinal chordomas vary with location and extent of the tumor.
  • Most frequently, tumors are localized at the sacrococcygeal level.
  • Because of an insidious onset, a considerable amount of time elapses between the onset of symptoms and diagnosis.
  • The most frequent symptom is low back pain or pain localized to the sacrum or coccyx, with no characteristic features to the pain.
  • Constipation is the next most frequently reported symptom.
  • Nerve root compression, with radicular leg pain and urinary complaints, suggests tumor invasion into the foramina.

Preferred Examination

MRI and computed tomography (CT) scanning have complementary roles in the evaluation of chordoma. CT scanning is needed to assess the degree of bone involvement or destruction and to detect patterns of calcification within the lesion. MRI provides excellent 3-dimensional analysis of the posterior fossa (especially the brainstem), sella turcica, cavernous sinuses, and middle cranial fossa.

Limitations of Techniques

MRI does not depict calcifications and the precise involvement of skull base osteolysis as well as CT scanning, especially for skull base foramina.

Similarly, in the spine, MRI and CT are complementary. In addition, it is much easier and more time efficient to survey large areas of the spinal axis and roots (or indeed, the entire spinal axis) with MRI than with CT scanning.

Differential Diagnoses

Other Problems to Be Considered

Chondroma
Ecchordosis physaliphora
Nasopharyngeal malignancies
Plasmacytoma

Radiography

Sacrococcygeal chordoma. Plain radiograph of the pelvis showing expansion of the sacrum, bone rarefaction, and large mass of soft tissue with some trabeculations.

Findings

Radiographically, chordomas have 4 pathognomonic characteristics on plain film evaluation, as follows:

• Expansion of the bone
• Rarefaction
• Trabeculation
• Calcification

Degree of Confidence

Radiographs are neither specific nor sensitive for detecting chordoma. For intracranial chordomas, plain films are no longer used.

For sacrococcygeal and spinal chordomas, plain films are often the first examination, but CT scanning and MRI are necessary for the diagnosis.

False Positives/Negatives

Because portions of a chordoma may have little or no calcification present, even if a destructive clival lesion is observed on plain films, the size of the tumor may be grossly underestimated because the soft-tissue component is not visualized.

Computed Tomography

Computed tomography (CT) scan of 2 patients with chordoma. Coronal plane (left): midline tumor with lateral extension, skull base destruction, and carotid canal lysis on the right. Note calcification or osseous debris. Axial plane (right): lysis of clivus (arrow).

Computed tomography (CT) scan. Recurrence of a sacrococcygeal chordoma. Note tumoral infiltration of gluteal muscles displacing the rectum anteriorly. Image courtesy of Editions Masson, Paris, 2002.

Computed tomography (CT) scan of sacrococcygeal chordoma. Note the sacral lysis with trabeculations.

Computed tomography (CT) scan of sacrococcygeal chordoma. Note the tumoral calcification in this huge pelvic tumor.

Computed tomography (CT) scan of sacrococcygeal chordoma. Note the right sacroiliac joint lysis.

Findings

On CT scans, the tumor appears homogeneous, with a density comparable to that of muscles. Tumor appearance on contrast enhancement is heterogeneous. Calcification is found in less than one half of patients, and differentiation from sequestered bone fragments is difficult.^4,5,6

• Intracranial chordomas
  • The most characteristic appearance of intracranial chordoma is of a centrally located soft-tissue mass arising from the clivus and causing adjacent bone destruction.
  • Calcification is common.
  • Areas of low attenuation within the soft-tissue mass—representing the myxoid and gelatinous material found on pathologic examination—are occasionally found on CT scans.
  • CT scanning reliably demonstrates petrous apex involvement and lysis of the skull base foramina.
• Sacrococcygeal chordomas
  • The usual radiographic pattern is lytic, with frequent calcification or sequestered bone fragments.
  • Chordomas are often massive, well-delineated tumors that shift the fatty tissue of the pelvis and involve bone structures and the epidural area.
  • Peripheral sclerosis may be observed in approximately 50% of patients.
  • Frequently, a discrepancy is found between a large soft-tissue component and the area of bone involvement.
  • Regional lymph nodes are usually invaded.
  • The association of osteolytic lesions and soft masses involving the discs and the vertebrae suggests other diagnoses, such as neurofibromas, lymphomas, metastases, and plasmacytomas.
  • The most reliable sign of sacral chordomas is the destruction of several sacral vertebrae associated with a tissue mass anterior to the sacrum.
• Spinal chordomas
  • Infrequently, chordomas arise in the mobile (ie, cervical, thoracic, lumbar) spine (15%).
  • The cervical spine is the most common site for tumors, with a predominance in the C2 vertebra. The thoracic and lumbar areas of the spine are involved less frequently.
  • Initially, the presentation of chordoma on CT scan is of bone destruction centered in the vertebral body, with an associated, anteriorly or laterally situated, paraspinal soft-tissue mass that may contain calcification.
  • Chordomas that occur in vertebrae above the sacrum appear to originate in a single vertebral body, initially producing lytic changes and ultimately resulting in vertebral collapse.
  • The vertebral body is involved first; then, the pedicles, laminae, and spinal process become involved as well.
  • Adjacent intervertebral disc spaces are usually spared.
  • Occasionally, contiguous vertebrae are involved, with sparing of the discs.
  • Epidural extension of the tumor is usual.
  • Both CT scanning and MRI can define the extravertebral extension of the tumor. The main bulk of the tumor is usually anterior to the spine.

Degree of Confidence

CT scanning is essential for evaluating bony integrity, bone destruction, and calcifications or bone fragments within the lesion; for these uses, it is highly sensitive and accurate. MRI is the best technique to evaluate extension of the tumor.

Magnetic Resonance Imaging

Morphology of chordoma. Chordoma of the upper part of the clivus with posterior extension to the pontine cistern. Contrast-enhanced sagittal T1-weighted spin-echo image. The bone appears expanded in this early form.

Recurrence of clival chordoma following surgery. Contrast-enhanced sagittal T1-weighted gradient-echo image showing brainstem and foramen magnum invasion.

Chordoma. Coronal T1-weighted spin-echo magnetic resonance image. High signal is a result of hemorrhage.

Chordoma. Contrast-enhanced sagittal gradient-echo T1-weighted magnetic resonance image demonstrates heterogeneous, lobulated tumor.

Coccygeal chordoma. Sagittal T1-weighted magnetic resonance image. Note the subcutaneous tissue infiltration.

Sacrococcygeal chordoma. Sagittal T1-weighted magnetic resonance image showing a huge, well-delineated tumoral mass invading the sacral canal, extending into the pelvis, and shifting the fat, uterus, bladder, and rectum. Image courtesy of Editions Masson, Paris, 2002.

Findings

Among imaging methods that contribute to the diagnosis, MRI is particularly reliable. T1- and T2-weighted sequences are needed before and after gadolinium injection. For clival chordomas, 3-dimensional gradient-echo T1-weighted sequences are helpful because they visualize the tumor in 3 planes within a short time and with a good analysis of tumoral signal.⁷

• Intracranial chordomas
  • Evaluation of the precise extent of the tumor and the degree of involvement of adjacent tissues is best performed by MRI. These attributes are relevant to diagnosis and choice of treatment (biopsy or surgical and/or radiosurgical treatment). MRI specifically shows tumor extension into critical structures, such as the cavernous sinuses, the circle of Willis, and the brainstem.
  • Chordomas originate from the midline, with varying degrees of lateral extension. This characteristic predilection for the midline may aid in the differential diagnosis.
  • Several authors have reported atypical sites of chordoma. They probably originate from ectopic notochord, and their prognosis differs from that of typical chordomas. The extension of chordomas is primarily posterior, with involvement of the pontine cistern and, occasionally, of the premedullary cistern. Anterior extension, which is also frequent, primarily occurs in massive tumors with significant destruction of the skull base. This can be anterosuperior to the sella turcica, displacing the pituitary gland, or anteroinferior to the nasopharynx or middle cranial fossa.
  • Overall, extension is primarily along the anteroposterior axis rather than laterally. However, some limited lateral extension commonly does occur into the cavernous sinuses, affecting treatment. It is observed in as many as 75% of patients.
  • Morphology and signal of the tumor are other elements in diagnosis.
  • The expansion of the bone in the early stage indicates that the tumor arises from bone and not from adjacent structures. This feature disappears as the tumor enlarges further.
  • Skull base chordomas are well delineated at the outset, since they displace adjacent structures; however, more advanced tumors become invasive and have a pseudomalignant appearance with bone erosion and soft-tissue invasion.
  • Most chordomas are isointense or demonstrate low signal on T1-weighted images. Some tumors an also demonstrate high signal, which is related on histologic examination to hemorrhage and mucinous collections. Tumoral signal on T1-weighted sequences is thus not entirely reliable.
  • Most chordomas exhibit high signal on T2-weighted images, which is also nonspecific.
  • Following gadolinium injection, chordomas usually show lobulated areas with a honeycomb appearance corresponding to low signal areas within the tumor. Chordoma signal is described as heterogeneous after gadolinium injection and on T1- and T2-weighted images. The pattern of contrast enhancement can be related to the pathologic features of the tumors, which are organized in lobules with mucinous and gelatinous contents. This may be a useful diagnostic sign. The borders of the tumor are better delineated with gadolinium injection.
• Chondromas may have the same radiologic appearance as chordomas. They originate from embryonic remnants of the primitive cartilage and tend to arise from and extend more laterally into the sellar and cerebellopontine-angle regions.
• Craniopharyngiomas have a different topography in addition to a relatively characteristic signal. The tumors are suprasellar, sellar, or infrasellar and are rarely at the level of the nasopharynx. Generally, the site is more anterior and superior, and extension is almost always posterosuperior (interpeduncular cistern).
• Invasive pituitary adenomas usually affect the sphenoid sinus, although in some patients they are posterior, in which case the hypophysis is not visible. Usually, the pontine cistern is not involved. Of course, clinical and biological data are the primary indications of pituitary origin, except in nonsecreting adenomas.
• Clivus meningiomas are differentiated easily from chordomas. They have a large dural attachment and do not appear similar to bone tumors. Homogeneity of their signal is an additional element.
• Chordomas can involve the petrous apex. Contrary to intrinsic petrous apex tumors (ie, rhabdomyosarcomas, metastases, plasmacytomas, cholesterol granulomas, epidermoid cysts), they originate from the midline.
• Aside from clinical data, few criteria exist for diagnosing lymphoma of the skull base. Similarly, bone metastases can be found in any part of the skull base with extensive osteolysis and a rapid course. Since skull base metastases are relatively infrequent in the absence of a primary neoplasm, differential diagnosis is easier. Neither type of tumor is frequent. Nasopharyngeal malignancies typically extend more anteriorly.
• Sacrococcygeal chordomas
  • MRI provides detailed multiplane information, with excellent contrast of the tumor and its surrounding anatomic structures. Tumoral extension is important to ascertain for preoperative planning and is observed as follows:
    • Proximal extension - Bone and sacral canal
    • Distal-lateral extension - Gluteus maximus, hamstrings, and sciatic nerve and notch
    • Anterior extension - Retroperitoneal lymph nodes and rectum
    • Posterior extension - Subcutaneous fat
  • On MRI, sacrococcygeal chordomas are lobulated tumors, typically with low to intermediate signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images. The pattern of gadolinium enhancement is the same as for clival chordomas.

Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or magnetic resonance angiography scans. 

NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin;yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.

Degree of Confidence

MRI is highly accurate in assessing soft-tissue extent of chordomas and in evaluating involvement of adjacent tissues.

Intervention

Clival chordomas: Chordomas are radioresistant tumors in which surgical management is difficult. However, the proximity of essential neurovascular structures does not exclude surgery, since these tumors may be dissected relatively safely in several cases. The prognosis is better when tumor removal is more complete.

• The best tool for demonstrating tumoral site and extension and for selecting the surgical approach is 3-dimensional MRI.
• Microsurgery is performed through subfrontal, subtemporal, and transsphenoidal approaches. Involvement of a cavernous sinus does not exclude surgery.
• Advances in hyperfocal radiosurgical techniques, such as a gamma knife, linear accelerator, or proton therapy, provide better control of the residual tumor, with high-dose radiation therapy sparing the adjacent tissues. This therapeutic procedure is particularly useful in clival chordomas because of the difficult surgical approach in this area and the close relationship between this tumor and essential neurovascular structures.

Lumbosacral chordomas: The patient's only chance for cure with this entity is an en bloc resection with a margin of healthy tissue. Chordomas below the S1 vertebra should be resected en bloc to provide a chance for cure.

• In other locations, a surgical strategy ensuring preservation of function should be chosen with adjunctive high-dose radiotherapy.
• Postsurgical bladder and rectal functions are major complications and related directly to the number of preserved nerve roots.
• Effective management of sacrococcygeal chordomas consists of early diagnosis, definitive and adequate surgical resection with proven tumor-free cut margins, and close follow-up care, including MRI and CT scanning.

Medical therapy: Research indicates that imatinib mesylate exhibits antitumor activity in patients with chordoma.⁸

Multimedia

Media file 1: Pathology of chordoma. Lobulated tumor with epithelial cords separated by mucinous material (hematoxylin-phloxine-saffron, magnification X25).

Media file 2: Pathology of chordoma. At higher magnification, vacuolated neoplastic cells and occasional physaliphorous cells are observed (hematoxylin-phloxine-saffron, magnification X225).

Media file 3: Immunopathology of chordoma. The epithelial nature of chordoma is assessed by the strong immunoreactivity to anticytokeratin antibody (immunoperoxidase, magnification X130).

Media file 4: Topography of chordoma. Differential diagnosis between chordoma and chondroma. 4a. Clival chordoma with a lateral extension to the left cavernous sinus. Coronal contrast-enhanced T1-weighted spin-echo image. The tumor is median with a lateral extension. 4b. Laterosellar chondroma. Coronal contrast-enhanced T1-weighted gradient-echo image demonstrates the strictly lateral localization of the tumor.

Media file 5: Morphology of chordoma. Chordoma of the upper part of the clivus with posterior extension to the pontine cistern. Contrast-enhanced sagittal T1-weighted spin-echo image. The bone appears expanded in this early form.

Media file 6: Differential diagnosis between chordoma and invasive pituitary adenoma. Sagittal contrast-enhanced T1-weighted magnetic resonance image. 6a. Clivus chordoma with posterior extension into pontine cistern and compression of brainstem. The tumor appears lobulated and enhances heterogeneously, while the pituitary gland shows more marked enhancement, suggesting that the tumor does not arise from it. 6b. Invasive pituitary adenoma. The signal of the mass in the sphenoid is not homogeneous. No posterior extension is observed; extension is mostly into the sphenoid sinus. The pituitary gland is not visible.

Media file 7: Recurrence of clival chordoma following surgery. Contrast-enhanced sagittal T1-weighted gradient-echo image showing brainstem and foramen magnum invasion.

Media file 8: Chordoma. Coronal T1-weighted spin-echo magnetic resonance image. High signal is a result of hemorrhage.

Media file 9: Chordoma. Contrast-enhanced sagittal gradient-echo T1-weighted magnetic resonance image demonstrates heterogeneous, lobulated tumor.

Media file 10: Computed tomography (CT) scan of 2 patients with chordoma. Coronal plane (left): midline tumor with lateral extension, skull base destruction, and carotid canal lysis on the right. Note calcification or osseous debris. Axial plane (right): lysis of clivus (arrow).

Media file 11: Sacrococcygeal chordoma. Plain radiograph of the pelvis showing expansion of the sacrum, bone rarefaction, and large mass of soft tissue with some trabeculations.

Media file 12: Coccygeal chordoma. Sagittal T1-weighted magnetic resonance image. Note the subcutaneous tissue infiltration.

Media file 13: Sagittal contrast-enhanced T1-weighted magnetic resonance image. Note the heterogeneous gadolinium enhancement (same patient as in Image 12).

Media file 14: Sagittal T2-weighted magnetic resonance image. The tumor appears lobulated and shows high signal (same patient as in Images 12 and 13). Image courtesy of Editions Masson, Paris, 2002.

Media file 15: Sacrococcygeal chordoma. Sagittal T1-weighted magnetic resonance image showing a huge, well-delineated tumoral mass invading the sacral canal, extending into the pelvis, and shifting the fat, uterus, bladder, and rectum. Image courtesy of Editions Masson, Paris, 2002.

Media file 16: Sagittal contrast-enhanced T1-weighted magnetic resonance image. Note the lobulated heterogeneous contrast enhancement (same patient as in Image 15). Image courtesy of Editions Masson, Paris, 2002.

Media file 17: Coronal T1-weighted magnetic resonance image (same patient as in Images 15 and 16). Image courtesy of Editions Masson, Paris, 2002.

Media file 18: Coronal contrast-enhanced T1-weighted magnetic resonance image (same patient as in Images 15-17). Image courtesy of Editions Masson, Paris, 2002.

Media file 19: Computed tomography (CT) scan. Note the calcification (same patient as in Images 15-18). Image courtesy of Editions Masson, Paris, 2002.

Media file 20: Computed tomography (CT) scan. Recurrence of a sacrococcygeal chordoma. Note tumoral infiltration of gluteal muscles displacing the rectum anteriorly. Image courtesy of Editions Masson, Paris, 2002.

Media file 21: Coronal T1-weighted magnetic resonance image. Tumoral infiltration of the thigh (same patient as in Image 20). Image courtesy of Editions Masson, Paris, 2002.

Media file 22: Axial T1-weighted magnetic resonance image (same patient as in Images 20 and 21). Image courtesy of Editions Masson, Paris, 2002.

Media file 23: Computed tomography (CT) scan of sacrococcygeal chordoma. Note the sacral lysis with trabeculations.

Media file 24: Computed tomography (CT) scan of sacrococcygeal chordoma. Note the tumoral calcification in this huge pelvic tumor.

Media file 25: Computed tomography (CT) scan of sacrococcygeal chordoma. Note the right sacroiliac joint lysis.

References

1. Sundaresan N, Rosen G, Boriani S. Primary malignant tumors of the spine. Orthop Clin North Am. Jan 2009;40(1):21-36. [Medline].

2. Weber K, Damron TA, Frassica FJ, Sim FH. Malignant bone tumors. Instr Course Lect. 2008;57:673-88. [Medline].

3. McPherson CM, Suki D, McCutcheon IE, et al. Metastatic disease from spinal chordoma: a 10-year experience. J Neurosurg Spine. Oct 2006;5(4):277-80. [Medline].

4. Park SA, Kim HS. F-18 FDG PET/CT evaluation of sacrococcygeal chordoma. Clin Nucl Med. Dec 2008;33(12):906-8. [Medline].

5. Miyazawa N, Ishigame K, Kato S, Satoh Y, Shinohara T. Thoracic chordoma: review and role of FDG-PET. J Neurosurg Sci. Dec 2008;52(4):117-22. [Medline].

6. Shankar J, Jayakumar P, Vasudev M, Ravishankar S, Sinha N. The Usefulness of CT Perfusion in Differentiation between Neoplastic and Tuberculous Disease of the Spine. J Neuroimaging. Oct 31 2008;[Medline].

7. Mehnert F, Beschorner R, Küker W, et al. Retroclival ecchordosis physaliphora: MR imaging and review of the literature. AJNR Am J Neuroradiol. Nov-Dec 2004;25(10):1851-5. [Medline]. [Full Text].

8. Casali PG, Messina A, Stacchiotti S, et al. Imatinib mesylate in chordoma. Cancer. Nov 1 2004;101(9):2086-97. [Medline].

9. Austin-Seymour M, Munzenrider J, Goitein M, et al. Fractionated proton radiation therapy of chordoma and low-grade chondrosarcoma of the base of the skull. J Neurosurg. Jan 1989;70(1):13-7. [Medline].

10. Bergh P, Kindblom LG, Gunterberg B, et al. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. May 1 2000;88(9):2122-34. [Medline].

11. Bjornsson J, Wold LE, Ebersold MJ, et al. Chordoma of the mobile spine. A clinicopathologic analysis of 40 patients. Cancer. Feb 1 1993;71(3):735-40. [Medline].

12. Bohm B, Milsom JW, Fazio VW, et al. Our approach to the management of congenital presacral tumors in adults. Int J Colorectal Dis. Sep 1993;8(3):134-8. [Medline].

13. Boriani S, Chevalley F, Weinstein JN, et al. Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine. Jul 1 1996;21(13):1569-77. [Medline].

14. Cable DG, Moir C. Pediatric sacrococcygeal chordomas: a rare tumor to be differentiated from sacrococcygeal teratoma. J Pediatr Surg. May 1997;32(5):759-61. [Medline].

15. Chandawarkar RY. Sacrococcygeal chordoma: review of 50 consecutive patients. World J Surg. Jul-Aug 1996;20(6):717-9. [Medline].

16. Colombo N, Berry I, Norman D. Vertebral tumors. In: Manelfe C, ed. Imaging of the Spine and Spinal Cord. New York, NY: Raven Press; 1992:445-87.

17. Doucet V, Peretti-Viton P, Figarella-Branger D, et al. MRI of intracranial chordomas. Extent of tumour and contrast enhancement: criteria for differential diagnosis. Neuroradiology. Aug 1997;39(8):571-6. [Medline].

18. Forsyth PA, Cascino TL, Shaw EG, et al. Intracranial chordomas: a clinicopathological and prognostic study of 51 cases. J Neurosurg. May 1993;78(5):741-7. [Medline].

19. Gay E, Sekhar LN, Rubinstein E, et al. Chordomas and chondrosarcomas of the cranial base: results and follow- up of 60 patients. Neurosurgery. May 1995;36(5):887-96; discussion 896-7. [Medline].

20. Klekamp J, Samii M. Spinal chordomas--results of treatment over a 17-year period. Acta Neurochir (Wien). 1996;138(5):514-9. [Medline].

21. Lanzino G, Sekhar LN, Hirsch WL, et al. Chordomas and chondrosarcomas involving the cavernous sinus: review of surgical treatment and outcome in 31 patients. Surg Neurol. Nov 1993;40(5):359-71. [Medline].

22. Leproux F, de Toffol B, Aesch B, et al. MRI of cranial chordomas: the value of gadolinium. Neuroradiology. 1993;35(7):543-5. [Medline].

23. Meyer JE, Lepke RA, Lindfors KK, et al. Chordomas: their CT appearance in the cervical, thoracic and lumbar spine. Radiology. Dec 1984;153(3):693-6. [Medline].

24. Meyer JE, Oot RF, Lindfors KK. CT appearance of clival chordomas. J Comput Assist Tomogr. Jan-Feb 1986;10(1):34-8. [Medline].

25. Meyers SP, Hirsch WL Jr, Curtin HD, et al. Chordomas of the skull base: MR features. AJNR Am J Neuroradiol. Nov-Dec 1992;13(6):1627-36. [Medline].

26. O''Connell JX, Renard LG, Liebsch NJ, et al. Base of skull chordoma. A correlative study of histologic and clinical features of 62 cases. Cancer. Oct 15 1994;74(8):2261-7. [Medline].

27. Ozaki T, Hillmann A, Winkelmann W. Surgical treatment of sacrococcygeal chordoma. J Surg Oncol. Apr 1997;64(4):274-9. [Medline].

28. Rosenberg AE, Brown GA, Bhan AK, et al. Chondroid chordoma--a variant of chordoma. A morphologic and immunohistochemical study. Am J Clin Pathol. Jan 1994;101(1):36-41. [Medline].

29. Saltutti C, Di Cello V, Marzocco M, et al. Sacrococcygeal chordoma: clinicoradiological study. Urol Int. 1990;45(6):372-5. [Medline].

30. Sen CN, Sekhar LN, Schramm VL, et al. Chordoma and chondrosarcoma of the cranial base: an 8-year experience. Neurosurgery. Dec 1989;25(6):931-40; discussion 940-1. [Medline].

31. Stephens GC, Schwartz HS. Lumbosacral chordoma resection: image integration and surgical planning. J Surg Oncol. Dec 1993;54(4):226-32. [Medline].

32. Sundaresan N, Galicich JH, Chu FC, et al. Spinal chordomas. J Neurosurg. Mar 1979;50(3):312-9. [Medline].

33. Sze G, Uichanco LS 3rd, Brant-Zawadzki MN, et al. Chordomas: MR imaging. Radiology. Jan 1988;166(1 Pt 1):187-91. [Medline].

34. Tai PT, Craighead P, Bagdon F. Optimization of radiotherapy for patients with cranial chordoma. A review of dose-response ratios for photon techniques. Cancer. Feb 1 1995;75(3):749-56. [Medline].

35. Taki S, Kakuda K, Kakuma K, et al. Posterior mediastinal chordoma: MR imaging findings. AJR Am J Roentgenol. Jan 1996;166(1):26-7. [Medline].

36. Wetzel LH, Levine E. Pictorial essay. MR imaging of sacral and presacral lesions. AJR Am J Roentgenol. Apr 1990;154(4):771-5. [Medline].

37. Wold LE, Laws ER Jr. Cranial chordomas in children and young adults. J Neurosurg. Dec 1983;59(6):1043-7. [Medline].

38. York JE, Kaczaraj A, Abi-Said D, et al. Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. Jan 1999;44(1):74-9; discussion 79-80. [Medline].

39. Yuh WT, Flickinger FW, Barloon TJ, et al. MR imaging of unusual chordomas. J Comput Assist Tomogr. Jan-Feb 1988;12(1):30-5. [Medline].

Keywords

chordoma, notochord tumor, primitive notochord tumor, notochord mass, intracranial tumor, intracranial mass

Contributor Information and Disclosures

Author

Paule Peretti, MD, Neuroradiologist, Radiological Department, Sainte Marguerite Hospital, France
Paule Peretti, MD is a member of the following medical societies: French Society of Radiology
Disclosure: Nothing to disclose.

Coauthor(s)

Hervé Brunel, MD, Consulting Staff, Department of Neuroradiology, Montpellier of Pr Bonafé, France
Disclosure: Nothing to disclose.

Frédéric P Borrione, MD, Assistant Professor of Orthopedic Surgery, University of Marseilles; President, Center for Evaluation of Osteoarthritis Diseases, Marseilles, France
Disclosure: Nothing to disclose.

Guillaume Gorincour, MD, Staff Physician, Department of Neuroradiology, University Hospital of Marseilles, France
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey L Creasy, MD, Associate Professor, Associate Section Head, Division of Neuroradiology, Director, Neuroradiology Fellowship, Department of Radiology, Vanderbilt University
Jeffrey L Creasy, MD is a member of the following medical societies: American College of Radiology, American Society of Neuroradiology, and Radiological Society of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

C Douglas Phillips, MD, Professor, Departments of Radiology, Neurosurgery, and Otolaryngology, University of Virginia Health Sciences Center
C Douglas Phillips, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Society of Head and Neck Radiology, American Society of Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Amirsys Royalty Consulting

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

© 1994- by Medscape.
All Rights Reserved
(http://www.medscape.com/public/copyright)