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
| Aneurysmal Bone Cyst | Giant Cell Tumor |
| Bone Metastases | Lymphoma, Bone |
| Chondrosarcoma | Meningioma, Brain |
| Craniopharyngioma | Pituitary Adenoma |
| Fibrous Dysplasia |
Other Problems to Be Considered
Chondroma
Ecchordosis physaliphora
Nasopharyngeal malignancies
Plasmacytoma
More on Chordoma |
 Overview: Chordoma |
| Imaging: Chordoma |
| Follow-up: Chordoma |
| Multimedia: Chordoma |
| References |
| Next Page » |
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Further Reading
Keywords
chordoma, notochord tumor, primitive notochord tumor, notochord mass, intracranial tumor, intracranial mass
