Since chordomas arise in bone, they are usually extradural and result in local bone destruction. They are locally aggressive but uncommonly metastasize. Chordomas can occur at any age but are usually seen in adults years. Those located in the spheno-occipital region most commonly occur in patients years of age, whereas sacrococcygeal chordomas are typically seen in a slightly older age group peak around 50 years They are commonly found in Caucasians 3.
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Since chordomas arise in bone, they are usually extradural and result in local bone destruction. They are locally aggressive but uncommonly metastasize. Chordomas can occur at any age but are usually seen in adults years. Those located in the spheno-occipital region most commonly occur in patients years of age, whereas sacrococcygeal chordomas are typically seen in a slightly older age group peak around 50 years They are commonly found in Caucasians 3. Macroscopically, chordomas present as firm masses.
Fluid and gelatinous mucoid substance associated with recent and old hemorrhage and necrotic areas are found within these tumors. In some patients, calcification and sequestered bone fragments are found as well.
The variety of these components may explain the signal heterogeneity observed on MRI. Microscopically, chordomas are characterized by physaliphorous cells. These tumors are often poorly marginated and microscopic distal extension of tumor cells likely explains the frequency of recurrences. True malignant forms of chordomas occasionally have areas of typical chordoma as well as undifferentiated areas, most often fibrosarcoma.
The prognosis in such cases is poor. Chordomas are found along the axial skeleton and a relatively evenly distributed among three locations:. In this location, a male predilection has been reported M:F ratio of , and the tumor may be particularly large at presentation Typically the mass projects posteriorly at midline, indenting the pons; this characteristic appearance has been termed the so-called thumb sign.
In contrast to sacrococcygeal tumors, there is no recognized gender difference. Chordomas of the vertebral bodies are rare, but nonetheless, after lymphoproliferative tumors, are the second most common primary malignancy of the spine in adults 2. They most commonly involve cervical particularly C2 , followed by lumbar, and then the thoracic spine. They often extend across the intervertebral disc space, involving more than one vertebral segment. They may extend into the epidural space compressing the spinal cord, or along the nerve roots enlarging the neural foramen.
MRI and CT scan have complementary roles in tumor evaluation. CT evaluation is needed to assess the degree of bone involvement and to detect patterns of calcification within the lesion. MRI provides excellent anatomical delineation of adjacent structures and is able to characterize the signal of the lesion usually allowing for a confident preoperative diagnosis. MRI is, however, limited in its ability to evaluate calcification and the precise involvement of skull base osteolysis less well than CT, especially for skull base foramina.
Although angiography is useful to assess vascular encasement and displacement, chordomas usually do not have significant tumoral vascularity 3. Traditionally, surgical resection has been the first line of treatment when feasible, with radiotherapy offered for recurrent cases. Some advocate the combination of radiation therapy and complete or subtotal surgical resection for select patients 6.
Percutaneous radiofrequency ablation has been trialled as an adjunct therapy 8. Recurrence, including seeding along the operative tract, is common. Histological subtype also has a substantial impact on prognosis with chondroid chordoma having the best prognosis and dedifferentiated chordoma the worst prognosis; the more common conventional chordoma having intermediate prognosis 14, The differential diagnosis for bone tumors is dependent on the age of the patient, with a very different set of differentials for the pediatric patient.
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Quiz questions. MRI of intracranial chordomas. Extent of tumour and contrast enhancement: criteria for differential diagnosis. Neuroradiology link - Pubmed citation. Related Radiopaedia articles Bone tumours The differential diagnosis for bone tumors is dependent on the age of the patient, with a very different set of differentials for the pediatric patient. Edit article Share article View revision history Report problem with Article. URL of Article.
Article information. Tags: neuroradiology , neuroradiology , spine. Support Radiopaedia and see fewer ads. Cases and figures. Figure 1: histology Figure 1: histology. Case 1: clival Case 1: clival.
Case 2: sacrococcygeal Case 2: sacrococcygeal. Case 3: clival Case 3: clival. Case 4: C2 vertebral body Case 4: C2 vertebral body. Case 5: clival Case 5: clival. Case 6: clival Case 6: clival. Case 7: sacrococcygeal Case 7: sacrococcygeal. Case 8: sacrococcygeal Case 8: sacrococcygeal. Case 9: clival Case 9: clival. Case sacrococcygeal Case sacrococcygeal.
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Intradural Retroclival Chordoma
Segmented trans-maxillary approach for clivus tumor resection. Case report. The complex anatomy exhibited by the vital structures of the skull base hinders surgical resection of tumors present in that area. The main problem when facing tumors lodged in the skull base resides in choosing the most suitable approach. The initial development of skull base surgery was a product of the collaboration between otorhinolaryngology and neurosurgery techniques. The participation of maxillofacial surgeons in these events has been a relatively recent endeavor.
Intradural retroclival chordoma without bone involvement: no tumor regrowth 5 years after operation
Chordoma is a rare slow-growing neoplasm thought to arise from cellular remnants of the notochord. 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 notochord regresses during fetal life. In layman's terms, chordoma is a type of spinal cancer. Chordomas can arise from bone in the skull base and anywhere along the spine. The two most common locations are cranially at the clivus and in the sacrum at the bottom of the spine. A small number of families have been reported in which multiple relatives have been affected by chordoma.