What are chordoma spinal tumors?

Updated: Nov 01, 2018
  • Author: Andrew A Sama, MD; Chief Editor: Jeffrey A Goldstein, MD  more...
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Answer

Luschka first described chordoma morphologically in 1856 in Virchow's laboratory. The discovery of the notochordal nature of the tumor and the coining of the term chordoma is credited to Ribbert in 1894.

Chordomas are uncommon, accounting for 2-4% of all primary malignant bone tumors with a prevalence of 0.51 per million. However, they are excluding lymphoproliferative tumors and metastases, the most common primary malignant tumor of the spine in the adult.

As Ribbert described, chordomas arise for the notochord remnant. The notochord normally evolves into the nucleus pulposus of the intervertebral discs. Nonneoplastic notochord vestiges also are found at the midline of the sphenooccipital synchondrosis and in the sacrococcygeal regions. The locations in which chordomas occur parallel these vestigial distributions.

Regarding chordoma prevalence, 30-35% occur in the sphenooccipital region, 50% in the sacrococcygeal region (especially S4-S5), and 15% occur in the other spinal segments.

Interestingly, chordomas have not been reported to arise from the intervertebral disks. Chordomas occur most commonly in patients aged 30-70 years, with a peak incidence in the fifth to sixth decades of life. Sphenooccipital lesions have equal sex distributions but sacrococcygeal lesions have a 3:1 male-to-female ratio.

Presentation of chordomas is often subtle, with a gradual onset of pain, numbness, motor weakness, and constipation or incontinence. Constipation is a uniform finding in most patients with sacrococcygeal lesions. Chordomas are typically slow-growing lesions and are often very large at the time of presentation.

On plain radiography, chordomas appear as a destructive lesion of a vertebral body in the midline, with a large associated soft-tissue mass. In sacrococcygeal lesions, osseous expansion is frequent and may extend across the sacroiliac joints. Mineralization within the tumor may be observed on the plain radiographs of 50-70% of sacrococcygeal lesions. The mineralization is amorphous and predominates in the periphery of the lesion.

Lesions in spinal segments above the sacrum are less expansile and demonstrate evidence of calcification in only 30% of cases. They may have areas of sclerosis in 43-62% of cases. The intervertebral disks above or below a chordoma may be involved and narrowed in a manner that simulates infection. The lesion can make its way through the intervertebral disk to infiltrate an adjacent level. This occurs in approximately 11-14% of cases.

CT demonstrates both osseous and soft-tissue components of the tumor. Coronal and sagittal reconstructions of the CT scan are helpful in assessing neural foraminal and sacroiliac joint involvement. MRI is an important adjunct in the workup of chordomas. The lesions appear with low-to-intermediate signal intensity on T1 images and with very high signal intensity on T2 images, reflecting the high water content of chordomas. Enhancement occurs following intravenous (IV) contrast on both CT and MRI.

Chordomas are lobulated neoplasms, which usually are contained within a pseudocapsule. Histology of these lesions reveals long cords of physaliphorous cells. Physaliphorous cells are clear cells containing intracytoplasmic vacuoles with abundant intracellular and extracellular mucin. Sarcomatous chondroid, osteoid, or fibroid elements may be found within the chordoma.

Surgical resection is the rule. Adjuvant postoperative radiation therapy, proton beam therapy, and brachytherapy all have been used with varying results. The prognosis depends on whether the tumor can be resected completely. The location of the lesion and the size at presentation often necessitate incomplete resection. The treatment of sacral chordoma is an arduous clinical undertaking that requires a multidisciplinary approach and attention to detail from the outset.

Despite aggressive, well-planned surgical management and adherence to strict surveillance protocols, frequent recurrence and the late onset of metastatic disease are to be expected in a substantial proportion of patients, especially those with a large chordoma or one at a more cephalad level. Adequate surgical treatment results in substantial functional impairment and numerous complications; however, it does offer the possibility of long-term disease-free survival. [28]

Persons with sacrococcygeal tumors often have improved survival because the surrounding structures are relatively more expendable and allow a more complete resection. Persons with sacrococcygeal lesions typically have 8-10 years survival, as opposed to 4-5 years survival for persons with chordomas in other spinal sites. Death usually is related to local recurrence and invasion rather than metastatic disease. Chordomas can metastasize. The most common sites of metastases are the liver, lungs, regional lymph nodes, peritoneum, skin, and heart.


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