Chordomas Involving Multiple Neuraxial Bones

Department of Neurosurgery, Ajou University School of Medicine, Suwon, Korea.
Journal of Korean Neurosurgical Society (Impact Factor: 0.64). 02/2009; 45(1):35-8. DOI: 10.3340/jkns.2009.45.1.35
Source: PubMed


We present a patient with multifocal symptomatic osseous chordomas having unusual growth patterns with review of the pertinent literature. The patient was 62-year-old male and had multiple osseous chordomas located in sacral, thoracic, and paraclival jugular foramen areas. There was no metastasis in other organs. All affected sites were osseous. The multicentric chordomas are extremely rare. This case could be considered as a chordoma involving multiple neuraxial bones. But, the possibility of multicentricity could also be thought. In such cases radical resection should be performed for each lesion at the initial diagnosis. If complete surgical resections are infeasible or impossible, preoperative or postoperative radiation therapy should be planned for the highest possibility of successful treatment.

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    ABSTRACT: Aim: Chordoma is a rare, slow-growing primary malignant tumor of the axial skeleton, arising from the embryonic cells of primitive notochord. Chordomas may arise at different sites of the vertebral column simultaneously or more probably they may metastasise along the neural axis insidiously. Recurrence despite radical surgery and following adjuvant therapy is possible. Material and methods: A 46-year-old female patient presented weakness and numbness of the lower extremities. She was operated for clivus chordoma five years ago at another institute. Results: First the patient underwent surgery for resection of the tumor at the cervical region. a second surgery was performed to resect tumor on the foramen magnum and at the C1 level. Histologic examination of the removed vertebra confirmed the diagnosis of chordoma involving the vertebral body. Radiotherapy was administered after the second surgery. Follow-up neurological and radiological examinations revealed no abnormal neurological symptoms 2,5 years after second surgery. There were no distant organ metastases. Conclusion: A patient with diagnosed chordoma of the spine must be investigated with MRI of other regions of the neuraxis to exclude second or even third source of chordoma metastases. In metastatic chordoma cases, radical or gross total resection should be performed for each lesion but if complete surgical resections are impossible, preoperative or postoperative radiation therapy should be planned to improve life expectancy.
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    ABSTRACT: Chordomas are malignant tumors arising from notochordal remnants. They are the most frequent tumors of the spine after plasmacytomas. Only 6% of chordomas are localized to the cervical level. In young patients, chordomas are rare and unpredictable. Despite this, the treatment of choice remains the total resection, as much as possible, followed by proton beam radiation. This case was managed using a precarotid and retrocarotid approach at the same time. The tumor was completely resected with the edges free from disease. The cervical spine was stabilized with an anterior plating C2-C4. Eighteen months after surgery the patient is still free from illness. Multilevel oblique corpectomies are an available and safe option for the treatment of upper cervical chordomas.
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    ABSTRACT: Chordoma arises from cellular remnants of the notochord. It is the most common primary malignancy of the spine in adults. Approximately 50% of chordomas arise from the sacrococcygeal area with other areas of the spine giving rise to another 15% of chordomas. Following complete resection, patients can expect a 5-year survival rate of 85%. Chordoma has a recurrence rate of 40%, which leads to a less favorable prognosis. Here, we report two cases of chordoma presenting with occipital neuralgia and cervicalgia. The first patient presented with a C1-C2 chordoma. He rejected surgical intervention and ultimately died of respiratory failure. The second patient had an atlantoaxial chordoma and underwent surgery because of continued occipital neuralgia and cervicalgia despite nerve block. This patient has remained symptom-free since his operation. The presented cases show that the patients' willingness to participate in treatment can lead to appropriate and aggressive management of cancer pain, resulting in better outcomes in cancer treatment.
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