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Contents:
  1. Chordoma: The Nonsarcoma Primary Bone Tumor
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  3. Chordoma: The Nonsarcoma Primary Bone Tumor
  4. Chordoma and Chondrosarcoma
  5. Original Research ARTICLE

All rights reserved. Please note that the copyright for the original figures submitted by the contributors is owned by Contributors. Cranial base chondroid tumors are rare in the pediatric population.


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In adults, chordomas occur predominantly in males, with a male to female ratio of about , but in the pediatric population they may have equal frequency between male and female patients ; however, our series would suggest that the male predilection seen in adults is preserved. Another unique feature of pediatric chordomas is their predilection for the skull base. In adults, most chordomas arise in the sacrococcygeal area and about a third arise at the skull base.

The clivus is the most common location; almost all tumors in patients younger than 5 years occur there. Older children can have tumors in the maxilla, orbit, nasopharynx, and axial spine.


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Rare locations such as the gluteal muscle and mediastinum have also been reported. The mean age of diagnosis in the pediatric population is about 10 years. Chondrosarcomas can be primary or secondary, arising from benign cartilaginous lesions. As in adults, there is a male predominance. Irradiation of chondrosarcomas, particularly in children, carries significant risk for the development of bone sarcomas commonly osteosarcoma and fibrosarcoma. Although, as in adults, pediatric chondrosarcomas present more commonly in the pelvis, extremities, and the thoracic cage, there is a four times higher frequency of craniofacial tumors in children.

In a review of 79 cases of cranial chordomas in children by Al-Mefty, patients younger than 5 years often presented with symptoms of elevated intracranial pressure and long tract signs. This may reflect the higher incidence of clival lesions in this age group. With this exception, children with chordomas and chondrosarcomas present with signs and symptoms similar to those of adults.

Older patients 5—20 years with cranial lesions often present with headache and double vision abducens nerve palsy.

Chordoma: The Nonsarcoma Primary Bone Tumor

Depending on the location of the tumor, they can also present with hypoglossal nerve palsy, nasal obstruction, dysphagia, and ataxia. Patients may also present with a retropharyngeal mass with associated breathing and swallowing problems Fig. Physical examination should include a complete assessment of cranial nerve function. In patients with nasal congestion or airway obstruction, evaluation by otolaryngology should be performed and include nasal endoscopy. Symptoms of hearing loss or vestibular dysfunction can be evaluated further with audiometric testing and vestibular tests if necessary.

Lower cranial nerve dysfunction may require evaluation of swallowing function and aspiration risk with a functional endoscopic examination of swallowing or radiography barium esophagram. Patients with sacrococcygeal chordomas most often present with pain, constipation, and less often, neurologic symptoms related to the involvement of the cauda equine.

2nd Edition

Pain and a palpable mass are also presenting features of pelvic chondrosarcomas. Magnetic resonance imaging is the preferred test for both initial diagnosis and follow-up. The radiographic characteristics in children are similar to adults.

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Children can have tumors in unusual locations. For lesions involving the skull base and extending into the upper cervical spine, it is useful to image the entire tumor, which may require extending standard brain protocols into the cervical spine or extending the standard spine protocol into the skull base. Since most patients will receive multimodality treatment that may span multiple institutions, it is helpful to use similar imaging techniques to improve comparison across studies.

A computed tomographic scan provides excellent definition of the bony anatomy at the skull base tumor, which is useful in planning the surgical approach Fig. Young children younger than 5 years may harbor poorly differentiated tumors. These may have different imaging characteristics because of their hypercellularity, often more hypodense on T2 with significant postgadolinium enhancement. The Chordoma Foundation recommends total spine imaging at some point around diagnosis, as some patients will harbor benign notochordal rests or tumors, chondromas, or even other coincident chordomas.

Finally, epidural venous metastasis may occur in a delayed fashion, typically in the cervical spine, as a mode of regional spread from the skull base.

Chordoma: The Nonsarcoma Primary Bone Tumor

The histology of pediatric tumors is similar to that of adult lesions, with a few exceptions. Three histological subtypes are described: classical, chondroid, and dedifferentiated. Almost two-thirds of cranial chordomas in children younger than 5 years have the dedifferentiated histological pattern.

This variant has a more sarcomatous appearance with cells arranged in sheets, clusters, or nodules. Necrosis is common.

Chordoma and Chondrosarcoma

Histological grading of pediatric chondrosarcomas is similar to that for tumors in adults. Surgical Considerations. Midline Subfrontal Approaches. Transsphenoidal Surgery for Chordomas and Chondrosarcomas. Transethmoid Transsphenoid Approaches. Maxillotomy for Clival Cartilaginous Tumors. Transoral Approaches. Facial Translocation.

Original Research ARTICLE

Anterolateral Approaches to Parasellar Chordomas and Chondrosarcomas. Lateral Approaches. Transtemporal Approaches to Clival Cartilaginous Tumors. Surgery of Tumors of the Mobile Spine. Sacral Chordoma Treatment by Posterior Resection. Radiation Therapy of Chordomas and Chondrosarcomas. Radiobiology and the Proton Beam.

CLIVAL CHORDOMA

Photon Irradiation. Proton Radiotherapy for Cranial Base Chordomas. Radiosurgery of Chordomas and Chondrosarcomas. Special Considerations. Chordomas and Chondrosarcomas in Children. Chemotherapy of Recurrent or Metastatic Disease. Harsh , Ivo P. Janecka , Henry J. Spinal instability as defined by the threecolumn spine concept in acute spinal trauma. Clin Orthop. Chondrosarcoma of the base of the skull: A clinicopathologic study of cases with emphasis on its distinction from chordoma.