Chordoma: Rare Cancer in Spine and Skull

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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.

Chordomas are rare tumours that arise from embryonic notochordal remnants along the length of the neuraxis at developmentally active sites. These sites are the ends of the neuraxis and the vertebral bodies. Chordomas comprise less than 1% of CNS tumours, and also rarely occur in extra axial locations. Chordomas are thought to arise from ectopic notochord remnants.

Treatment

In most cases, complete surgical resection followed by radiation therapy offers the best chance of long-term control. Incomplete resection of the primary tumor makes controlling the disease more difficult and increases the odds of recurrence. The decision whether complete or incomplete surgery should be performed primarily depends on the anatomical location of the tumor and its proximity to vital parts of the central nervous system.

Chordomas are relatively radioresistant, requiring high doses of radiation to be controlled. The proximity of chordomas to vital neurological structures such as the brain stem and nerves limits the dose of radiation that can safely be delivered. Therefore, highly focused radiation such as proton therapy and carbon ion therapy are more effective than conventional x-ray radiation.

There are no drugs currently approved to treat chordoma, however a clinical trial conducted in Italy using the PDGFR inhibitor Imatinib demonstrated a modest response in some chordoma patients. The same group in Italy found that the combination of imatinib and sirolimus caused a response in several patients whose tumors progressed on imatinib alone.

The Journal of Orthopedic Oncology offers information in all aspects of primary, malignant tumors. Osteosarcoma, Ewing's sarcoma, chondrosarcoma, chordoma, and soft tissue sarcomas etc it also deals with diagnostic methods, therapeutic approaches, clinical, laboratory research and reconstructive techniques.

The Journal includes a wide range of fields in its discipline to create a platform for the authors to make their contribution towards the journal and the editorial office promises a peer review process for the submitted manuscripts for the quality of publishing.

 

The Journal is using Editorial Manager System for quality in review process. Editorial Manager is an online manuscript submission, review and tracking systems. Review processing is performed by the editorial board members of Orthopedics & oncology or outside experts; at least two independent reviewer’s approval followed by editor approval is required for acceptance of any citable manuscript. Authors may submit manuscripts and track their progress through the system.

The Journal Considers Research article, Review articles, editorial, letter, case reports, short communications, original articles from leading scientists and scholars around the world in all areas of related to Malignantosteoid, Multilobular tumour of bone, Chondrosarcoma, Chordoma, Osteosarcoma, Ewing's sarcomaetc., which come under the scope of the journal.

You may submit your manuscript as an e-mail attachment to the Editorial Office at 
orthooncol@emedicalsci.orgorthooncol@orthopaedicsjournals.com

Best Regards,

Stella

Coordinator| Journal of Orthopedic Oncology

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