Chordoma: current concepts, management, and future directions

Summary Chordoma is a rare bone cancer that is aggressive, locally invasive, and has a poor prognosis. Chordomas are thought to arise from transformed remnants of notochord and have a predilection for the axial skeleton, with the most common sites being the sacrum, skull base, and spine. The gold st...

Full description

Saved in:
Bibliographic Details
Published in:The lancet oncology 2012-02, Vol.13 (2), p.e69-e76
Main Authors: Walcott, Brian P, Dr, Nahed, Brian V, MD, Mohyeldin, Ahmed, MD, Coumans, Jean-Valery, MD, Kahle, Kristopher T, MD, Ferreira, Manuel J, MD
Format: Article
Language:English
Subjects:
Airway management
Bone Neoplasms - diagnosis
Bone Neoplasms - pathology
Bone Neoplasms - therapy
Cancer therapies
Chordoma - diagnosis
Chordoma - pathology
Chordoma - therapy
Clinical Trials, Phase I as Topic
Disease
Epidemiology
Gene Expression Regulation, Neoplastic
Hematology, Oncology and Palliative Medicine
Histopathology
Humans
Hypotheses
Medical prognosis
Metastasis
Pathogenesis
Prognosis
Radiation
Rare Diseases - diagnosis
Rare Diseases - pathology
Rare Diseases - therapy
Receptors, Platelet-Derived Growth Factor - metabolism
Spine - pathology
Surgeons
Survival analysis
Transcription factors
Treatment Outcome
Tumors
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Summary Chordoma is a rare bone cancer that is aggressive, locally invasive, and has a poor prognosis. Chordomas are thought to arise from transformed remnants of notochord and have a predilection for the axial skeleton, with the most common sites being the sacrum, skull base, and spine. The gold standard treatment for chordomas of the mobile spine and sacrum is en-bloc excision with wide margins and postoperative external-beam radiation therapy. Treatment of clival chordomas is unique from other locations with an enhanced emphasis on preservation of neurological function, typified by a general paradigm of maximally safe cytoreductive surgery and advanced radiation delivery techniques. In this Review, we highlight current standards in diagnosis, clinical management, and molecular characterisation of chordomas, and discuss current research.
ISSN:1470-2045
1474-5488
DOI:10.1016/S1470-2045(11)70337-0