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Chordoma and Chondrosarcoma
Built from Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
What’s inside
10 sections · 95 slides
Overview
- Scope of this topic
- Basis for grouping the two tumours
- Shared surgical problem
- Standard therapy for both tumours
Origin and epidemiology
- Chordoma: cell of origin
- Historical recognition of chordoma
- Distribution along the axial skeleton
- Behaviour of chordoma
- Spectrum of notochordal lesions
- Ecchordosis physaliphora
- Chondrosarcoma: cell of origin
- Site of origin of skull base chondrosarcoma
- Anatomic distribution of chondrosarcoma
- Syndromic associations of chondrosarcoma
- Comparative biology of the two tumours
Presenting characteristics
- Chordoma: age and mode of presentation
- Upper clival chordoma and Dorello canal
- Symptoms from tumour bulk and lower clivus
- Risk factors and genetic predisposition
- Chondrosarcoma: presentation differences
- T2 hyperintense clival lesion: prior probability
- Purpose of the petroclival chondrosarcoma stage
- Petroclival chondrosarcoma staging system
Imaging characteristics
- Chordoma on MRI
- Enhancement pattern in an institutional series
- Diffusion and calcification in chordoma
- Anatomic involvement at presentation
- Whole clival chordoma on MRI
- Minimally enhancing chordoma
- Chondrosarcoma on MRI and CT
- Petrous apex and cavernous sinus chondrosarcoma
- Chondroid matrix on CT
- Mesenchymal chondrosarcoma with haemorrhage
Histologic characteristics
- Gross appearance of chordoma
- Histological types and immunohistochemistry
- Physaliphorous cells
- Chondrosarcoma histology and grading
- Grade distribution in a series of 200 patients
Surgical management
- Multidisciplinary skull base team
- En bloc versus piecemeal resection
- Evolution of surgical approach
- Extent of bony resection
- Carotid artery involvement and injury
- Lower clival tumours and craniocervical stability
- Dura, brainstem and cranial nerve policy
- Abducens nerve in Dorello canal at surgery
- Selection of surgical corridor
- Advanced endoscopic approaches
- Endoscopic resection with pituitary transposition
- Subpetrous approach for petrous apex chondrosarcoma
- Staged endoscopic and far-lateral resection of a lower clival chordoma
- Skull base closure and CSF leak prevention
- Reconstruction options when the septal flap is unavailable
- Multilayered closure after large chordoma resection
- Inferior turbinate and lateral wall flap reconstruction
- Role of transoral and lateral approaches
- Conservative strategy in chondrosarcoma
Radiation and systemic therapy
- Indications for adjuvant radiation
- Conventional and fractionated stereotactic radiotherapy
- Rationale for particle therapy
- Proton beam outcomes
- Carbon ion therapy
- IMRT and stereotactic radiosurgery
- Chemotherapy
Patient outcomes
- Interpreting the outcome literature
- Natural history and survival in chordoma
- Extent of resection and survival
- Outcomes in chondrosarcoma
- Recurrence and salvage therapy
Molecular profiling
- Early cytogenetics in chordoma
- Brachyury and the TBXT gene
- Sequencing findings in chordoma
- Dedifferentiated chordoma and the tumour microenvironment
- Molecular findings in chondrosarcoma
- IDH mutation and targeted therapy
- Molecular therapy in chordoma
Summary
- Key differences to remember
- Management principles
- Conclusions
- References
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
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