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Published on: July 5, 2021
Chordomas of the skull base: manifestations and management
Michele St Martin1, Samuel C Levine
1Department of Otolaryngology, University of Minnesota, Minneapolis, Minnesota 55455, USA.
Purpose Of Review:
Chordomas are rare congenital tumors of the midline skull base. They are slow-growing and usually cause symptoms only when they compress a cranial nerve.
Recent Findings:
The MRI will show the lesion with irregular uptake of gadolinium. The only way to define the diagnosis is with tissue, and there are two primary pathologic forms of the disease. Resection approaches vary with extent of disease, surgeon preference, and critical structures involved.
Summary:
There is building evidence that proton beam therapy improves survival and duration of a disease-free state.
Insights
Chordomas are rare congenital midline skull base tumors. Emerging evidence suggests proton beam therapy can improve survival and disease-free duration for chordoma patients.
Area of Science:
- Oncology
- Neurosurgery
- Radiation Oncology
Background:
- Chordomas are rare, slow-growing congenital tumors originating from the midline skull base.
- Symptoms typically arise late, resulting from compression of adjacent cranial nerves.
Purpose of the Study:
- To review the current understanding and management of chordomas.
- To highlight diagnostic criteria and treatment modalities for skull base chordomas.
Main Methods:
- Review of existing literature on chordoma diagnosis and treatment.
- Discussion of magnetic resonance imaging (MRI) findings, including gadolinium uptake.
- Analysis of surgical resection approaches based on disease extent and critical structures.
Main Results:
- MRI reveals characteristic lesions with irregular gadolinium enhancement.
- Histopathological analysis is essential for definitive diagnosis, identifying two primary pathologic forms.
- Surgical resection strategies are tailored to individual patient factors and tumor characteristics.
Conclusions:
- Proton beam therapy is increasingly recognized for its potential to enhance survival rates.
- Evidence supports proton beam therapy in prolonging the disease-free state for chordoma patients.
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