General
- Uses methylation profiling to form subgroups
Numbers
- Increasing frequency of spinal ependymomas with age.
- 20% of primary spinal tumours in children and 18% of adults >20
- 18-53% of patients with neurofibromatosis type 2
Molecular subtypes
Molecular group | Implicated gene | Gene name | Cytogenic band | Pathogenic impact | Gene promoter | Gene suppressor |
HOXB13 | Homeobox B 13 | 17q21.32 | Amplification | Change of cellular energetics | Escaping programmed cell death | |
MYCN | MYCN proto-oncogene, BHLH transcription factor | 2p24.3 | Amplification | Proliferative signaling; escaping immunic response to cancer; angiogenesis; genome instability and mutations; change of cellular energetics | Cell replicative immortality; invasion and metastasis; escaping programmed cell death |
Management of WHO grades 2 and 3 spinal cord ependymomas
- Gross total resection is the goal of spinal ependymoma surgery.
- Postoperative MRI should be performed to evaluate the extent of resection. Because a risk of CSF dissemination exists for all patients with newly diagnosed ependymoma, disease staging, including both craniospinal MRI and CSF cytology, is recommended following surgery (not earlier than 2-3 wk).
- In case of WHO grade 3 (anaplastic) ependymomas, postoperative radiotherapy with doses of 45-54 Gy is recommended regardless of the extent of resection.
- In case of WHO grade 2 ependymomas following gross total resection, a watch-and-wait strategy is recommended.
- In case of incomplete resection of a WHO grade 2 ependymoma, postoperative local radiotherapy is recommended with doses of 45-54 Gy.
- Because of the risk of asymptomatic and/or late relapses, patients should be followed long term with an enhanced MRI.
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