Background and recommendations Flashcards
What is the downside of RT in patients with NF1?
High incidence of second CNS tumors
Late complications of RT?
- Endocrine dysfunction
2. Vision loss
What RT dose is recommended?
54 Gy at 1.8 Gy/fx
When is RT recommended?
After chemotherapy has failed, the patient has progressive symptoms or signs of intracranial extension
What chemotherapy options are available for patients with optic nerve gliomas?
Carboplatin/vincristine
Should these tumors be biopsied?
No
How do these tumors appear on MRI?
Well circumscribed, homgenously enhancing
DDx of optic nerve mass?
Optic neuritis
Retinablastoma
Optic nerve meningioma
Lymphoma
What ancillary studies should be done as part of work up?
Endocrine labs
Eye exam
What is the median age at diagnosis?
Bimodal
5 years
52 years
What grade are optic pathway gliomas?
Usually low grade
How often do patients with optic nerve gliomas have NF1?
10-38%
How often do patients with NF1 have optic nerve sheath gliomas?
15-40%
What are the subtypes of ONGs?
- Prechiasmal
- Chiasmal
- hypothalamic
What are the common locations in children? Adults?
Children: prechiasmal
Adults: chiasmal or hypothalamic