Background and recommendations Flashcards

1
Q

What is the downside of RT in patients with NF1?

A

High incidence of second CNS tumors

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2
Q

Late complications of RT?

A
  1. Endocrine dysfunction

2. Vision loss

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3
Q

What RT dose is recommended?

A

54 Gy at 1.8 Gy/fx

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4
Q

When is RT recommended?

A

After chemotherapy has failed, the patient has progressive symptoms or signs of intracranial extension

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5
Q

What chemotherapy options are available for patients with optic nerve gliomas?

A

Carboplatin/vincristine

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6
Q

Should these tumors be biopsied?

A

No

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7
Q

How do these tumors appear on MRI?

A

Well circumscribed, homgenously enhancing

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8
Q

DDx of optic nerve mass?

A

Optic neuritis
Retinablastoma
Optic nerve meningioma
Lymphoma

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9
Q

What ancillary studies should be done as part of work up?

A

Endocrine labs

Eye exam

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10
Q

What is the median age at diagnosis?

A

Bimodal

5 years

52 years

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11
Q

What grade are optic pathway gliomas?

A

Usually low grade

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12
Q

How often do patients with optic nerve gliomas have NF1?

A

10-38%

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13
Q

How often do patients with NF1 have optic nerve sheath gliomas?

A

15-40%

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14
Q

What are the subtypes of ONGs?

A
  1. Prechiasmal
  2. Chiasmal
  3. hypothalamic
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15
Q

What are the common locations in children? Adults?

A

Children: prechiasmal
Adults: chiasmal or hypothalamic

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16
Q

What is the local control and OS for patients with prechiasmal lesions after RT?

A

LC: 60-90%
OS: 90-100%

17
Q

What elements of history and physical do you want to acquire at work up?

A

Hx: Headaches, proptosis, changes in visual fields, or visual acuity, changes in sleep, or appetite

Physical exam: fundiscopic exam, CN nerve exam, test gait, focal weakness or numbness

18
Q

What imaging and labs are needed at work up?

A

MRI of brain

Labs: endocrine labs, CBC, CMP

19
Q

What is the general treatment paradigm for ONGs?

A
  1. Observation
  2. Chemotherapy
  3. RT if refractory to chemo in terms of symptoms, or intracranial extension