88 Neurocutaneous Disorders

Question 1

Peripheral Nerve Sheath Tumors

• What do these usually appear as?
• What cells are usually involved?
• What age do these manifest?
• What genes are usually involved?

Answer 1

Question 2

Schwannomas

• What are these?
• What cranial nerve is usually affected?
  
  • How does this manifest?

Answer 2

Question 3

Schwannomas

• What do about 10% of patients have? (Genetic Condition)
  
  • What is the hallmark of this condition?
  • What do these patients NOT have?

Answer 3

Question 4

Schwannomas and NF2

• What gene shows a dominant loss of function?
  
  • What does the protein that this gene encodes fro actually do?

Answer 4

Question 5

Schwannomas

• What do these appear as, grossly?
• What is another name for these?

Answer 5

Question 6

Schwannomas

• What do these shows show a mixture of, microscopically?

Answer 6

Question 7

Schwannomas

• What occurs in the dense Antoni A areas?
• What are Verocay bodies?

Answer 7

Question 8

Schwannomas

Answer 8

Question 9

Schwannomas

• What is seen in the Antoni B areas?

Answer 9

Question 10

Neurofibromas

• What are the 3 benign subtypes?

Answer 10

Question 11

Localized Cutaneous Neurofibromas

• What do these arise as?
• What syndrome can cause multiple lesions?

Answer 11

Question 12

Plexiform Neurofibromas

• How do these grow?
• What syndrome are they associated with?
• Can they be malignant?

Answer 12

Question 13

Diffuse Neurofibromas

• What do these usually take the form of?
• What condition are they usually associated with?

Answer 13

Question 14

Neurofibromas

• Are these encapsulated?
• What does the background stromas of these cells look like?

Answer 14

Question 15

Plexiform Neurofibromas

• What is involved from multiple affected nerves?

Answer 15

Question 16

NF1

• What kind of inheritance does this have?
• What gene is associated with this?
  
  • What dos the associated protein do?

Answer 16

Question 17

NF1

• What kind of nodules are associated with this?

Answer 17

Question 18

Schwannomatosis

• How rare is this?
• What does it NOT involve?
• What gene is implicated?

Answer 18

Question 19

Malignant Peripheral Nerve Sheath Tumors

• Who gets this?
• What condition is it associated with?
  
  • Which neurofibroma is it associated with?

Answer 19

Question 20

Malignant Peripheral Nerve Sheath Tumors

• What 5 features are seen on a histological scale?

Answer 20