Oncology

Question 1

The most common CNS tumors to cause precocious puberty

Answer 1

Hypothalamic hamartoma

Question 2

NF1 gene locus and product

Answer 2

17q11.2
Neurofibromin

Question 3

NF2 gene locus and product

Answer 3

22q12.2
Schwannomin

Question 4

Most common intramedullary tumor in NF1

Answer 4

Astrocytoma

Question 5

Most common intramedullary tumor in NF2

Answer 5

Ependymoma

Question 6

Criteria for diagnosis of NF1

Answer 6

2 or more of the following

• > =6 cafe au lait spots each are > 5 mm in prepubertal and >15 in post-pubertal.
  ->=2 skin neurofibroma of any type or 1 plexiform neurofibroma.
  -Axillary or inguinal frecklings.
  -Optic pathway glioma/pilocytic astrocytoma.
  ->=2 Lisch nodules
  -Distinctive osseous abnormalities
  -A first degree relative with NF1

Question 7

What is the typical location of a myxoid glioneuronal tumor?

Answer 7

Lateral ventricles

Question 8

What is the percentage of each type of pituitary adenoma in association with MEN 1?

Answer 8

Prolactinoma (20%)
GH (10%)
NF (5%)
ACTH (2%)
TSH and others (rare)

Question 9

Most common location for intraventricular meningioma

Answer 9

Trigone

Question 10

What are other conditions associated with NF1?

Answer 10

Aqueductal stenosis
Macrocephaly
Unilateral superior orbital defect
Cognitive impairment and learning disabilities
Kyphoscoliosis
Syringomyelia
Intracranial tumor: astrocytoma, meningioma
extracranial tumors: Schwann, cell, tumors, neuroblastoma, sarcoma, leukemia, wilm’s tumor, pheochromocytoma.
Moyamoya disease.