neuro tumors

Question 1

neurocutaneous syndromes

Answer 1

group of hereditary conditions characterized by concomitant neoplastic or hamartomatous lesions predominantly affecting the skin and nervous system

Question 2

subependmal giant cell astrocytoma

Answer 2

astrocytic tumor growing from walls of lateral ventricles

Question 3

subependmal giant cell astrocytoma is pathognomonic of…

Answer 3

tuberous sclerosis which is caused by mutations of the TS1 or TS2 genes

Question 4

mutations of the TS1 or TS2 genes cause

Answer 4

tuberous sclerosis

Question 5

tuberous sclerosis, caused by mutations of the TS1 or TS2 genes cause, manifests with multiple hamartomatous lesions of the skin, CNS and visceral organs including (6)…

Answer 5

1. subependmal giant cell astrocytoma
2. cortical tubers (malformed nodules of the cortex probably resulting from faulty cortical dvpmt)
3. hypopigmented “ash leaf” spots of the skin
4. cardiac rhabdomyomas
5. renal angiomyolipomas
6. facial lesions (adenoma sebaceum)

Question 6

“cafe-au-lait spots”

Answer 6

pigmented macules found in both types of neurofibromatosis

Question 7

hemangioblastoma

Answer 7

vascular tumor of unknown histologic origin that frequently develops in the cerebellum of pt with von-Hippel-Lindau syndrome

Question 8

von-Hippel-Lindau syndrome genetics

Answer 8

an autosomal dominant mutation of the tumor suppressor VHL gene

Question 9

von-Hippel-Lindau syndrome is commonly associated with

Answer 9

1. hemangioblastoma (vascular tumor in cerebellum)

2. renal cell carcinomas

Question 10

Lisch nodules- what are they? what disease are they assoc with?

Answer 10

small, pigmented, nodular lesions of a hamartomatous nature that are present in the iris of pt with neurofibromatosis type 1

Question 11

Lisch nodules are assoc with what disease?

Answer 11

neurofibromatosis type 1

Question 12

schwannomas of the 8th cranial nerve, especially when bilateral, are assoc with what disease?

Answer 12

neurofibromatosis type 2

Question 13

tuberous sclerosis- genetics?

Answer 13

autosomal dominant

Question 14

someone with the following has what?

1. subependmal giant cell astrocytoma
2. cortical tubers (malformed nodules of the cortex probably resulting from faulty cortical dvpmt)
3. hypopigmented “ash leaf” spots of the skin
4. cardiac rhabdomyomas
5. renal angiomyolipomas
6. facial lesions (adenoma sebaceum)

Answer 14

tuberous sclerosis (an autosomal dominant mutations)

Question 15

most common cause of a bitemporal hemianopsia (the bilat. loss of periph vision)

Answer 15

mass in the sella turcica (like a pituitary adenoma, craniopharyngioma or neurosarcoid lesion)

Question 16

compression of the optic chiasm by a mass at the sella turcica leads to

Answer 16

bitemporal hemianopsia (loss of periph vision in both eyes) bc nasal fibers are compressed

Question 17

most common type of pituitary adenoma

Answer 17

prolactinoma

Question 18

prolactinomas usually cause…

Answer 18

secrete prolactin —? galactorrhea (milky dischareg) + bitemporal hemianopsia (loss of periph vision because optic chiasm is compressed at the sella turcica) + amenorrhea