Benign Mesenchymal Tumors - part III start of midterm 3

Question 1

T/F: encephalotrigeminal angiomatosis (Sturge-Weber anomaly) is an inherited condition

Answer 1

false, congenital abnormality

Question 2

encephalotrigeminal angiomatosis (Sturge-Weber anomaly)

Answer 2

dermal capillary vascular malformation affecting superficial and deeper tissues in region of CN V

Question 3

neurological deficits caused by encephalotrigeminal angiomatosis (Sturge-Weber anomaly)

Answer 3

1. seizures
2. intellectual disability
3. hemiplegia
4. migraines
5. stroke-like episodes
6. growth hormone deficiency
7. central hypothyroidism
8. ocular involvement

Question 4

which division of CN V does port wine stain distribute to?

Answer 4

1st and sometimes 2nd or 3rd division

Question 5

what does encephalotrigeminal angiomatosis (Sturge-Weber anomaly) involve?

Answer 5

deeper soft tissue and meninges of brain

Question 6

which nerve is the primary sensory nerve of the head?

Answer 6

trigeminal n.

Question 7

if port wine stain involves entire distribution of V1, what is the percent risk for neurologic and ocular involvement?

Answer 7

78%

Question 8

radiographic feature of encephalotrigeminal angiomatosis (Sturge-Weber anomaly)?

Answer 8

“tramline” calcifications seen on skull film

Question 9

what does the parallel calcifications on the radiographs for encephalotrigeminal angiomatosis (Sturge-Weber anomaly) represent?

Answer 9

represent calcified walls of abnormal blood vessels

Question 10

treatment for encephalotrigeminal angiomatosis (Sturge-Weber anomaly) depends on what?

Answer 10

severity

Question 11

treatment for port wine stain

Answer 11

pulsed dye laser

Question 12

treatment for Sturge-Weber syndrome may involve what?

Answer 12

neurosurgery

Question 13

lymphangioma

Answer 13

developmental harmartoma of lymphatic vessel differentiation, rather than true neoplasm

Question 14

lymphangioma has a predilection for what location?

Answer 14

head and neck

Question 15

what does lymphangioma look similar to clinically?

Answer 15

vesicular surface similar to “frogs eggs” pr tapioca pudding

Question 16

what color is lymphangioma?

Answer 16

red-purple if trauma/hemorrhage

Question 17

what i the most common intraoral site for lymphangioma?

Answer 17

tongue

Question 18

cystic hygroma

Answer 18

a very large lymphangioma

Question 19

locations for cystic hygroma

Answer 19

1. neck
2. mediastinum
3. axilla
4. oral cavity

Question 20

T/F: cystic hygroma can cause airway obstruction especially if secondarily infected

Answer 20

true

Question 21

histopathologic features of lymphangioma

Answer 21

1. lymphatic vessels showing mild dilation to large cystic spces containing fluid, ± red blood cells
2. vessels infiltrate soft tissue
3. thin endothelial lining

Question 22

treatment for lymphangioma depends on what?

Answer 22

size of lesion

Question 23

treatment for lymphangioma

Answer 23

surgical excision

Question 24

why is it more difficult to treat hemangioma than lymphangioma?

Answer 24

because it is often difficult to discern the borders of the tumor

Question 25

prognosis for lymphangioma

Answer 25

good for most patients

Question 26

T/F: lymphangioma has low recurrence rate

Answer 26

false, high (up to 40% in some series)

Question 27

T/F: large lymphangioma tumors of the head and neck may result in airway obstruction and death

Answer 27

true

Question 28

benign muscle tumors

Answer 28

1. leiomyoma | 2. rhabdomyoma

Question 29

leiomyoma

Answer 29

tumor of smooth muscle differentiation

Question 30

most common locations of leiomyoma

Answer 30

1. uterus 2. GI tract 3. skin

Question 31

T/F: leiomyoma is common in the oral cavity

Answer 31

false, rare

Question 32

T/F: most leiomyoma that occur in the oral region probably arise from vascular smooth muscle (vascular leiomyoma)

Answer 32

true

Question 33

clinical features of leiomyoma

Answer 33

1. well-demarcated 2. rubbery firm 3. <1 cm diameter 4. slow growing 5. usually painless

Question 34

what color are leiomyoma lesions?

Answer 34

reddish-purple color due to vascular nature

Question 35

who is affected by leiomyoma?

Answer 35

any age

Question 36

what are the most common oral sites for leiomyoma?

Answer 36

1. upper lip 2. tongue 3. palate 4. buccal mucosa

Question 37

histopathologic features of leiomyoma

Answer 37

1. well-circumscribed 2. spindle-shaped. with cigar-shaped nuclei, eosinophilic cytoplasm 3. no significant atypia, no mitotic activity 4. positive for IHC markers of muscle differentiation

Question 38

angiomyoma

Answer 38

well-circumscribed tumor exhibiting prominent blood vessels surrounded by smooth muscle

Question 39

treatment for leiomyoma

Answer 39

conservative excision

Question 40

prognosis of leiomyoma

Answer 40

excellent

Question 41

T/F: there is a high chance of recurrence with leiomyoma

Answer 41

false, virtually NO chance of recurrence

Question 42

rhabdomyoma

Answer 42

tumor of striated muscle differentiation

Question 43

T/F: rhabdomyoma is common

Answer 43

false, rare

Question 44

50% rhabdomyoma are what?

Answer 44

hamartomas associated with tuberous sclerosis

Question 45

most rhabdomyomas are of what type?

Answer 45

cardiac rhabdomyomas

Question 46

extracardiac rhabdomyomas have a predilection for what location?

Answer 46

head and neck

Question 47

who is affected by rhabdomyoma in the head and neck?

Answer 47

middle-age and older males

Question 48

clinical features of rhabdomyoma in the head and neck

Answer 48

1. non-tender 2. well-circumscribed nodule (may be multinodular) 3. may grow several centimeters

Question 49

histopathologic features of rhabdomyoma

Answer 49

1. round to polygonal cells 2. granular eosinophilic cytoplasm 3. multiple vacuolated cells, many with "spider web" appearance

Question 50

treatment for rhabdomyoma

Answer 50

conservative surgical excision

Question 51

prognosis for rhabdomyoma

Answer 51

good

Question 52

T/F: recurrence for rhabodomyoma has been reported but is not common

Answer 52

true

Question 53

what may cause recurrence of rhabodomyoma?

Answer 53

may be due to incomplete removal

Question 54

skeletal muscle marker

Answer 54

desmin