Benign Mesenchymal Tumors Part 3 Flashcards

1
Q

Sturge-Weber Syndrome is a vascular malformation that is also known as _____ ______

A

Encephalotrigeminal angiomatosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are some neurological deficits that are associated with Sturge-Weber Syndrome?

A

Seizures, intellectual disability, hemiplegia, migraines, stroke-like episodes, growth hormone deficiency, central hypothyroidism, ocular involvement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Sturge-Weber Syndrome is a port wine stain in distribution of ____ and sometimes ____ or ____ division of cranial nerve _____

A

1st, 2nd, 3rd, V

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What area of the brain does Sturge-Weber affect?

A

Deeper soft tissue and meninges

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What happens if the port wine stain in Sturge-Weber involves the entire distribution of V1?

A

The risk for neurologic and ocular involvement increases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is a classic radiographic finding of Sturge-Weber?

A

“Tram-line” calcifications seen on skull film (parallel calcifications probably represent calcified walls of abnormal blood vessels)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is a possible treatment for port wine stain? What about a treatment for Sturge-Weber?

A

Pulsed dye laser

Sturge-Weber may involve neurosurgery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Developmental hamartoma of lymphatic vessel differentiation, rather than true neoplasm

A

Lymphangioma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Lymphangioma can also be called ___ ____

A

Cystic hygroma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What area of the body does lymphangioma favor?

A

H&N

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

When are lymphangiomas present?

A

1/2 at birth, the rest present by age 2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Has a surface similar to “frogs eggs” or tapioca pudding

A

Lymphangioma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What color is lymphangioma if there is trauma/hemorrhage?

A

Red/purple

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the most common intraoral site for lymphangioma and what can that often result in?

A

Tongue - macroglossia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Areas for lymphangioma

A

Neck, mediastinum, axilla, oral cavity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What does lymphangioma have the potential for?

A

Potential to cause airway obstruction, especially if secondarily infected

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Describe the histology of lymphangioma

A

Lymphatic vessels showing mild dilation to large cystic spaces containing fluid +/- red blood cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the treatment of choice for lymphangioma?

A

Surgical excision

19
Q

Why is lymphangioma more difficult to treat than hemangioma?

A

It is often difficult to discern the borders of the tumor

20
Q

What is the prognosis for lymphangioma?

A

Good, but relatively high recurrence

21
Q

What might larger lymphangiomas in the H&N result in?

A

Airway obstruction and death

22
Q

2 types of benign mesenchymal tumors in the muscle

A

Leiomyoma and Rhabdomyoma

23
Q

Tumor of smooth muscle differentiation

A

Leiomyoma

24
Q

Where are Leiomyomas most common?

A

Uterus, GI tract, Skin

25
Q

Are Leiomyomas common in the oral cavity? Where do oral regions probably arise from?

A

No - vascular smooth muscle (vascular leiomyoma)

26
Q

Leiomyomas are ____-demarcated, ____ ____, with < ____ diameter

A

Well
Rubbery firm
< 1cm

27
Q

What color are leiomyomas and why?

A

Reddish-purple due to vascular nature

28
Q

Reddish-purple tumor that is slow growing, can occur at any age, and is usually painless

A

Leiomyoma

29
Q

What is the most common oral site for leiomyomas?

A

Upper lip, tongue, palate, buccal mucosa

30
Q

Leiomyomas are ____-shaped with ____-shaped nuclei and ______ cytoplasm

A

Spindle
Cigar
Eosinophilic

31
Q

Tumor with no significant atypia and no mitotic activity

A

Leiomyoma

32
Q

Tumor positive for IHC markers of muscle differentiation

A

Leiomyoma

33
Q

Treatment for leiomyoma

A

Conservative excision

34
Q

Prognosis for leiomyoma

A

Excellent, no recurrence

35
Q

Tumor of striated muscle differentiation

A

Rhabdomyomas

36
Q

Are Rhabdomyomas common or rare?

A

Rare

37
Q

Most Rhabdomyomas are ____

A

Cardiac

38
Q

Extracardiac rhabdomyomas have a striking predilection for what region?

A

H&N

39
Q

Tumor of the H&N that is associated with middle-age and older males

A

Rhabdomyoma

40
Q

Tumor with round to polygonal cells, granular eosinophilic cytoplasm, and multiple vacuolated cells with “spider web” appearance

A

Rhabdomyoma

41
Q

Rhabdomyoma has a positivity for ____ (a skeletal muscle marker)

A

Desmin

42
Q

Treatment for Rhabdomyoma

A

Conservative excision

43
Q

Prognosis for Rhabdomyoma

A

Good, recurrence is not common (may be due to incomplete removal)