Benign Mesenchymal Tumors Part 3

Question 1

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

Answer 1

Encephalotrigeminal angiomatosis

Question 2

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

Answer 2

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

Question 3

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

Answer 3

1st, 2nd, 3rd, V

Question 4

What area of the brain does Sturge-Weber affect?

Answer 4

Deeper soft tissue and meninges

Question 5

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

Answer 5

The risk for neurologic and ocular involvement increases

Question 6

What is a classic radiographic finding of Sturge-Weber?

Answer 6

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

Question 7

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

Answer 7

Pulsed dye laser

Sturge-Weber may involve neurosurgery

Question 8

Developmental hamartoma of lymphatic vessel differentiation, rather than true neoplasm

Answer 8

Lymphangioma

Question 9

Lymphangioma can also be called ___ ____

Answer 9

Cystic hygroma

Question 10

What area of the body does lymphangioma favor?

Answer 10

H&N

Question 11

When are lymphangiomas present?

Answer 11

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

Question 12

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

Answer 12

Lymphangioma

Question 13

What color is lymphangioma if there is trauma/hemorrhage?

Answer 13

Red/purple

Question 14

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

Answer 14

Tongue - macroglossia

Question 15

Areas for lymphangioma

Answer 15

Neck, mediastinum, axilla, oral cavity

Question 16

What does lymphangioma have the potential for?

Answer 16

Potential to cause airway obstruction, especially if secondarily infected

Question 17

Describe the histology of lymphangioma

Answer 17

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

Question 18

What is the treatment of choice for lymphangioma?

Answer 18

Surgical excision

Question 19

Why is lymphangioma more difficult to treat than hemangioma?

Answer 19

It is often difficult to discern the borders of the tumor

Question 20

What is the prognosis for lymphangioma?

Answer 20

Good, but relatively high recurrence

Question 21

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

Answer 21

Airway obstruction and death

Question 22

2 types of benign mesenchymal tumors in the muscle

Answer 22

Leiomyoma and Rhabdomyoma

Question 23

Tumor of smooth muscle differentiation

Answer 23

Leiomyoma

Question 24

Where are Leiomyomas most common?

Answer 24

Uterus, GI tract, Skin

Question 25

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

Answer 25

No - vascular smooth muscle (vascular leiomyoma)

Question 26

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

Answer 26

Well Rubbery firm < 1cm

Question 27

What color are leiomyomas and why?

Answer 27

Reddish-purple due to vascular nature

Question 28

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

Answer 28

Leiomyoma

Question 29

What is the most common oral site for leiomyomas?

Answer 29

Upper lip, tongue, palate, buccal mucosa

Question 30

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

Answer 30

Spindle Cigar Eosinophilic

Question 31

Tumor with no significant atypia and no mitotic activity

Answer 31

Leiomyoma

Question 32

Tumor positive for IHC markers of muscle differentiation

Answer 32

Leiomyoma

Question 33

Treatment for leiomyoma

Answer 33

Conservative excision

Question 34

Prognosis for leiomyoma

Answer 34

Excellent, no recurrence

Question 35

Tumor of striated muscle differentiation

Answer 35

Rhabdomyomas

Question 36

Are Rhabdomyomas common or rare?

Answer 36

Rare

Question 37

Most Rhabdomyomas are ____

Answer 37

Cardiac

Question 38

Extracardiac rhabdomyomas have a striking predilection for what region?

Answer 38

H&N

Question 39

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

Answer 39

Rhabdomyoma

Question 40

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

Answer 40

Rhabdomyoma

Question 41

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

Answer 41

Desmin

Question 42

Treatment for Rhabdomyoma

Answer 42

Conservative excision

Question 43

Prognosis for Rhabdomyoma

Answer 43

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