Ch12 Vascular,Lymph, etc

Question 1

Hemangioma vs vascular malformations: __________ are considered to be benign tumors of infancy that display a rapid growth phase with endothelial cell PROLIFERATION, followed by gradual INVOLUTION

Answer 1

Hemangioma

Question 2

Hemangioma vs vascular malformations: ___________ are structural anomalies of blood vessels with NORMAL endothelial cell turnover. By definition, they are present at birth and persist throughout life.

Answer 2

vascular malformations

Question 3

What are the most common tumors of infancy?

Answer 3

hemangiomas

Question 4

Hemangioma: gender? race? location? # of tumors usually?

Answer 4

FEMALE (3:1/5:1), white, head and neck (60%), 20% have multiple tumors

Question 5

Do hemangiomas of infancy blanch?

Answer 5

no

Question 6

what % of hemangiomas regress by age 5? how many by age 9?

Answer 6

50%…90%

Question 7

What % of hemangiomas of infancy regress without scarring, telangectasias, etc?

Answer 7

50% (40% will have these..the other 10%? fuck if I know)

Question 8

What are the 2 variations of congenital hemangioma?

Answer 8

1.rapidly involuting congenital hemangioma (RICH) and 2.Noninvoluting congenital hemangioma (NICH)

Question 9

When does a RICH typically involute?

Answer 9

9 to 14 months of age

Question 10

What is the most common complication in congenital hemangiomas?

Answer 10

ulceration

Question 11

What is PHACE(S) syndrome?

Answer 11

1. Posterior fossa brain anomalies (Dandy-Walker malformation) 2.Hemangioma (cervical segmental) 3.Arterial anomalies 4.Cardiac defects and Coarctation of the aorta 5. Eye anomalies 6. Sternal cleft / Supraumbilical raphe

association between infantile haemangiomas and malformation of eyes, heart, major arteries, and brain.

Question 12

_________ phenomenon is a serious coagulopa-

thy that has been associated with two rare vascular tumors

Answer 12

Kasabach-Merritt

characterised by the combination of rapidly growing vascular tumour, thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy. The blood clotting disorder results from platelets and other clotting factors of the blood being used up within the tumor.

Question 13

Kasabach-Merritt is associated with what two rare vascular tumors?

Answer 13

tufted hemangioma and kaposiform hemangioendothelioma

Question 14

This disorder is characterized by severe thrombocy- topenia and hemorrhage because of platelet trapping within the tumor. The mortality rate is as high as 20% to 30%.

Answer 14

Kasabach-Merritt

Question 15

Port wine stains are relatively common _______ malformations that occur in 0.3% of newborns and have been associated with somatic mutations in the ____ gene.

Answer 15

capillary…GNAQ

Question 16

_________ are most common on the face, particularly along the distribution of the trigeminal nerve

Answer 16

Port wine stain

Question 17

In __________, associated intracranial lesions are present with port wine stains

Answer 17

Sturge-Weber syndrome

Question 18

As the patient gets older, port wine stains often darken and becomes nodular because of vascular ______.

Answer 18

ectasia

Question 19

Typically, ______ malformations are blue and are easily compressible. They often grow proportionately with the patient, but they may swell when dependent or with increased venous pressure. Secondary thrombosis and phlebolith formation can occur.

Answer 19

VENOUS

Question 20

Because of the FAST vascular flow through these lesions, a palpable thrill or bruit often is noticeable. The overlying skin typically feels warmer to touch.

Answer 20

Arteriovenous malformations

Question 21

When are jaw hemangiomas typically discovered?

Answer 21

first 3 decades of life

Question 22

jaw hemangioma: gender? location?

Answer 22

female, 3x more often in mandible

Question 23

Can intrabony hemangiomas cause swelling? tooth mobility? bleeding from ginigival sulcus?

Answer 23

yes, not commonly but yes

Question 24

What are 3 buzz terms for radiographic presentations of intrabony hemangiomas?

Answer 24

honeycomb, soap bubble, sunburst

Question 25

________ can be helpful in demonstrating the vascular nature of and intrabony hemangioma

Answer 25

Angiography

Question 26

Because of their similar features, many vascular malformations are incorrectly categorized as ________.

Answer 26

hemangiomas

Question 27

______ is an immunohistochemical marker that is consistently positive in the hemangioma of infancy

Answer 27

GLUT1

Question 28

Interesting.... _______ is negative in other developmental vascular tumors and anomalies (RICH, NICH, tufted angioma, kaposiform hemangioendothelioma, pyogenic granuloma, and vascular malformations)

Answer 28

GLUT1 aka GLUT1 is positive in hemangiomas of infancy(infantile hemangiomas) BUT negative in congenital hemangioms(RICH/NICH), kaposiform, tufted, and PG

Question 29

For problematic or life-threatening hemangiomas, pharmacologic therapy with _______ has become the first-line treatment in recent years.

Answer 29

the beta blocker propranolol

Question 30

Hemangioma tx: __________ also may help to reduce the size of the lesion, but this approach is associated with a greater risk potential than propranolol therapy. Intralesional and topical __________ sometimes have been used for smaller localized, problematic lesions.

Answer 30

Systemic corticosteroids...corticosteroids

Question 31

Intravenous (IV) ________ may be considered for complicated hemangiomas

Answer 31

vincristine (chemotherapy drug)

Question 32

Whats another term for port wine stain?

Answer 32

nevus flammeus

Question 33

Sclerotherapy involves the injection of sclerosing agents, such as 95% ________ or sodium ______ sulfate, directly into the lesion to induce fibrosis.

Answer 33

ethanol...tetradecyl

Question 34

What are two alternate names for Sturge-Weber Syndrome?

Answer 34

Encephalotrigeminal Angiomatosis....Sturge-Weber angiomatosis

Question 35

Sturge-Weber syndrome is a rare, nonhereditary develop- mental condition that is characterized by a ________ vascular proliferation involving the tissues of the _____ and face.

Answer 35

hamartomatous...brain

Question 36

Sturge-Weber recently was shown to be related to a somatic activating mutation of the _____ gene on chromosome 9q21.

Answer 36

GNAQ

Question 37

Sturge-Weber: embryologic mutation results in failure of the primitive cephalic venous plexus to regress and mature properly during the _____ trimester of pregnancy

Answer 37

1st

Question 38

Only __% to __% patients with facial port wine nevi will have Sturge-Weber syndrome

Answer 38

8 to 10%

Question 39

If the port-wine stain involves the entire distribution of ___, the risk for neurologic and ocular involvement is 78%

Answer 39

V1

Question 40

In addition to the facial port wine nevus, individuals with Sturge-Weber syndrome have leptomeningeal _______ that overlie the ipsilateral cerebral cortex.

Answer 40

angiomas

Question 41

meningeal angiomatosis usually is associated with a ______ disorder and often results in intellectual disability or contralateral hemiplegia.

Answer 41

convulsive

Question 42

Sturge-Weber: Imaging studies of the brain may reveal gyriform “______” calcifications on the affected side

Answer 42

tramline

Question 43

Intraoral involvement in Sturge-Weber syndrome is common, resulting in ???

Answer 43

hypervascular changes to the ipsilateral mucosa

Question 44

Name that condition: The gingiva may exhibit slight vascular hyperplasia or a more massive hemangiomatous proliferation that can resemble a pyogenic granuloma. Such gingival hyperplasia may be attributable to the increased vascular component, anticonvulsant therapy used to control the epileptic seizures, or both. Destruction of the underly- ing alveolar bone has been reported in rare instances.

Answer 44

Sturge-Weber

Question 45

The __________ is a rare vascular and fibrous tumorlike lesion that occurs only in the nasopharynx.

Answer 45

nasopharyngeal angiofibroma

Question 46

nasopharyngeal angiofibromas occur almost EXCLUSIVLEY in what gender?

Answer 46

males

Question 47

What is an alternate name for nasopharyngeal angiofibroma? (based on age it typically shows)

Answer 47

juvenile nasopharyngeal angiofibroma...10-17 years old

Question 48

nasopharyngeal angiofibroma: recurrence? malignant transformation?

Answer 48

20-40%...transition into fibrosarcoma has been reported with prior radiation therapy

Question 49

What are two alternate names for lymphatic malformations?

Answer 49

lymphangioma and cystic hygroma

Question 50

What are the three classifications of lymphatic malformations? Whats the size cut off?

Answer 50

macrocystic(>2cm), microcystic(<2cm spaces), mixed...2 cm

Question 51

Macrocystic lymphatic malformations are also called..where do they most commonly occur?

Answer 51

cystic hygroma...neck

Question 52

Lymphatic malformations have a marked predilection for the head and neck, which accounts for ___% to ___% of all cases. About ____% of all lesions are noted at birth, and around ___% develop by 2 years of age.

Answer 52

50-75%....50% at birth...90% by 2yrs

Question 53

Where are oral lymphatic malformations are most common? What does this cause?

Answer 53

anterior 2/3 of tongue causing macroglossia

Question 54

What are two clinical buzz terms for the appearance of lymphatic malformations on the tongue?

Answer 54

frog egg or tapioca pudding

Question 55

Small lymphatic anomalies less than 1 cm in size occur on the _______ in around 4% of black neonates. These lesions often occur bilaterally on the ________ and show a 2 : 1 ________ distribution.

Answer 55

alveolar ridge...mandibular ridge...male-to-female

Question 56

In WHAT ENTITY do the vessels often diffusely infiltrate the adjacent soft tissues and may demonstrate lymphoid aggregates in their walls?

Answer 56

Lymphatic malformation

Question 57

Lymphatic malformation of the tongue: tx?

Answer 57

typically not excised, high recurrence rate...In recent years, percutaneous sclerotherapy has proven to be a successful alternative to surgery for many lymphatic malformations.

Question 58

_______cystic lesions of the cervical region are often well circumscribed and have a lower rate of recurrence.

Answer 58

Macrocystic

Question 59

In the United States, the most widely used sclerosant currently is ________.

Answer 59

doxycycline

Question 60

Sclerotherapy is most successful in the management of ______cystic lesions, with most patients showing 75% to 100% clinical resolution.

Answer 60

macro

Question 61

What are the three types of leiomyomas found in the oral cavity? Which two account for 75% of cases?

Answer 61

1. Solid leiomyomas 2. vascular leiomyomas 3. epithelioid leiomyomas...(solid or vascular = 75% of cases in the mouth)

Question 62

What are the 4 most common sites for leiomyomas in the mouth? Extremely rare intraosseous examples may present as unilocular radiolucencies of the jaws.

Answer 62

lips, tongue, palate, and cheek (80% of cases)

Question 63

What special stain turns the smooth muscle cells in a leio red?

Answer 63

trichrome

Question 64

What 4 stains are positive in leios?

Answer 64

vimentin, SMA, muscle-specific actin, and desmin

Question 65

Recurrence of leio?

Answer 65

"should not recur"

Question 66

What are the two classifications of rhabdomyomas in the head and neck?

Answer 66

1.adult and 2. fetal

Question 67

Adult rhabdomyomas of the head and neck occur primarily in WHAT AGE? patients, with about 75% of cases found in ____.

Answer 67

middle age and older...MEN

Question 68

What are the 3 most common sites in the head and neck for rhabdomyomas? What are the 3 most common sites for them in the oral cavity?

Answer 68

The most frequent sites are the pharynx, oral cavity, and larynx; intraoral lesions are most common in the floor of the mouth, soft palate, and base of tongue.

Question 69

Where are fetal rhabdomyomas most common?

Answer 69

face and periauricular region

Question 70

What is a histiologic buzzword for rhabdomyomas?

Answer 70

"spider web" cytoplasm

Question 71

What 3 stains can prove a rhabdomyoma?

Answer 71

myoglobin, desmin, muscle-specific actin

Question 72

The fetal rhabdomyoma has a less mature appearance and consists of a haphazard arrangement of spindle-shaped muscle cells that sometimes are found within a ______ stroma. Some tumors may show considerable cellularity and mild pleomorphism, which makes them easily mistaken for __________.

Answer 72

myxoid....rhabdomyosarcomas

Question 73

Recurrence of rhabdomyomas has been reported in ___-____% of cases, but this largely may be due to incomplete removal.

Answer 73

10 to 42%

Question 74

A ________ is a tumorlike growth of microscopically normal tissue in an abnormal location.

Answer 74

choristoma

Question 75

What are 5 types of choristoma that can occur in the mouth?

Answer 75

gastric mucosa, glial tissue, sebaceous glands, bone, cartilage

Question 76

Osseous and cartilaginous choristomas show a striking predilection for the _______, which accounts for 85% of cases.

Answer 76

tongue

Question 77

Do choristomas recur?

Answer 77

Naw

Question 78

CT/MRI findings in nasopharyngeal angiofibroma

Answer 78

anterior bowing of the posterior wall of the maxillary sinus

Question 79

common presenting symptom of nasopharyngeal fibroma

Answer 79

nasal obstruction and epistaxis

Question 80

What % of 1 year old children have hemangiomas of infancy

Answer 80

4-5%

Question 81

What is the term for a superficial hemangioma of infancy of the skin that appears bosselated with a bright red color?

Answer 81

"strawberry" hemangioma

Question 82

What are the 2 names for a hemangioma that is early in development and therefore has unique histology? What is the name that has been OUTLAWED for this lesion?

Answer 82

1.juvenile or 2. cellular hemangioma...OUTLAWED: juvenile hemangioendothelioma (due to intermediate malignant potential)

Question 83

What are the 2 alternate names for Sturge-Weber Syndrome?

Answer 83

1.Encephalotrigeminal Angiomatosis 2.Sturge-Weber Angiomatosis

Question 84

Whats the recurrence of a lymphatic malformation? What % show spontaneous regression?

Answer 84

"high" recurrence rate....3% spontaneous regression