Ch12 Vascular,Lymph, etc Flashcards
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
Hemangioma
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.
vascular malformations
What are the most common tumors of infancy?
hemangiomas
Hemangioma: gender? race? location? # of tumors usually?
FEMALE (3:1/5:1), white, head and neck (60%), 20% have multiple tumors
Do hemangiomas of infancy blanch?
no
what % of hemangiomas regress by age 5? how many by age 9?
50%…90%
What % of hemangiomas of infancy regress without scarring, telangectasias, etc?
50% (40% will have these..the other 10%? fuck if I know)
What are the 2 variations of congenital hemangioma?
1.rapidly involuting congenital hemangioma (RICH) and 2.Noninvoluting congenital hemangioma (NICH)
When does a RICH typically involute?
9 to 14 months of age
What is the most common complication in congenital hemangiomas?
ulceration
What is PHACE(S) syndrome?
- 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.
_________ phenomenon is a serious coagulopa-
thy that has been associated with two rare vascular tumors
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.
Kasabach-Merritt is associated with what two rare vascular tumors?
tufted hemangioma and kaposiform hemangioendothelioma
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%.
Kasabach-Merritt
Port wine stains are relatively common _______ malformations that occur in 0.3% of newborns and have been associated with somatic mutations in the ____ gene.
capillary…GNAQ
_________ are most common on the face, particularly along the distribution of the trigeminal nerve
Port wine stain
In __________, associated intracranial lesions are present with port wine stains
Sturge-Weber syndrome
As the patient gets older, port wine stains often darken and becomes nodular because of vascular ______.
ectasia
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.
VENOUS
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.
Arteriovenous malformations
When are jaw hemangiomas typically discovered?
first 3 decades of life
jaw hemangioma: gender? location?
female, 3x more often in mandible
Can intrabony hemangiomas cause swelling? tooth mobility? bleeding from ginigival sulcus?
yes, not commonly but yes
What are 3 buzz terms for radiographic presentations of intrabony hemangiomas?
honeycomb, soap bubble, sunburst
________ can be helpful in demonstrating the vascular nature of and intrabony hemangioma
Angiography
Because of their similar features, many vascular malformations are incorrectly categorized as ________.
hemangiomas
______ is an immunohistochemical marker that is consistently positive in the hemangioma of infancy
GLUT1
Interesting…. _______ is negative in other developmental vascular tumors and anomalies (RICH, NICH, tufted angioma, kaposiform hemangioendothelioma, pyogenic granuloma, and vascular malformations)
GLUT1
aka GLUT1 is positive in hemangiomas of infancy(infantile hemangiomas) BUT negative in congenital hemangioms(RICH/NICH), kaposiform, tufted, and PG
For problematic or life-threatening hemangiomas, pharmacologic therapy with _______ has become the first-line treatment in recent years.
the beta blocker propranolol
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.
Systemic corticosteroids…corticosteroids
Intravenous (IV) ________ may be considered for complicated hemangiomas
vincristine (chemotherapy drug)
Whats another term for port wine stain?
nevus flammeus
Sclerotherapy involves the injection of sclerosing agents, such as 95% ________ or sodium ______ sulfate, directly into the lesion to induce fibrosis.
ethanol…tetradecyl
What are two alternate names for Sturge-Weber Syndrome?
Encephalotrigeminal Angiomatosis….Sturge-Weber angiomatosis
Sturge-Weber syndrome is a rare, nonhereditary develop- mental condition that is characterized by a ________ vascular proliferation involving the tissues of the _____ and face.
hamartomatous…brain
Sturge-Weber recently was shown to be related to a somatic activating mutation of the _____ gene on chromosome 9q21.
GNAQ
Sturge-Weber: embryologic mutation results in failure of the primitive cephalic venous plexus to regress and mature properly during the _____ trimester of pregnancy
1st
Only __% to __% patients with facial port wine nevi will have Sturge-Weber syndrome
8 to 10%
If the port-wine stain involves the entire distribution of ___, the risk for neurologic and ocular involvement is 78%
V1
In addition to the facial port wine nevus, individuals with Sturge-Weber syndrome have leptomeningeal _______ that overlie the ipsilateral cerebral cortex.
angiomas
meningeal angiomatosis usually is associated with a ______ disorder and often results in intellectual disability or contralateral hemiplegia.
convulsive
Sturge-Weber: Imaging studies of the brain may reveal gyriform “______” calcifications on the affected side
tramline
Intraoral involvement in Sturge-Weber syndrome is common, resulting in ???
hypervascular changes to the ipsilateral mucosa
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.
Sturge-Weber
The __________ is a rare vascular and fibrous tumorlike lesion that occurs only in the nasopharynx.
nasopharyngeal angiofibroma
nasopharyngeal angiofibromas occur almost EXCLUSIVLEY in what gender?
males
What is an alternate name for nasopharyngeal angiofibroma? (based on age it typically shows)
juvenile nasopharyngeal angiofibroma…10-17 years old
nasopharyngeal angiofibroma: recurrence? malignant transformation?
20-40%…transition into fibrosarcoma has been reported with prior radiation therapy
What are two alternate names for lymphatic malformations?
lymphangioma and cystic hygroma
What are the three classifications of lymphatic malformations? Whats the size cut off?
macrocystic(>2cm), microcystic(<2cm spaces), mixed…2 cm
Macrocystic lymphatic malformations are also called..where do they most commonly occur?
cystic hygroma…neck
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.
50-75%….50% at birth…90% by 2yrs
Where are oral lymphatic malformations are most common? What does this cause?
anterior 2/3 of tongue causing macroglossia
What are two clinical buzz terms for the appearance of lymphatic malformations on the tongue?
frog egg or tapioca pudding
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.
alveolar ridge…mandibular ridge…male-to-female
In WHAT ENTITY do the vessels often diffusely infiltrate the adjacent soft tissues and may demonstrate lymphoid aggregates in their walls?
Lymphatic malformation
Lymphatic malformation of the tongue: tx?
typically not excised, high recurrence rate…In recent years, percutaneous sclerotherapy has proven to be a successful alternative to surgery for many lymphatic malformations.
_______cystic lesions of the cervical region are often well circumscribed and have a lower rate of recurrence.
Macrocystic
In the United States, the most widely used sclerosant currently is ________.
doxycycline
Sclerotherapy is most successful in the management of ______cystic lesions, with most patients showing 75% to 100% clinical resolution.
macro
What are the three types of leiomyomas found in the oral cavity? Which two account for 75% of cases?
- Solid leiomyomas 2. vascular leiomyomas 3. epithelioid leiomyomas…(solid or vascular = 75% of cases in the mouth)
What are the 4 most common sites for leiomyomas in the mouth? Extremely rare intraosseous examples may present as unilocular radiolucencies of the jaws.
lips, tongue, palate, and cheek (80% of cases)
What special stain turns the smooth muscle cells in a leio red?
trichrome
What 4 stains are positive in leios?
vimentin, SMA, muscle-specific actin, and desmin
Recurrence of leio?
“should not recur”
What are the two classifications of rhabdomyomas in the head and neck?
1.adult and 2. fetal
Adult rhabdomyomas of the head and neck occur primarily in WHAT AGE? patients, with about 75% of cases found in ____.
middle age and older…MEN
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?
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.
Where are fetal rhabdomyomas most common?
face and periauricular region
What is a histiologic buzzword for rhabdomyomas?
“spider web” cytoplasm
What 3 stains can prove a rhabdomyoma?
myoglobin, desmin, muscle-specific actin
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 __________.
myxoid….rhabdomyosarcomas
Recurrence of rhabdomyomas has been reported in ___-____% of cases, but this largely may be due to incomplete removal.
10 to 42%
A ________ is a tumorlike growth of microscopically normal tissue in an abnormal location.
choristoma
What are 5 types of choristoma that can occur in the mouth?
gastric mucosa, glial tissue, sebaceous glands, bone, cartilage
Osseous and cartilaginous choristomas show a striking predilection for the _______, which accounts for 85% of cases.
tongue
Do choristomas recur?
Naw
CT/MRI findings in nasopharyngeal angiofibroma
anterior bowing of the posterior wall of the maxillary sinus
common presenting symptom of nasopharyngeal fibroma
nasal obstruction and epistaxis
What % of 1 year old children have hemangiomas of infancy
4-5%
What is the term for a superficial hemangioma of infancy of the skin that appears bosselated with a bright red color?
“strawberry” hemangioma
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?
1.juvenile or 2. cellular hemangioma…OUTLAWED: juvenile hemangioendothelioma (due to intermediate malignant potential)
What are the 2 alternate names for Sturge-Weber Syndrome?
1.Encephalotrigeminal Angiomatosis 2.Sturge-Weber Angiomatosis
Whats the recurrence of a lymphatic malformation? What % show spontaneous regression?
“high” recurrence rate….3% spontaneous regression
