Benign Mesenchymal Tumors - part III start of midterm 3 Flashcards
T/F: encephalotrigeminal angiomatosis (Sturge-Weber anomaly) is an inherited condition
false, congenital abnormality
encephalotrigeminal angiomatosis (Sturge-Weber anomaly)
dermal capillary vascular malformation affecting superficial and deeper tissues in region of CN V
neurological deficits caused by encephalotrigeminal angiomatosis (Sturge-Weber anomaly)
- seizures
- intellectual disability
- hemiplegia
- migraines
- stroke-like episodes
- growth hormone deficiency
- central hypothyroidism
- ocular involvement
which division of CN V does port wine stain distribute to?
1st and sometimes 2nd or 3rd division
what does encephalotrigeminal angiomatosis (Sturge-Weber anomaly) involve?
deeper soft tissue and meninges of brain
which nerve is the primary sensory nerve of the head?
trigeminal n.
if port wine stain involves entire distribution of V1, what is the percent risk for neurologic and ocular involvement?
78%
radiographic feature of encephalotrigeminal angiomatosis (Sturge-Weber anomaly)?
“tramline” calcifications seen on skull film
what does the parallel calcifications on the radiographs for encephalotrigeminal angiomatosis (Sturge-Weber anomaly) represent?
represent calcified walls of abnormal blood vessels
treatment for encephalotrigeminal angiomatosis (Sturge-Weber anomaly) depends on what?
severity
treatment for port wine stain
pulsed dye laser
treatment for Sturge-Weber syndrome may involve what?
neurosurgery
lymphangioma
developmental harmartoma of lymphatic vessel differentiation, rather than true neoplasm
lymphangioma has a predilection for what location?
head and neck
what does lymphangioma look similar to clinically?
vesicular surface similar to “frogs eggs” pr tapioca pudding
what color is lymphangioma?
red-purple if trauma/hemorrhage
what i the most common intraoral site for lymphangioma?
tongue
cystic hygroma
a very large lymphangioma
locations for cystic hygroma
- neck
- mediastinum
- axilla
- oral cavity
T/F: cystic hygroma can cause airway obstruction especially if secondarily infected
true
histopathologic features of lymphangioma
- lymphatic vessels showing mild dilation to large cystic spces containing fluid, ± red blood cells
- vessels infiltrate soft tissue
- thin endothelial lining
treatment for lymphangioma depends on what?
size of lesion
treatment for lymphangioma
surgical excision
why is it more difficult to treat hemangioma than lymphangioma?
because it is often difficult to discern the borders of the tumor
prognosis for lymphangioma
good for most patients
T/F: lymphangioma has low recurrence rate
false, high (up to 40% in some series)
T/F: large lymphangioma tumors of the head and neck may result in airway obstruction and death
true
benign muscle tumors
- leiomyoma
2. rhabdomyoma
leiomyoma
tumor of smooth muscle differentiation
most common locations of leiomyoma
- uterus
- GI tract
- skin
T/F: leiomyoma is common in the oral cavity
false, rare
T/F: most leiomyoma that occur in the oral region probably arise from vascular smooth muscle (vascular leiomyoma)
true
clinical features of leiomyoma
- well-demarcated
- rubbery firm
- <1 cm diameter
- slow growing
- usually painless
what color are leiomyoma lesions?
reddish-purple color due to vascular nature
who is affected by leiomyoma?
any age
what are the most common oral sites for leiomyoma?
- upper lip
- tongue
- palate
- buccal mucosa
histopathologic features of leiomyoma
- well-circumscribed
- spindle-shaped. with cigar-shaped nuclei, eosinophilic cytoplasm
- no significant atypia, no mitotic activity
- positive for IHC markers of muscle differentiation
angiomyoma
well-circumscribed tumor exhibiting prominent blood vessels surrounded by smooth muscle
treatment for leiomyoma
conservative excision
prognosis of leiomyoma
excellent
T/F: there is a high chance of recurrence with leiomyoma
false, virtually NO chance of recurrence
rhabdomyoma
tumor of striated muscle differentiation
T/F: rhabdomyoma is common
false, rare
50% rhabdomyoma are what?
hamartomas associated with tuberous sclerosis
most rhabdomyomas are of what type?
cardiac rhabdomyomas
extracardiac rhabdomyomas have a predilection for what location?
head and neck
who is affected by rhabdomyoma in the head and neck?
middle-age and older males
clinical features of rhabdomyoma in the head and neck
- non-tender
- well-circumscribed nodule (may be multinodular)
- may grow several centimeters
histopathologic features of rhabdomyoma
- round to polygonal cells
- granular eosinophilic cytoplasm
- multiple vacuolated cells, many with “spider web” appearance
treatment for rhabdomyoma
conservative surgical excision
prognosis for rhabdomyoma
good
T/F: recurrence for rhabodomyoma has been reported but is not common
true
what may cause recurrence of rhabodomyoma?
may be due to incomplete removal
skeletal muscle marker
desmin
