Benign Mesenchymal Tumors Part 3 Flashcards
Sturge-Weber Syndrome is a vascular malformation that is also known as _____ ______
Encephalotrigeminal angiomatosis
What are some neurological deficits that are associated with Sturge-Weber Syndrome?
Seizures, intellectual disability, hemiplegia, migraines, stroke-like episodes, growth hormone deficiency, central hypothyroidism, ocular involvement
Sturge-Weber Syndrome is a port wine stain in distribution of ____ and sometimes ____ or ____ division of cranial nerve _____
1st, 2nd, 3rd, V
What area of the brain does Sturge-Weber affect?
Deeper soft tissue and meninges
What happens if the port wine stain in Sturge-Weber involves the entire distribution of V1?
The risk for neurologic and ocular involvement increases
What is a classic radiographic finding of Sturge-Weber?
“Tram-line” calcifications seen on skull film (parallel calcifications probably represent calcified walls of abnormal blood vessels)
What is a possible treatment for port wine stain? What about a treatment for Sturge-Weber?
Pulsed dye laser
Sturge-Weber may involve neurosurgery
Developmental hamartoma of lymphatic vessel differentiation, rather than true neoplasm
Lymphangioma
Lymphangioma can also be called ___ ____
Cystic hygroma
What area of the body does lymphangioma favor?
H&N
When are lymphangiomas present?
1/2 at birth, the rest present by age 2
Has a surface similar to “frogs eggs” or tapioca pudding
Lymphangioma
What color is lymphangioma if there is trauma/hemorrhage?
Red/purple
What is the most common intraoral site for lymphangioma and what can that often result in?
Tongue - macroglossia
Areas for lymphangioma
Neck, mediastinum, axilla, oral cavity
What does lymphangioma have the potential for?
Potential to cause airway obstruction, especially if secondarily infected
Describe the histology of lymphangioma
Lymphatic vessels showing mild dilation to large cystic spaces containing fluid +/- red blood cells
What is the treatment of choice for lymphangioma?
Surgical excision
Why is lymphangioma more difficult to treat than hemangioma?
It is often difficult to discern the borders of the tumor
What is the prognosis for lymphangioma?
Good, but relatively high recurrence
What might larger lymphangiomas in the H&N result in?
Airway obstruction and death
2 types of benign mesenchymal tumors in the muscle
Leiomyoma and Rhabdomyoma
Tumor of smooth muscle differentiation
Leiomyoma
Where are Leiomyomas most common?
Uterus, GI tract, Skin
Are Leiomyomas common in the oral cavity? Where do oral regions probably arise from?
No - vascular smooth muscle (vascular leiomyoma)
Leiomyomas are ____-demarcated, ____ ____, with < ____ diameter
Well
Rubbery firm
< 1cm
What color are leiomyomas and why?
Reddish-purple due to vascular nature
Reddish-purple tumor that is slow growing, can occur at any age, and is usually painless
Leiomyoma
What is the most common oral site for leiomyomas?
Upper lip, tongue, palate, buccal mucosa
Leiomyomas are ____-shaped with ____-shaped nuclei and ______ cytoplasm
Spindle
Cigar
Eosinophilic
Tumor with no significant atypia and no mitotic activity
Leiomyoma
Tumor positive for IHC markers of muscle differentiation
Leiomyoma
Treatment for leiomyoma
Conservative excision
Prognosis for leiomyoma
Excellent, no recurrence
Tumor of striated muscle differentiation
Rhabdomyomas
Are Rhabdomyomas common or rare?
Rare
Most Rhabdomyomas are ____
Cardiac
Extracardiac rhabdomyomas have a striking predilection for what region?
H&N
Tumor of the H&N that is associated with middle-age and older males
Rhabdomyoma
Tumor with round to polygonal cells, granular eosinophilic cytoplasm, and multiple vacuolated cells with “spider web” appearance
Rhabdomyoma
Rhabdomyoma has a positivity for ____ (a skeletal muscle marker)
Desmin
Treatment for Rhabdomyoma
Conservative excision
Prognosis for Rhabdomyoma
Good, recurrence is not common (may be due to incomplete removal)
