Benign Soft Tissue Tumors and Vascular Malformations II

Question 1

What are vascular malformations?

Answer 1

Structural anomalies of blood vessels with no endothelial proliferation

Present at birth and persist through life

Question 2

How can vascular malformations be categorized?

Answer 2

Can be categorized by type of vessels involved and according to hemodynamic features

Question 3

What are the clinical features of vascular malformations?

Answer 3

Port wine stains are common esp on the face along the dsitribution of the trigeminal nerve

Typicall pink or purple macular lesions that grow commensurately with the patient

Question 4

How do vascular malformations lesion colors change over time?

Answer 4

Lesions farkens and becomes nodular because of vascular ectasia over time

Question 5

What is the clinical appearance low flow venous malformations?

Answer 5

typically blue and easily compressible

Grows proportionately with patient, may swell when dependent or with increased venous pressure

Secondary thrombosis and phlebolith formation can occur

Isolated ecstasias to complex growths involving multiple organs

Question 6

What are the clinical features of arteriovenous malformations?

Answer 6

High flow lesions that result from persistent direct arterial and venous communication

Because of fast vascular flow through these lesions, a palpable thrill or bruit is noticeable

Overlying skin feels warmer to touch

Presenting symptoms = pain, bleeding and skin ulceration

Question 7

Clinical features of intrabony vascular malformations

Answer 7

mobility of teeth or bleeding from the gingival sulcus may occur

Bruit or pulsation may be apparent on asuculation and palpation

Multilocular raidoluscent defect

Individual malformations may be small (honeycomb) or large (soap bubble)

Question 8

What can large intrabony vascular malformations cause?

Answer 8

Large intrabony vascular malformations can cause cortical expansion, and occasionally a sunburst radiographic pattern is produced

Question 9

Histopathologic features of vascular malformations

Answer 9

capillary malformations – vascular proliferation forming multiple capillary blood vessels

venous malformations – multiple, large dilated blood vessels

Question 10

Tx for vascular malformaition port wine stains

Answer 10

flashlamp pulsed dye lasers

Question 11

Tx for small stable vascular malformations

Answer 11

no Tx

Question 12

Tx for large problematic vascular malformation lesions

Answer 12

combination of sclerotherapy (sclerosing agent induces fibrosis) and surgical excision

Question 13

Tx for arteriovenous malformation?

Answer 13

depends on size and degre of involvement of vitla structures

Question 14

Why must vascular malformation lesions be aspirated before biopsy or extraction of teeth on vascular malformation area?

Answer 14

Fatal hemorrhages have
occurred after incisional
biopsy or extraction of teeth
in the area of such lesions

Question 15

What is Sturge-Weber angiomatosis?

Answer 15

Rare nonhereditary developmental condition that is characterized by hamartomatous vascular proliferation involving the tissues of the brain and face

Question 16

What are the non-oral clinical features of Sturge-Weber angiomatosis?

Answer 16

Patients born with port wine stain or nevus flammeus (dermal capillary vascular malformation of the face). Unilateral distribution along the trigeminal nerve

Affected individuals have leptomeningeal angiomas that overlie the ipsilateral cerebral cortex. Associated with convulsive disorder and results in mental retardation or contralateral hemiplegia

Brain reveals gyriform “tramline” calcifications on the affected side

Glaucoma and vascular malformation on the conjuctiva, episclera, choroid and retina

Question 17

What are the oral manifestations of Sturge-Weber angiomatosis?

Answer 17

Gingiva may exhibit slight vascular hyperplasia or a more massive hemangiomatous proliferation. May be from increased vascular component, phenytoin therapy used to control epileptic seizures or both

Destruction of underlying alveolar bone seen in some cases

Question 18

What are the histopathologic features of Sturge-Weber angiomatosis?

Answer 18

Port wine nevus is characterized by excessive numbers of dilated blood vessels in the middle and deep dermis

Intraoral lesions show vascular dilation

Proliferative gingival lesions may resemble pyogenic granuloma

Question 19

Tx for Sturge-Weber angiomatosis

Answer 19

Tx depends on the nature and severity of the possibile clinical features

Port wine nevi can be improved with flashlamp pulsed laser dyes

Cortical excision of the angiomatous meningeal lesions

PTs with intractable epilepsy and progressive mental retardation may require further neurological tx (lobectomy or hemispherectomy)

Question 20

How can port wine nevi in Sturge-Weber angiomatosis affect oral health?

Answer 20

Can make flossing and dental prophy difficult

Question 21

What is a lymphangioma?

Answer 21

Benign hamartomatous tumors of lymphatic vessels

Developmental malformation that arises from the sequestrations of lymphatic tissue that do not communicate normally with the rest of the lymphatic system

Question 22

What is the clinical appearance of lymphangioma lesions?

Answer 22

Pebbly, vesicle appearance

Question 23

What part of the body does lymphagioma usually occur?

Answer 23

Marked predilection for the head and neck (50-75% of all cases)

Question 24

What is the histopathologic feature of cavernous lymphangioma?

Answer 24

dilated lymphatic vessels beneath the atrophic surface epithelium and in the deeper CT

Question 25

Tx for lymphangioma

Answer 25

surgical excision

lymphangioma does not respond to sclerosing agents like hemangiomas

Question 26

Reccurence in lymphangiomas?

Answer 26

Recurrence is common

Question 27

Prognosis for lymphangiomas?

Answer 27

Prognosis is good for most patients although some large tumors may result in airway obstruction

Question 28

What is Leiomyoma?

Answer 28

Benign tumor of smooth muscle

Question 29

Where are Leiomyomas most often seen?

Answer 29

Uterus, GI tract and skin

Question 30

How often do Leiomyomas occur in the oral cavity?

Answer 30

Rarely

Question 31

What are the clinical features of Leiomyoma lesions?

Answer 31

Usually a slow-growing, firm mucosal nodule

Oral Leiomyoma can occur at any age

Question 32

What kind of stain is used to determine the histopathologic features of Leiomyoma and what are the features?

Answer 32

Masson trichoma stain shows bundles of smooth muscle with normal collagen

Question 33

How are oral Leiomyoma treated?

Answer 33

local surgical excision

Question 34

Recurrence for oral Leiomyoma

Answer 34

lesions should not recur

Question 35

What is a rhabdomyoma?

Answer 35

benign neoplasm of skeletal muscle

Question 36

Where do rhabdomyomas often occur?

Answer 36

Predilection for the head and neck

Question 37

Histopathologic appearance of rhabdomyomas?

Answer 37

Uniform tumor composed of rounded and polygonal cells with focal vacuolization (spider web appearance)

Cells exhibit abundant granular eosinophilic cytoplasm

PTAH stain demonstrates focal cross striations in some cells