Oral and Maxillofacial Pathology: Chapter 12: Soft Tissue Tumors Flashcards

What is the most common "tumor" of the oral cavity?

Clinical Features

• associated with irritation or trauma
• occurs on buccal mucosa, tongue, and gingiva
• smooth-surfaced pink nodule
• similar in color to surrounding mucosa
• may appear white from hyperkeratosis
• most 1.5 cm or less
• no symptoms, unless due to secondary trauma
• most common in older patients (fourth to sixth decades)
• more common in females than males (2 : 1)

Histopathologic Features

• nodular mass of fibrous connective tissue
• covered by stratified squamous epithelium
• not encapsulated
• collagen bundles in haphazard patterns

Pink nodule of the posterior buccal mucosa near the level of the occlusal plane (along the "bite line").

Black patient with a smooth-surfaced gray-brown pigmented nodule on the buccal mucosa near the commissure.

A firm smooth-surfaced pink nodule on the lateral border of the tongue, similar in color to the surrounding tissue.

A smooth-surfaced, pink nodular mass of the palatal gingiva between the cuspid and first bicuspid.

A fibrous hyperplasia presenting as a small, asymptomatic, exophytic growth attached to the maxillary labial frenum.

Low-power view showing an exophytic nodular mass of dense fibrous connective tissue covered by stratified squamous epithelium.

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High-power view demonstrating dense collagen bundles beneath the surface of stratified squamous epithelium.

What is the treatment and prognosis for fibroma?

Clinical Features

• not associated with irritation or trauma
• asymptomatic sessile or pedunculated nodule
• usually less than 1 cm in size
• most often diagnosed in first three decades (60%)
• occur on ginviva (50%), tongue, or palate
• occur twice as often on the mandibular gingiva

Histopathologic Features

• mass of vascular, loose fibrous connective tissue
• numerous large, stellate fibroblasts
• pebbly surface with thin and atrophic epithelium

Exophytic nodule on the dorsum of the tongue. Compared with irritation fibroma, this lesion usually occurs at a younger age.

Papillary growth on the lingual mandibular gingiva. Because of the rough surface, this lesion would be easily mistaken for a papilloma.

Bilateral papular lesions on the gingiva lingual to the mandibular canines, which are microscopically similar to giant cell fibroma.

1. Low-power view showing a nodular mass of fibrous connective tissue covered by stratified squamous epithelium. Note the elongation of the rete ridges.
2. High-power view showing multiple large stellate-shaped and multinucleated fibroblasts.

What is the treatment and prognosis for giant cell fibroma?

Clinical Features

• associated with ill-fitting dentures
• folds of hyperplastic tissue in alveolar vestibule
• usually firm and fibrous, but may be ulcerated
• range from less than 1 cm to the entire vestibule
• usually on the facial aspect of the alveolar ridge
• most often occurs in middle-aged and older adults
• pronounced female predilection (66 to 75%)

Histopathologic Features

• hyperplasia of the fibrous connective tissue
• grooves where denture impinges on tissue
• hyperparakeratotic epithelium
• irregular hyperplasia of the rete ridges
• variable chronic inflammatory infiltrate
• rare formation of osteoid or chondroid

Hyperplastic folds of tissue in the anterior maxillary vestibule in a denture-wearing patient.

1. Several folds of hyperplastic tissue in the maxillary vestibule.
2. An ill-fitting denture fits into the fissure between two of the folds.

Redundant folds of tissue arising in the floor of the mouth in association with a mandibular denture.

Flattened mass of tissue arising on the hard palate beneath a maxillary denture; note its pedunculated nature.

Low-power photomicrograph demonstrating folds of hyperplastic fibrovascular connective tissue covered by stratified squamous epithelium.

What is the treatment and prognosis for epulis fissuratum?

Clinical Features

• usually occurs on hard palate beneath a denture base
• usually asymptomatic
• erythematous mucosa with pebbly or papillary surface
• often associated with denture stomatitis

Histopathologic Features

• numerous papillary growths
• hyperplastic, stratified squamous epithelium
• chronic inflammatory cell infiltrate is usually seen

Erythematous, pebbly appearance of the palatal vault in a denture-wearing patient.

An advanced case exhibiting pronounced papular lesions of the hard palate in a denture-wearing patient.

Medium-power view showing fibrous and epithelial hyperplasia resulting in papillary surface projections. Heavy chronic inflammation is present.

Higher-power view showing pseudoepitheliomatous hyperplasia of the epithelium. This epithelium has a bland appearance that should not be mistaken for carcinoma.

What is the treatment and prognosis for inflammatory papillary hyperplasia?

Clinical Features

• most on the skin of the extremities
• tend to occur orally in middle-aged and older adults
• tend to occur cutaneously in younger adults
• painless nodular mass
• can reach several centimeters in size

Histopathologic Features

• spindle-shaped fibroblastic cells with vesicular nuclei
• arranged in short, intersecting fascicles ("storiform")
• poorly defined margins

A nodular mass on the dorsum of the tongue. These lesions more commonly occur on the skin, especially in younger patients.

Medium-power view of a skin tumor showing spindle-shaped cells arranged in a storiform pattern.

High-power view of a skin tumor demonstrating spindle-shaped cells with vesicular nuclei.

What is the treatment and prognosis for fibrous histiocytoma?

Clinical Features

• reported primarily in adults (rare in children)
• slow-growing, painless, soft tissue mass
• easily removed from the surrounding tissues
• most common orally in the buccal mucosa

Histopathologic Features

• well-circumscribed lesions
• blood vessels may show irregular "staghorn" branching
• cells may arranged in a disorganized “patternless pattern”
• positive for CD34 and bcl-2 in nearly all cases

1. "Staghorn” blood vessels surrounded by haphazardly arranged cells.
2. Moderately cellular fibrous proliferation (“patternless pattern”) with prominent vascularity, slightly myxoid areas, and scattered dense collagen bundles.

What is the treatment and prognosis for solitary fibrous tumor?

Clinical Features

• intermediate between benign lesion and fibrosarcoma
• firm, painless mass
• may exhibit rapid or insidious growth
• most frequently occurs in children or young adults
• most orally site in paramandibular soft tissues
• can grow to considerable size
• adjacent bone destruction may be observed

Histopathologic Features

• spindle-shaped cells arranged in streaming fascicles
• variable amount of collagen
• cells lack hyperchromatism and pleomorphism

Streaming fascicles of spindle-shaped fibroblastic cells that demonstrate little pleomorphism.

What is the treatment and prognosis for fibromatosis?

Clinical Features

• spindle cell neoplasm of myofibroblasts
• most frequent in the first four decades (22 years)
• occur in the mandible, tongue, and buccal mucosa
• painless mass, sometimes exhibits rapid enlargement
• intrabony tumors create radiolucent defects

Histopathologic Features

• interlacing bundles of spindle cells
• tapered or blunt-ended nuclei and eosinophilic cytoplasm
• positive for smooth muscle actin and muscle-specific actin

Computed tomography (CT) scan showing an expansile lytic mass of the posterior mandible on the left side of the illustration.

Proliferation of spindle-shaped cells with both fibroblastic and smooth muscle features.

What is the treatment and prognosis for myofibroma?

Clinical Features

• most common in young adults
• predilection for females (2 : 1)
• most common on gingiva (75%) and hard palate
• sessile or pedunculated, painless nodular mass
• same color as the surrounding mucosa
• up to 2 cm in diameter

Histopathologic Features

• well-localized but nonencapsulated
• myxomatous connective tissue
• surrounded by denser, collagenous connective tissue
• often causes flattening of the rete ridges
• fibroblasts with delicate, fibrillar processes

Nodular mass arising from the gingiva between the mandibular first and second molars.

Low-power view showing a nodular mass of loose, myxomatous connective tissue surrounded by denser, collagenous connective tissue.

High-power view demonstrating the myxomatous change and fibroblasts with delicate, fibrillar processes.

Clinical Features

• smooth or lobulated mass, usually pedunculated
• characteristically ulcerated surface
• pink to purple, depending on age of lesion
• may measure up to several centimeters
• typically painless, but often bleeds easily
• may exhibit rapid growth
• occur on ginviga (85%), lips, tongue, buccal mucosa
• most common in children and young adults
• female predilection, especially pregnant mothers

Histopathologic Features

• highly vascular proliferation resembling granulation tissue
• endothelium-lined channels engorged with red blood cells
• vessels sometimes are organized in lobular aggregates
• surface is usually ulcerated
• mixed inflammatory cell infiltrate

Erythematous, hemorrhagic mass arising from the maxillary anterior gingiva.

Ulcerated and lobulated mass on the dorsum of the tongue.

Unusually large lesion arising from the palatal gingiva in association with an orthodontic band. The patient was pregnant.

1. Large gingival mass in a pregnant woman just before childbirth.
2. The mass has decreased in size and undergone fibrous maturation 3 months after childbirth.

Nodular mass of granulation tissue resembling a pyogenic granuloma that developed in a recent extraction site.

Low-power view showing an exophytic mass of granulation-like tissue with an ulcerated surface. Note the lobular endothelial proliferation in the deeper connective tissue.

Higher-power view of an exophytic mass of granulation-like tissue showing capillary blood vessels and scattered inflammation.

What is the treatment and prognosis for pyogenic granuloma?

Clinical Features

• occur exclusively on gingiva or edentulous alveolar ridge
• red or red-blue nodular mass
• most are smaller than 2 cm
• can be sessile or pedunculated
• may or may not be ulcerated
• most occur in first to sixth decades
• slight female predilection (52% to 60%)

Histopathologic Features

• proliferation of multinucleated giant cells
• background of plump mesenchymal cells
• mitotic figures are fairly common
• hemosiderin deposits from abundant hemorrhage

Nodular blue-purple mass of the mandibular gingiva. These lesions occur exclusively on gingiva or edentulous alveolar ridge.

Ulcerated mass of the mandibular gingiva. These lesions occur exclusively on gingiva or edentulous alveolar ridge.

Low-power view showing a nodular proliferation of multinucleated giant cells within the gingiva.

High-power view showing scattered multinucleated giant cells within a hemorrhagic background of ovoid and spindle-shaped mesenchymal cells.

What is the treatment and prognosis for peripheral giant cell granuloma?

Clinical Features

• occur exclusively on the gingiva
• nodular mass, either pedunculated or sessile
• usually emanates from interdental papilla
• color ranges from red to pink
• surface is frequently ulcerated
• most are less than 2 cm
• most occur in teenagers (10 to 19 years)
• more frequent in females (66%)
• most common in maxillary incisor-cuspid region

Histopathologic Features

• fibrous proliferation associated with mineralized product
• bone, cementum-like material, or dystrophic calcifications
• multinucleated giant cells may be found

This red, ulcerated mass of the maxillary gingiva has recurred twice. Such ulcerated lesions are easily mistaken for a pyogenic granuloma.

Pink, nonulcerated mass arising from the maxillary gingiva. The remaining roots of the first molar are present.

Ulcerated gingival mass demonstrating fibrous proliferation and formation of focal early mineralization.

1. Nonulcerated fibrous mass of the gingiva showing central bone formation.
2. Higher-power view showing trabeculae of bone with adjacent fibrous connective tissue.

What is the treatment and prognosis for peripheral ossifying fibroma?

What is the most common mesenchymal neoplasm?

Clinical Features

• soft, smooth-surfaced nodular mass
• can be sessile or pedunculated
• asymptomatic and usually less than 3 cm
• often yellowish, but deeper lesions are pinker
• occur intraorally on buccal mucosa and vestibule
• most patients are 40 years or older
• more common in obese people

Histopathologic Features

• well circumscribed with a thin fibrous capsule
• distinct lobular arrangement of cells
• may have a fibrous component (fibro-type)
• may contain blood vessels (angio-type)
• may contain spindle cells (spindle-cell type)
• may contain bizzare giant cells (pleomorphic type)
• may infiltrate skeletal muscle (intramuscular type)

Soft, yellow nodular mass in the floor of the mouth. Deeper lesions of this type may be more pink in appearance.

Nodular mass of the posterior buccal mucosa. Similar presentations may actually represent herniation of the buccal fat pad through the buccinator muscle subsequent to removal of third molars.

Low-power view of a tumor of the tongue demonstrating a mass of mature adipose tissue.

High-power view of a tumor of the tongue showing the similarity of the tumor cells to normal fat.

What is the treatment and prognosis for lipoma?

Clinical Features

• smooth-surfaced, nonulcerated nodule
• occur by mental foramen, tongue, and lower lip
• most often in middle-aged adults
• slightly more common in women
• altered sensations range from anesthesia to pain

Histopathologic Features

• haphazard proliferation of nerve bundles
• fibrous connective tissue stroma
• mild chronic inflammatory cell infiltrate

Painful nodule of the mental nerve as it exits the mental foramen. Such lesions often arise subsequent to surgical procedures.

Note the irregular nodular proliferation along the mental nerve that is being exposed at the time of surgery.

Low-power view showing the haphazard arrangement of nerve bundles within the background fibrous connective tissue.

High-power view showing cross-sectioned nerve bundles within dense fibrous connective tissue.

What is the treatment and prognosis for traumatic neuroma?

Clinical Features

• striking predilection for the face (90%)
• most occur between fifth and seventh decades
• smooth-surfaced, painless, papule or nodule
• usually less than 1 cm
• occur on hard palate, gingiva, and labial mucosa

Histopathologic Features

• well circumscribed and often encapsulated
• interlacing fascicles of spindle cells
• nuclei are characteristically wavy and pointed
• no significant pleomorphism or mitotic activity
• positive reaction for S-100 protein

Small, painless nodule of the lateral hard palate. These lesions most commonly occur extraorally on the face.

1. Low-power view showing a well-circumscribed, nodular proliferation of neural tissue.
2. Higher-power view demonstrating spindle cells with wavy nuclei.

Immunohistochemical reaction demonstrating spindle-shaped cells that are strongly positive for S-100 protein.

What is the treatment and prognosis for palisaded encapsulated neuroma?

Clinical Features

• slow-growing, encapsulated tumor
• typically associated with a nerve trunk
• usually asymptomatic, but pain may occur
• most occur in young to middle-aged adults
• can reach several centimeters in size
• most common intraorally on the tongue

Histopathologic Features

• two microscopic patterns
• fascicles of spindle-shaped Schwann cells ("Antoni A")
• randomly arranged spindle-shaped cells ("Antoni B")
• central acellular, eosinophilic areas ("Verocay bodies")
• diffuse, positive reaction for S-100 protein

Nodular mass in the floor of the mouth. The most common intraoral site for these lesions is on the tongue.

1. Low-power view showing well-organized Antoni A tissue (right) with adjacent myxoid and less organized Antoni B tissue (left)
2. The Schwann cells of the Antoni A tissue form a palisaded arrangement around acellular zones known as Verocay bodies.

What hereditary condition, caused by mutation of a tumor suppressor gene on chromosome 22, is characterized by bilateral schwannomas of the auditory-vestibular nerve?

NF2 is an autosomal dominant condition caused by a mutation of a tumor suppressor gene (NF2) on chromosome 22. What protein is encoded by this gene?

What genetic disorder, related to mutation of the SMARCB1 gene on chromosome 22, is characterized by multiple schwannomas without involvement of the auditory-vestibular nerve?

What is the treatment and prognosis for schwannoma?

What is the most common type of peripheral nerve neoplasm?

Clinical Features

• can arise in isolation or as a component of NF
• slow-growing, soft, painless lesions
• vary in size from small nodules to larger masses
• most frequently occur on the skin
• occur intraorally on tongue and buccal mucosa
• can also arise centrally within bone

Histopathologic Features

• interlacing bundles of spindle-shaped cells
• often exhibit wavy nuclei
• delicate collagen bundles
• variable amounts of myxoid matrix
• numerous mast cells (helpful diagnostic feature)
• scattered, positive reaction for S-100 protein

Smooth-surfaced, nodular mass of the maxillary gingiva and alveolar mucosa. These lesions occur more commonly on the tongue and buccal mucosa.

Intraosseous tumor filling the right mandibular ramus. These lesions may present as well-demarcated or poorly defined unilocular or multilocular radiolucencies.

Low-power view showing a cellular tumor mass below the epithelial surface. Immunostaining would reveal scattered, positive reaction for S-100 protein.

High-power view showing spindle-shaped cells with wavy nuclei. Immunostaining would reveal scattered, positive reaction for S-100 protein.

What is the treatment and prognosis for neurofibroma?

What hereditary condition, caused mutations of a tumor suppressor gene on chromosome 17, is associated with development of multiple neurofibromas and various other skin lesions?

NF1 is an autosomal dominant condition caused by a mutation of a tumor suppressor gene (NF1) on chromosome 17. What protein is encoded by this gene

Clinical Features

• multiple neurofibromas, most commonly on the skin
• massive baggy masses (elephantiasis neuromatosa)
• yellow-tan to dark-brown "café au lait" macules
• freckling of the axilla ("Crowe sign")
• brown-pigmented spots on the iris ("Lisch nodules")
• most common medical problem is hypertension
• most common oral finding is enlarged fungiform papillae

Both NF1 and polyostotic fibrous dysplasia are associated with the formation of "café au lait" pigmentation on the skin. How are they distinguished from one another?

NF1 can be diagnosed if the patient meets at least two of the diagnostic criteria. What are the seven diagnostic criteria for NF1?

Multiple tumors of the trunk and arms. These often appear during puberty and may continue to develop throughout adulthood.

Baggy, pendulous neurofibroma of the lower neck.

A dark-brown macule of the skin. These lesions may vary in diameter from 1 to 2 mm to several centimeters.

Diffuse neurofibroma resulting in unilateral enlargement of the tongue. The most finding is enlargement of fungiform papillae.

What is the treatment and prognosis for neurofibromatosis type I?

Tumor of the left cheek in a patient with type I neurofibromatosis. These most commonly occur on the trunk and extremities.

Tumor of the mandibular buccal vestibule in a patient with type I neurofibromatosis. The patient eventually died of this tumor.

High-power view of an intraoral tumor that developed in a patient with neurofibromatosis type I. There is a cellular spindle cell proliferation with numerous mitotic figures.

What group of rare autosomal dominant conditions are characterized by tumors or hyperplasias of the neuroendocrine tissues?

Clinical Features

• marfanoid body build
• everted upper eyelids
• oral mucosal neuromas (usually the first sign)
• pheochromocytoma (50%)
• medullary thyroid carcinoma (MTC)

Laboratory Values

• elevated urinary calcitonin
• elevated urinary vanillylmandelic acid

Histopathologic Features

• marked hyperplasia of nerve bundle
• normal or loose connective tissue background
• prominent thickening of the perineurium

Note the narrow face and eversion of the upper eyelids, which occurs due to thickening of the tarsal plate.

Multiple neuromas along the anterior margin of the tongue and bilaterally at the commissures (highly characteristic).

Low-power view of an oral mucosal neuroma showing marked hyperplasia of nerve bundles.

High-power view of an oral mucosal neuroma. Note the prominent thickening of the perineurium.

What is the treatment and prognosis for multiple endocrine neoplasia (MEN) type 2B?

Clinical Features

• develops in young children (first year of life)
• predilection for anterior maxilla (69%)
• rapidly expanding blue or black mass
• often destroys the underlying bone
• displacement of the developing teeth

Laboratory Values

• elevated urinary vanillylmandelic acid

Histopathologic Features

• biphasic population of cells in a collagenous stroma
• epithelioid cells with dark-brown pigment
• neuroblastic cells inwith hyperchromatic nuclei

Infant with an expansile mass of the anterior maxilla. These lesions are almost always diagnosed during the first year of life.

Radiolucent destruction of the anterior maxilla associated with displacement of the developing teeth

Low-power view showing nests of epithelioid cells containing granules of dark-brown melanin pigment within a fibrous stroma.

High-power view of a tumor nest demonstrating two cell types: 1) small, hyperchromatic round cells and 2) larger epithelioid cells with vesicular nuclei. Some stippled melanin pigment is also present.

What is the treatment and prognosis for melanotic neuroectodermal tumor of infancy?

Clinical Features

• most common in head and neck area
• usually occur in middle-aged adults (41 to 47 years)
• most common type is in the carotid body
• slowly enlarging, painless mass

Histopathologic Features

• round or polygonal epithelioid cells
• organized into nests or "zellballen"
• nests consist primarily of chief cells
• positive for neuroendocrine markers

Large tumor in the left neck producing a visible external swelling. This is seen more frequently in patients who live at high altitudes.

An magnetic resonance image (MRI) showing a tumor mass at the carotid bifurcation. Arrows indicate the external and internal branches of the carotid artery.

Tumor cells organized into zellballen consisting primarily of chief cells with centrally located, vesicular nuclei and somewhat granular, eosinophilic cytoplasm.

What is the treatment and prognosis for paraganglioma?

Clinical Features

• most occur on tongue (50%), especially dorsal surface
• occurs in fourth to sixth decades of life
• more common in females (2 : 1)
• asymptomatic sessile nodule
• usually 2 cm or less
• typically pink, but sometimes yellow
• usually solitary, although multiple may occur

Histopathologic Features

• large, polygonal cells, usually arranged in sheets
• abundant pale eosinophilic, granular cytoplasm
• dark or vesicular nuclei
• indistinct cell borders ("syncytial" appearance)
• not encapsulated, intermingles with adjacent tissues
• acanthosis or pseudoepitheliomatous hyperplasia (50%)

Submucosal nodule on the dorsum of the tongue. The buccal mucosa is the second most common intraoral location for this lesion.

Nodular mass of the buccal mucosa near the commissure. The dorsum of the tongue is the most common location for this lesion.

Medium-high–power view showing polygonal cells with abundant granular cytoplasm and vesicular nuclei.

Marked pseudoepitheliomatous hyperplasia overlying a tumor. Such cases may easily be mistaken for squamous cell carcinoma.

What is the treatment and prognosis for granular cell tumor?

Clinical Features

• occur almost exclusively on alveolar ridges of newborns
• pink-to-red, smooth-surfaced, polypoid mass
• most are 2 cm or less in size
• more common on the maxillary ridge
• most occur lateral to the midline, in incisor-canine area
• striking predilection for females (90%)

Histopathologic Features

• large, rounded cells
• abundant granular, eosinophilic cytoplasm
• round to oval, lightly basophilic nuclei
• atrophy of the rete ridges

Polypoid mass of the anterior maxillary alveolar ridge in a newborn. These occur almost exclusively on alveolar ridges of newborns.

Low-power photomicrograph showing a nodular tumor mass. Note the atrophy of the rete ridges.

High-power view of rounded cells with abundant granular, eosinophilic cytoplasm and round to oval, lightly basophilic nuclei.

What is the treatment and prognosis for congenital epulis?

1. Nodular mass on the maxillary alveolar ridge. Instead of being excised, the lesion was monitored clinically.
2. Clinical appearance of the child at 1 year of age. The mass has disappeared without treatment.

Clinical Features

• most common tumors of infancy (4% to 5% of 1-year-olds)
• more common in females (3 : 1 to 5 : 1)
• occur more frequently in whites
• most occur on the head and neck (60%)
• rarely present at birth, but develop rapidly in first weeks
• appear raised and bosselated with bright-red color
• firm and rubbery to palpation
• most common problem is ulceration

Histopathologic Features

• numerous plump endothelial cells
• often-indistinct vascular lumina
• endothelial cells become more flattened
• small, vascular spaces become more evident
• spaces replaced by fibrofatty connective tissue
• positive for GLUT1

Infant with two red, “strawberry” nodular masses on the posterior scalp and neck. These are the most common tumors of infancy.

Low-power photomicrograph showing a circumscribed cellular mass of vascular endothelial cells arranged in lobular aggregates.

High-power view showing a highly cellular endothelial proliferation forming occasional indistinct vascular lumina.

High-power photomicrograph demonstrating well-formed capillary-sized vessels. As this lesion progresses, the vascular spaces become less prominent and are replaced by fibrofatty connective tissue.

Large, segmental cervicofacial hemangioma can be a component of PHACE(S) syndrome. What does this acronym stand for?

What coagulopathy associated with vascular tumors, characterized by severe thrombocytopenia and hemorrhage because of platelet trapping within the tumor, has a mortality rate as high as 30%?

What is the treatment and prognosis for hemangioma?

Clinical Features

• present at birth and persist throughout life
• blue and are easily compressible
• grow proportionately with the patient

Histopathologic Features

• do not show active endothelial cell proliferation
• may show more dilated vessels
• many incorrectly categorized as hemangiomas

Blue-purple mass of the anterior tongue. In contrast to hemangiomas, these lesions present at birth and persist throughout life.

Well-circumscribed radiolucency that contains fine trabeculations. A bruit or pulsation may be apparent on auscultation and palpation.

Occlusal radiograph demonstrating cortical destruction and a “sunburst” periosteal reaction resembling osteosarcoma.

1. Periapical radiograph showing an expansile, mottled radiolucency in the mandibular incisor region.
2. Angiogram demonstrating a vascular proliferation between the mandibular incisors.

Low-power photomicrograph showing multiple large, dilated blood vessels. Because of their similar features, these lesions are often incorrectly categorized as hemangiomas.

What is the treatment and prognosis for vascular malformation?

Clinical Features

• vascular malformation of the face ("port wine stain")
• unilateral distribution along trigeminal nerve
• brain imaging may reveal “tramline” calcifications
• hypervascular changes to ipsilateral mucosa

Histopathologic Features

• excessive numbers of dilated blood vessels
• proliferative gingival lesions

Deep purple lesion of the malar area. Unless the lesion includes the region innervated by the ophthalmic branch of the trigeminal nerve, usually the patient does not have CNS involvement.

Port wine stain of the left face, including involvement along the ophthalmic branch of the trigeminal nerve.

Skull film showing “tramline” calcifications.

Unilateral vascular involvement of the soft palate.

What is the treatment and prognosis for Sturge-Weber syndrome?

Clinical Features

• occur almost exclusively in males
• predilection for adolescents (10 to 17 years)
• nasal obstruction and epistaxis
• anterior bowing of posterior wall of maxillary sinus

Histopathologic Features

• dense fibrous connective tissue
• numerous dilated, thin-walled blood vessels

A contrasted computed tomography (CT) scan showing a tumor of the nasopharynx and pterygopalatine fossa, with characteristic anterior bowing of the posterior wall of the right maxillary sinus.

Moderately cellular fibrous connective tissue with prominent blood vessels.

What is the treatment and prognosis for nasopharyngeal angiofibroma?

Clinical Features

• predilection for the head and neck (50% to 75%)
• 50% noted at birth, 90% by age of 2 years
• occur orally on anterior two thirds of the tongue
• pebbly surface resembling translucent vesicles

Histopathologic Features

• dilatated lymphatic vessels (microcystic type)
• macroscopic cystlike structures (macrocystic type)

Young boy with a cystic hygroma primarily involving the right side of the face.

Pebbly, vesicle-like appearance of a tumor of the right lateral tongue. These lesions have been likened to the appearance of frog eggs.

Dorsal tongue lesion demonstrating a purple color, which can be caused by secondary hemorrhage or an associated hemangiomatous component.

Lesion of the tongue showing dilated lymphatic vessels beneath the epithelium and in the deeper connective tissues.

High-power photomicrograph showing dilated, lymph-filled vessels immediately below the atrophic surface epithelium.

Lesion from the neck showing markedly dilated lymphatic vessels. The vessels often diffusely infiltrate the adjacent soft tissues.

What is the treatment and prognosis for lymphatic malformation?

Clinical Features

• slow-growing, firm, mucosal nodule
• most are asymptomatic, but some are painful
• normal in color, or may have a bluish hue
• most common on lips, tongue, palate, and cheek (80%)

Histopathologic Features

• well-circumscribed
• bundles of spindle-shaped smooth muscle cells
• nuclei are elongated, pale staining, and blunt ended
• stains bright red with Masson trichrome stain

Small, pink-red nodule on the posterior hard palate lateral to the midline. A tissue bispsy of this lesion would stain bright red with Masson trichrome stain.

Low-power view showing a well-circumscribed cellular mass of spindle-shaped smooth muscle cells.

High-power view showing spindle-shaped cells with blunt-ended nuclei. Immunohistochemical analysis shows strong positivity for smooth muscle actin.

Well-circumscribed tumor exhibiting prominent blood vessels surrounded by smooth muscle.

Masson trichrome stain demonstrating bundles of smooth muscle (red) with adjacent normal collagen (blue).

What is the treatment and prognosis for leiomyoma?

Clinical Features

• occur in middle-aged and older patients
• more common in men (75%)
• most located on pharynx, oral cavity, and larynx
• occur intraorally on floor, soft palate, and tongue
• nodule or mass can grow to many centimeters in size

Histopathologic Features

• well-circumscribed lobules
• large, polygonal cells
• abundant granular, eosinophilic cytoplasm
• peripheral vacuolization (“spider web” appearance)
• positive for myoglobin, desmin, muscle-specific actin

Nodular mass in the left cheek. Laryngeal and pharyngeal lesions of this type often lead to airway obstruction.

Computed tomography (CT) scan of a tumor. These lesions can grow many centimeters before discovery.

Medium-power view showing a uniform tumor composed of rounded and polygonal cells with focal vacuolization.

Phosphotungstic acid hematoxylin (PTAH) stain demonstrates focal cross striations in some cells of this lesion.

What is the treatment and prognosis for rhabdomyoma?

Clinical Features

• occur on the tongue (85%) near the foramen cecum
• firm, smooth-surfaced, sessile or pedunculated nodule
• between 0.5 and 2.0 cm in diameter

Histopathologic Features

• well-circumscribed mass
• dense lamellar bone or mature cartilage
• surrounded by dense fibrous connective tissue

Hard pedunculated nodule on the posterior dorsum of the tongue. Microscopic examination showed a mass of dense lamellar bone.

Mass of dense lamellar bone surrounded by dense fibrous connective tissue beneath the surface epithelium.

What is the treatment and prognosis for osseous or cartilaginous choristomas?

Clinical Features

• slow-growing masses
• may reach considerable size before they produce pain
• can develop at any age
• can occur anywhere in the head and neck region

Histopathologic Features

• spindle-shaped cells in a “herringbone” pattern
• little variation in size and shape
• variable numbers of mitotic figures

Child with a large mass of the hard palate and maxillary alveolar ridge. These lesions can develop at any age and occur anywhere in the head and neck region.

Cellular mass of spindle-shaped cells in a “herringbone” pattern demonstrating mild pleomorphism.

What is the treatment and prognosis for fibrosarcoma?

Clinical Features

• occur primarily in older age groups
• expanding mass, may be painful or ulcerated
• most occur in deep tissues of trunk and extremities

Histopathologic Features

• plump spindle cells in a storiform pattern
• admixed areas of pleomorphic giant cells

Spindle cell neoplasm demonstrating marked pleomorphism and scattered mitoses.

What is the treatment and prognosis for undifferentiated pleomorphic sarcoma?

Clinical Features

• most common soft tissue sarcoma
• seen primarily in adults (mean age, 57 years)
• soft, slow-growing, ill-defined mass
• may appear normal in color or yellow
• most common in head and neck region
• occur intraorally on the tongue and cheek

Histopathologic Features

• scattered lipoblasts and atypical, hyperchromatic stromal cells (well-differentiated type)
• proliferating lipoblasts within a myxoid stroma that contains a rich capillary network (myxoid type)
• aggressive form of myxoid type with less differentiated, rounded cells (round cell type)
• extreme cellular pleomorphism and bizarre giant cells (pleomorphic type)
• well-differentiated type with poorly differentiated changes (dedifferentiated type)

High-power view showing vacuolated lipoblasts with pleomorphic nuclei.

What is the treatment and prognosis for liposarcoma?

Clinical Features

• most common in young adults
• associated with neurofibromatosis
• enlarging mass, sometimes rapidily growing
• commonly cause pain or nerve deficits
• occur on mandible, lips, and buccal mucosa

Histopathologic Features

• fascicles of atypical spindle-shaped cells
• cells irregular in shape with wavy nuclei
• heterologous elements found in some tumors
• positive for S-100 protein (50%)

What is the treatment and prognosis for malignant peripheral nerve sheath tumor?

Clinical Features

• more common in adults (45 to 56 years)
• arises close to the cribriform plate
• may extend into adjacent paranasal sinuses
• nasal obstruction, epistaxis, pain, and anosmia

Histopathologic Features

• small basophilic cells in sheets and lobules
• rosette and pseudorosette formation
• delicate neurofibrillary material

A T1-weighted sagittal magnetic resonance image (MRI) showing a tumor filling the superior nasal cavity and ethmoid sinus, with extension into the anterior cranial fossa.

Sheet of small, basophilic cells adjacent to the sinonasal epithelium (top). Areas of delicate neurofibrillary material may be seen.

What is the treatment and prognosis for olfactory neuroblastoma?

Clinical Features

• most common in older adult patients
• early lesions often resemble bruises
• enlarge to form elevated, nodular, or ulcerated surface
• occur orally on tongue and mandible

Histopathologic Features

• infiltrative proliferation of endothelium-lined blood vessels
• endothelial cells appear hyperchromatic and atypical
• positive for CD31 and factor VIII–related antigen

Slightly elevated, bluish purple lesion on the scalp. This rare malignancy of vascular endothelium may arise from either blood or lymphatic vessels.

Sinusoidal vascular spaces lined by pleomorphic endothelial cells. Immunohistochemical studies show the tumor cells to be positive for CD31 and factor VIII–related antigen.

What is the treatment and prognosis for angiosarcoma?

Clinical Features

• caused by infection with HHV-8
• primarily a disease of late adult life
• most cases occur in men (70% to 95%)
• affects Italian, Jewish, or Slavic ancestry
• blue-purple macules and plaques
• occur on skin of lower extremities

Histopathologic Features

• proliferation of miniature vessels (patch stage)
• development of spindle cells (plaque stage)
• formation of tumorlike mass (nodular stage)

Multiple purple macules and plaques on the lower leg of an older man infected with HHV-8.

Low-power photomicrograph showing a cellular spindle cell tumor within the connective tissue. This patient is infected with HHV-8.

High-power photomicrograph showing spindle cells and poorly defined vascular slits. This patient is infected with HHV-8.

What is the treatment and prognosis for Kaposi sarcoma?

Clinical Features

• most common in middle-aged and older adults
• half of all oral cases occur in the jawbones
• nonspecific enlarging mass, may be painful

Histopathologic Features

• fascicles of pleomorphic spindle-shaped cells
• abundant eosinophilic cytoplasm
• blunt-ended, cigar-shaped nuclei
• glycogen-positive periodic acid-Schiff (PAS) stain
• bright red with a Masson trichrome stain

Ulcerated mass of the anterior maxillary alveolar ridge. These lesions are clinically non-specific, but immunohistochemical analysis usually reveals the presence of one or more of myogenic markers.

What is the treatment and prognosis for leiomyosarcoma?

Clinical Features

• occurs during the first decade of life
• approximately 60% occur in males
• painless, infiltrative mass that may grow rapidly
• face and orbit are most frequent locations
• palate is the most frequent intraoral site

Histopathologic Features

• small round or oval cells
• hyperchromatic nuclei
• indistinct cytoplasm
• strap-shaped rhabdomyoblasts

Young child with a mass of the right maxilla. The tumor is most often a painless, infiltrative mass that may grow rapidly.

Computed tomography (CT) scan showing expansile lytic lesion of the maxilla characterized by skeletal muscle differentiation.

Medium-power view showing a sheet of small, round cells with hyperchromatic nuclei. Better-differentiated lesions show round to ovoid rhabdomyoblasts with distinctly eosinophilic cytoplasm.

This tumor shows strap-shaped rhabdomyoblasts that are positive for desmin with immunohistochemical staining. Note the cross striations.

What is the treatment and prognosis for rhabdomyosarcoma?

Clinical Features

• occur in teenagers and young adults
• slight male predilection
• gradually enlarging mass, often painful
• occurs primarily near large joints and bursae
• occur orally in the tongue and cheek

Histopathologic Features

• biphasic tumor of spindle and epithelial cells
• spindle cells predominate
• epithelial cells that surround glandlike spaces
• calcifications may be seen (30%)

Biphasic tumor consisting of spindle cells intermixed with cuboidal to columnar epithelial cells that line glandlike spaces.

What is the treatment and prognosis of synovial sarcoma?

Clinical Features

• slow-growing, painless mass
• most common in young adults and children
• lower extremity is the most frequent location
• can occur orally on the tongue
• female predilection in first two decades (2 : 1)
• more common in men after age 30 years

Histopathologic Features

• polygonal cells arranged around alveolar spaces
• abundant granular, eosinophilic cytoplasm
• one to several vesicular nuclei
• PAS-positive, diastase-resistant crystals
• strong positivity for TFE3 transcription factor

Alveolar collections of large, polygonal cells containing abundant granular cytoplasm. Special stains will reveal PAS-positive, diastase-resistant crystals that are highly characteristic for this tumor.

What is the treatment and prognosis for alveolar soft-part sarcoma?

Clinical Features

• most occur on gingiva (54%) and tongue (22.5%)
• nodular mass often resembles hyperplastic growth
• occasionally appears as a surface ulceration
• destruction of underlying bone may loosen teeth
• more common in males
• most frequent in middle-aged and older adults

Histopathologic Features

• resemble tumor of origin
• most cases represent carcinomas

Pigmented nodule of the mandibular gingiva.

Nodular mass of the left lateral border of the tongue.

1. Focal swelling of the left retromolar pad area.
2. Same patient 4 weeks later. Note the marked enlargement of the lesion.

Aggregates of malignant epithelial cells below the surface epithelium.

What is the treatment and prognosis for metastatic oral tumors?