Chapter 12: Soft Tissue Tumors Flashcards
what is the treatment of a palisaded encaspulated neuroma?
surgical excision
are most melanotic neuroectodermal tumors of infancy benign or malignant?
benign
inflammatory papillary hyperplasia is a condition related to what 3 things?
- ill-fitting denture
- poor denture hygiene
- wearing a denture 24 hours/day
what is the appearance of peripheral ossifying fibroma?
nodular mass, usually originating from the dental papilla
inflammatory papillary hyperplasia
___% of peripheral ossifying fibromas recur. why?
15%, usually because the base of the lesion was not removed
giant cell fibroma
multiple endocrine neoplasia type 2B
notice the multiple mucosal neromas on the tongue and the bilateral neuromas of the commissural mucosa
palisaded encapsulated neuroma
___ of neurofibromatosis type I is pathognomonic
plexiform variant
feels like a bag of worms
sturge-weber angiomatosis
ipsilateral hypervascular changes
are pyogenic granulomas more common in males or females?
- females
- frequently occurs in pregnant women, most commonly in the 1st trimester
- some regress after pregnancy
leptomeningeal angiomas are typically associated with a ___, and may result in mental retardation or ___
- convulsive disorder
- contralateral hemiplagia (one-sided paralysis)
lipomas are the most common ___ neoplasm
mesenchymal
more common on the trunk
is the mechanism by which tumors can spread to the oral cavity well understood?
no
peripheral giant cell granuloma
notice the bluish color
what are the two types of rhabdomyomas?
- adult
- 70% in adult men
- nodular mass most commonly occurring int he pharynx and floor of mouth
- fetal
- young male patients
- predilection for the face
endemic (african) kaposi’s sarcoma has 4 subtypes. what are they?
- benign nodular (similar to classic)
- aggressive
- florid
- lymphadenopathic (children)
what are the diagnostic criteria for neurofibromatosis type I and how many do you need for a positive diagnosis?
need 2 or more for a positive diagnosis
- six or more cafe au lait macules
- “coast of california”
- two or more neurofibromas or one plexiform neurofibroma
- freckling in the axillary region (crowe’s sign)
- optic glioma
- two or more iris hamartomas (lisch nodules)
- osseous lesion such as sphenoid dysplasia
- first degree relative with neurofibromatosis type I
what is the treatment for fibromas?
conservative surgical exision
port wine stain aka nevus flammeus characteristic of sturge-weber angiomatosis
describe the classic type of kaposi’s sarcoma
- 80% in older men
- italian, jewish, or slavic descent
- multiple, asymptomatic blue-purple macules on the lower extremities which grow into nodules
___ is reactive hyperplasia of fibrous connective tissue in response to local irritation or trauma
fibroma
what is the treatment of peripheral ossifying fibroma?
excision down to periosteum and scale adjacent teeth
what is the clinical presentation of lymphangiomas?
frog eggs or tapioca pudding
granular cell tumor
pyogenic granulomas may exhibit ___ growth
rapid
50-75% of lymphangiomas are in what location?
head and neck
what is the treatment of lipomas?
surgical excision
___ is a reactive tissue growth that develops under a denture
inflammatory papillary hyperplasia
patients with sturge-weber angiomatosis have what two characteristic features?
- port wine stain (aka nevus flammeus)
- leptomeningeal angiomas
congenital epulis’ are 3x more common in the maxillary or mandibular alveolar ridge? what specific location is most frequent?
- maxillary
- most frequently where the developing lateral incisor-canine area
neurofibroma
what is the treatment for a granular cell tumor?
conservative excision is typically curative
___ is a benign tumor of lymphatic vessels
lymphangioma
primary malignancies from adjacent tissues may spread via ___
lymphatics
leptomeningeal angiomas overly the ipsilateral or contralateral cerebral cortex?
ipsilateral
describe a possible explanation of head and neck metastases in the absence of lung metastases
batson’s plexus
- this is a valveless vertebral venous plexus that might allow retrograde spread of tumor cells and bypass the lungs
what is the most common location of neurofibromas? what about intraoral sites?
- skin is the most common overall
- intraoral lesions are common on the tongue and buccal mucosa
most patients with inflammatory papillary hyperplasia also have ___
a candidal infection
___ neuromas of the ___ mucosa are highly characteristic of multiple endocrine neoplasia type 2B
bilateral neuromas of the commissural mucosa
melanotic neuroectodermal tumor of infancy
how many recognized forms of neurofibromatosis are there, and what is the most common form?
- 8 forms
- neurofibromatosis type I
are schwannomas typically symptomatic or asymptomatic?
asymptomatic
inflammatory papillary hyperplasia typically occurs where?
on the hard palate
what is the average age of someone with a palisaded encapsulated neuroma?
50yo
retrocuspid papilla
___ is the most common type of peripheral nerve neoplasm
neurofibroma
what is the treatment for inflammatory fibrous hyperplasia?
surgical removal and remake the denture
rhabdomyosarcomas make up ___% of soft tissue sarcomas of childhood, and ___% are found in the head and neck
- 60%
- 35%
what are the histologic features of a schwannoma?
- antoni A - streaming fascicles of spindle-shaped schwann cells which form a palisaded arrangement around central acellular, eosinophilic areas known as verocay bodies
- antoni B - basically everything that’s not antoni A
what is the treatment for rhabdomyomas?
excision is curative
describe traumatic neuromas
- not a true neoplasm; it is a proliferation of neural tissue after nerve injury
- most common in mental foramen area
- 1/3 are painful
- surgical removal is typically curative
intraoral involvement of sturge-weber angiomatosis is common and results in ___. this lesion may ___ when manipulated
- hypervascular changes to the ipsilateral mucosa
- hemmorhage
do all patients with a port wine stain have sturge-weber angiomatosis?
no
what is the presentation of pyogenic granulomas?
- smooth or lobulated mass that is typically pedunculated
- surface is characteristically ulcerated and lesion bleeds easily
- young lesions appear red; older lesions are more collagenized and pink
peripheral ossifying fibroma
in histology photos, what would you see that would indicate a peripheral ossifying fibroma?
bone
50% of lipomas are in what location?
buccal mucosa
giant cell fibromas typically have a ___ surface
papillary
what is another name for a schwannoma?
neurilemoma
inflammatory papillary hyperplasia
what is a retrocuspid papilla?
- it is microscopically similar to a giant cell fibroma, located lingual to the mandibular cuspid
- typically bilateral, present in up to 99% of children
- normal anatomic variation that regresses with age
what is the prognosis of metastases to oral soft tissues?
poor
neurofibroma
what is typically the first sign of multiple endocrine neoplasia type 2B?
oral mucosal neuromas, present as soft, painless papules or nodules
what population are hemangiomas more common?
- more common in females (3:1, 5:1)
- more common in caucasians vs other racial groups
what are the 4 clinical presentations of kaposi’s sarcoma?
- classic
- endemic (african)
- iatrogenic immunosuppression associated
- AIDS related
inflammatory papillary hyperplasia may show improvement after ___ therapy
antifungal
giant cell fibroma
floats in formalin
lipoma
___ is a rare, nonhereditary developmental condition caused by the persistence of a vascular plexus around the cephalic portion of the neural tube
sturge-weber angiomatosis
what age patient are lipomas most common?
>40
rhabdomyosarcoma
up to half of schwannoma cases occur in what location?
head and neck
what are the syptoms of neurofibromatosis type II?
deafness, dizziness, tinnitus
what is the treatment of neurofibromatosis type I?
directed toward prevention or management of complications
___ occurs almost exclusively on the alveolar ridges of newborns and appears as a mucosal-colored, smooth-surfaced, polypoid mass typically less than 2cm
congenital epulis
neurofibromas can arise as ___ tumors or be a component of ___
- solitary
- neurofibromatosis
what is the prognosis of classic kaposi’s sarcoma?
90% survival
how do rhabdomyosarcomas present?
painless, infiltrative mass that grows rapidly
how should pyogenic granulomas that develop during pregnancy be treated?
treatment should be deferred unless functional or esthetic problems develop
what is the treatment of neurofibromas?
surgical excision
what population with granular cell tumors might experience multiple tumors?
african americans
pyogenic granuloma
___ is a benign neural neoplasm of schwann cell origin; it is a slow-growing, encapsulated tumor which arises in associated with a nerve trunk
schwannoma
palisaded encapsulated neuromas are benign neural tumors that are typically found in what location?
on the face (90%)
what do lesions look like that have metastasized to the oral soft tissues?
nodular mass that resembles a hyperplastic growth, such as a pyogenic granuloma
sturge-weber angiomatosis has a _(unilateral/bilateral)_ distribution along one or more segments of the ___ nerve
- unilateral
- trigeminal nerve
describe the iatrogenic type of kaposi’s sarcoma
- most common in organ transplant recipients
- 0.5% of renal transplant patients
what is the most common location of fibromas?
buccal mucosa along the bite line
granular cell tumors typically occur around what age? male to female ratio?
- age 40; rare in kids
- 2:1 F:M
what are the common locations of pyogenic granulomas?
most common on gingiva (75%) > lips, tongue, buccal mucosa
what is the treatment of rhabdomyosarcoma?
surgery and chemotherapy with radiation
what are two types of neuromas?
can be either traumatic neuroma or palisaded encapsulated neuroma
___ may produce a “cupping” resorption of the undelrying alveolar bone
peripheral giant cell granuloma
what is the treatment for pyogenic granulomas?
surgical excision
___ is a malignancy of skeletal muscles
rhabdomyosarcoma
are congenital epulis’ more common in males or females?
90% occur in females
___ typically appears as an asymptomatic, sessile, smooth-surfaced nodule that is similar in color to the surrounding mucosa
fibroma
lymphangioma
what is another name for a palisaded encapsulated neuroma?
solitary circumscribed neuroma
which type of lymphangioma is more commonly found in the mouth?
cavernous
what are the 3 types of lympangioma?
capillary, cavernous, cystic
what are the 3 oral manifestations of neurofibromatosis type I?
- nelargement of the fungiform papilla in 50%
- intraoral neurofibromas in 25%
- enlargement of the mandibular foramen or canal
what physical characteristics do patients with multiple endocrine neoplasia type 2B have?
- marfanoid build with thin, elongated limbs
- narrow face with characteristically thick and protuberant lips
___ is a benign tumor of fat
lipoma
50% of patients with multiple endocrine neoplasia type 2B develop ___, and 90% develop ___
- pheochromocytoma
- medullar carcinoma of the thyroid gland
pyogenic granuloma
lymphangioma
75% of leiomyoma oral cases are a ___ variant
vascular
fibroma
which type of hemangioma typically blanches, and is darker red to purple?
cavernous
___ is the most common “tumor” of the oral cavity
fibroma
50% of peripheral ossifying fibromas occur in the ___ region
incisor-cuspid region
what is the clinical color of melanotic neuroectodermal tumor of infancy?
black and/or blue
oral focal mucinosis is most common in what population?
young females
what is the average age patient that peripheral ossifying fibromas are most common in? male or female predilection?
- average age is 15yo (teenagers and young adults 10-19)
- 2/3 occur in females
hemangioma
most cases of metastases to oral soft tissues represent ___ rather than ___
carcinomas rather than sarcomas
what is the survival of someone with rhabdomyosarcoma?
ranges from 50-95% depending on histologic subtype
up to 5% of patients with neurofibromatosis type I have ___ tumors with an associated 5-year survival of ___%
- malignant peripheral nerve sheath tumors (MPSNT)
- 15%
lipoma
what is the treatment for more advanced inflammatory papillary hyperplasia lesions?
surgical removal
what age patient are schwannomas common in?
young and middle-aged adults
will a congenital epulis be S-100 positive or negative?
negative - no pseudopeitheliomatous hyperplasia (different from granular cell tumor)
melanotic neuroectodermal tumor of infancy
___ is extremely important in the management of neurofibromatosis type I
genetic counseling
inflammatory fibrous hyperplasia (epulis fissuratum)
what is the most common location of a schwannoma?
tongue
giant cell fibromas have a predilection for what location?
gingiva
treatment of kaposi’s sarcoma depends on what? what are the treatment types?
- type, stage, and location
- radiation, surgery, and chemotherapy are used
what does the tissue look like in inflammatory papillary hyperplasia?
asymptomatic, erythematous tissue with a pebbly or papillary surface
what is the most common location in the oral cavity of fibrous histiocytomas?
buccal mucosa
oral manifestations of neurofibromatosis type I occur in what percent of patients with NF1?
90%
floats in formalin
lipoma
fibrous histiocytoma
___% of patients who continuously wear their denture have inflammatory papillary hyperplasia
20%
peripheral ossifying fibromas occur exclusively on what location?
gingiva
granular cell tumor
oral focal mucinosis
which type of hemangioma may not blanch clinically, and is usually red in color?
capillary
what fraction of patients with neurofibromatosis type I have mild disease?
2/3
do pyogenic granuloma lesions recur?
occasionally
what is the treatment of a congenital epulis?
surgical excision is curative, even with incomplete removal
congenital epulis
neurofibromatosis is a ___ condition affecting 1 in ___ people
- hereditary
- 1/3000
is a pyogenic granuloma a true granuloma?
no
do giant cell fibromas occur at younger or older ages than other fibromas?
younger
leiomyomas are most common in what 3 locations?
uterus, GI tract, skin (smooth muscle from vasculature)
pyogenic granuloma
___ is the most common site for metastases to the oral soft tissues. __ is the second most common site.
- gingiva (50%)
- tongue (25%)
what are common locations of rhabdomyosarcomas? male or female predilection?
face and orbit
male predilection
what is the treatment of giant cell fibromas?
conservative surgical excision
neurofibromatosis type I
patients with sturge-weber angiomatosis that have involvement along the distribution of the ___ are at risk for the full condition
ophthalmic branch
___ is a reactive lesion caused by local irritation or trauma, that occurs exclusively on the gingiva or edentulous alveolar ridge
peripheral giant cell granuloma
what is the most common location of hemangiomas?
head and neck, which accounts for 60% of all cases
the histological diagnosis of tumor-like hyperplasia of fibrous connective tissue on association with the flange of an ill-fitting denture is ___, while the clinical diagnosis is ___
- inflammatory fibrous hyperplasia
- epulis fissuratum
___ is a benign neoplasm of skeletal muscle
rhabdomyoma
___ is the name for inflammatory fibrous hyperplasia in association with the flange of an ill-fitting denture
epulis fissuratum
___ is a rare pigmented tumor that usually occurs during the first year of life
melanotic neuroectodermal tumor of infancy
___ rhabdomyomas are very rare but have a predilection for the head and neck
extracardiac
in other words, cardiac rhabdomyomas are the most common
schwannoma
what is multiple endocrine neoplasia type 2B characterized by?
- parathyroid tumors
- pituitary tumors
- pancreatic tumors
- adrenal gland tumors (pheochromocytoma)
- thyroid tumors (medullary carcinoma)
- mucosal neuromas with a predilenction for the oral cavity
what location is oral focal mucinosis most common in? second most common?
- gingiva - 2/3
- hard palate - 1/3
fibroma
granular cell tumors exhibit significant ___; why is it significant?
- pseudoepitheliomatous hyperplasia
- if you submit a small dorsal tongue lesion for microscopic review and get back a reply of “squamous cell carcinoma” ask for a second opinion
soft tissue sarcomas account for ___% of cancers
<1%
lipomas are more common in what type of patient?
obese patients, but the metabolism is independent of normal body fat
peripheral ossifying fibroma
how do leiomyomas typically present?
asymptomatic, slow-growing, firm, mucosal nodule
neurofibromatosis type I is autosomal ___
dominant
___ is a true neoplasm exhibiting fibroblastic and histiocytic differentiation
fibrous histiocytoma
what are 2 other names for pyogenic granulomas?
pregnancy tumor or granuloma gravidarum
fibrous histiocytomas occur most commonly on what location?
the skin (termed a dermatofibroma)
neurofibromatosis type I is also termed ___
von recklinghausen’s disease of the skin
___% of peripheral giant cell granuloma lesions recur
10%
what is the treatment for leiomyomas?
excision is curative
do melanotic neuroectodermal tumor of infancy expand rapidly or slowly?
rapidly
describe the presentation of granular cell tumors
- asymptomatic, sessile nodule that is less than 2cm
- may be yellow or mucosal colored
should excised tissue from a fibroma be submitted for microscopic examination?
yes
what is the average age patient that peripheral giant cell granulomas are seen in? male or female predilection?
35yo, 60% occur in females
which type of lymphangioma is more common on the neck?
cystic lymphangioma (cystic hygroma)
fibroma
traumatic neuroma
what is the presentation of a peripheral giant cell granuloma?
appears as an erythematous mass similar to pyogenic granuloma, although peripheral giant cell granulomas are more often blue or purple compared to the bright red presentation of pyogenic granulomas
are soft tissue sarcomas common in the oral and maxillofacial region?
no
what is the most common site of granular cell tumors?
- tongue (up to 50%)
- dorsal surface is the most common site
oral lymphangiomas occur most frequently on the ___, where they often result in ___
- anterior 2/3 of the tongue
- macroglossia
inflammatory fibrous hyperplasia (epulis fissuratum)
what are the features of neurofibromatosis type II?
“acoustic neuromas” of vestibular nerve, meningiomas and ependymomas of the central nervous system
___ is diagnosed between the ages of 18-25 and has a marked propensity for metastasis, with an average age of death of 21
multiple endocrine neoplasia type 2B
___ are a characteristic feature of the neurofibromatosis type II
bilateral schwannomas of auditory-vestibular nerve
what is the treatment of retrocuspid papillas?
it should be recognized clinically, and no biopsy or treatment is needed
what is the most common tumor of infancy?
hemangioma
what is the prognosis of endemic kaposi’s sarcoma (other than benign nodular)?
poor prognosis
peripheral giant cell granuloma
notice the blueish color
what are the two types of hemangiomas?
capillary and cavernous
what is the treatment of oral focal mucinosis?
surgical excision
what may heal very early inflammatory papillary hyperplasia lesions?
removal of the denture
___ is a fibrous tumor with distinctive features and is not associated with chronic irritation
giant cell fibroma
pyogenic granuloma
fibroma
what is the S-100 stain? for a granular cell tumor, will the test be positive or negative?
tests for neural tissue and will be positive if it is a granular cell tumor
what is the prognosis of kaposi’s sarcoma in transplant patients?
survival somewhere between classic (90% survival) and endemic (poor prognosis)
what is a trick used to help diagnose a lipoma?
it will float in formalin
port wine stain aka nevus flammeus characteristic of sturge-weber angiomatosis
what is the differential for a nodule on the gingiva (4 things)?
- peripheral ossifying fibroma
- peripheral giant cell granuloma
- pyogenic granuloma
- inflammatory fibrous hyperplasia
fibrous histiocytomas appear as a ___
painless nodular mass
___ is a reactive lesion to local irritation or trauma (poor oral hygiene)
pyogenic granuloma
giant cell fibroma
oral focal mucinosis results from overproduction of ___
overproduction of hyaluronic acid by fibroblasts
for gingival pyogenic granuloma lesions, where should the excision extend to?
periosteum and adjacent teeth should be scaled
kaposi’s sarcoma is caused by what virus?
HHV-8
granular cell tumors are a ___ soft tissue neoplasm that shows a predilection for the ___
- benign
- oral cavity
inflammatory fibrous hyperplasia usually develops in what location?
facial aspect of the alveolar ridge
what is the treatment of peripheral giant cell granuloma?
local surgical excision down to underlying bone, scaling of adjacent teeth
what age patient are pyogenic granulomas most common?
children and young adults
___ is a tumor-like hyperplasia of fibrous connective tissue
inflammatory fibrous hyperplasia
metastases to the oral soft tissues from lower parts of the body are likely ___ and therefore would be expected to be found in the ___
- blood-borne
- lungs
what age patients do cutaneous fibrous histiocytomas commonly occur in? what about oral lesions?
- cutaneous - young patients
- oral lesions - older patients
patients with sturge-weber angiomatosis are born with a dermal capillary vascular malformation known as ___ or ___
port wine stain or nevus flammeus
what is the treatment for fibrous histiocytoma?
surgical excision
any patient with a lesion diagnosed as a neurofibroma must be evaluated for ___
neurofibromatosis
describe neurofibromatosis type II
- autosomal dominant
- mutation of a tumor suppressor gene on chromsome 22 (merlin)
melanotic neuroectodermal tumor of infancy has a striking predilection for what location?
anterior maxilla (61%)
half of all lymphangioma lesions are noted at what age?
at birth
90% develop by 2 years of age
oral focal mucinosis
congenital epulis
___ represents the oral counterpart of cutaneous focal mucinosis
oral focal mucinosis
inflammatory fibrous hyperplasia (epulis fissuratum)
___ are benign tumors of smooth muscle
leiomyoma
what is the presentation of lipomas?
appear as soft, smooth-surfaced nodular masses
lesion may be yellow or mucosal-colored
what are 6 types of soft tissue sarcomas?
- fibrosarcomas (head and neck)
- malignant fibrous histiocytoma (now termed undifferentiated pleomorphic sarcoma)
- liposarcoma (thigh, retroperitoneum)
- leiomyosarcoma (uterus, GI)
- angiosarcoma (scalp and forehead)
- malignant peripheral nerve sheath tumor (MPNST)
melanotic neuroectodermal tumor of infancy has characteristically high urinary levels of ___
vanillymandelic acid (VMA)
neurofibromas are most common in what what age patient? what is the presentation?
- young adults
- slow-growing, soft, painless lesions
