Benign Mesenchymal Tumors - part II Flashcards
lipoma
benign tumor of adipose tissue (fat)
T/F: lipoma occurs in children
false, adults
clinical features of lipoma
- slow-growing
- non-tender
- soft, doughy
- occasionally found intraorally
- yellow if close to the surface
where is lipoma commonly found?
in head and neck
histopathologic features of lipoma
demarcated or encapsulated collection of mature fat cells (adipocytes)
tx of lipoma
enucleation or conservative surgical excision
T/F: lipoma has little to no tendency to recur
true
T/F: lipoma has evidence of malignant transformation
false, no evidence
NERVE benign mesenchymal tumors
- traumatic neuroma
- neurilemoma (schwannoma)
- neurofibroma
- melanotic neuroectodermal tumor of infancy
- granular cell tumor
T/F: traumatic neuroma is NOT a true neoplasm
true
what causes a traumatic neuroma?
- uncommon rxn to sectioning of a nerve
2. reactive proliferation of n. tissue after damage of the n. bundle
what is the nerve in a traumatic neuroma trying to do?
attempts to regenerate and reestablish innervation of the distal segment; encounters scar tissue
clinical features of traumatic neuroma
- smooth-surfaced
- dome-shaped papule or nodule, usually <1 cm
- ± tender to palpation
approximately how many oral traumatic neuromas are painful?
only ~ 1/4 to 1/3 oral traumatic neuromas are painful
T/F: pain from oral traumatic neuromas can be intermittent or constant
true
T/F: pain from oral traumatic neuromas can be mild tenderness to burning severe radiating pain
true
common intraoral sites for traumatic neuroma
- tongue
2. buccal vestibule, especially mental foramen area
T/F: a traumatic neuroma on the buccal vestibule especially in the mental foramen area will be painful when impinged upon by a denture
true
histopathologic features of traumatic neuroma
tangled mass of peripheral nerve fibers in a collagenous background
tx for traumatic neuroma
surgical excision, including a small portion of the proximal nerve bundle
prognosis of traumatic neuroma
good
T/F: pain from a traumatic neuroma may persist or return at a later date even after being tx’d
true
T/F: recurrence of a traumatic neuroma is common
false, not common
neurilemoma (schwannoma)
benign tumor of schwann cell origin (surround axons of peripheral nerves)
who is affected by neurilemoma (schwannoma)?
most in adults, but any age
where does neurilemoma (schwannoma) occur?
occur anywhere in the body
clinical features of neurilemoma (schwannoma)
- slow-growing
- solitary
- encapsulated
- rubbery-firm
- non-tender
- may cause expansion
- may push on the nerve or other structures
- can occur centrally within bone
where are the most common intraoral locations for neurilemoma (schwannoma)?
- lips
- tongue
- buccal mucosa
how does neurilemoma (schwannoma) appear on radiographs?
radiolucent
T/F: neurilemoma (schwannoma) appears unilocular in radiographs
false, unilocular AND multilocular
histopathologic features of neurilemoma (schwannoma)
- well-developed capsule
- benign proliferation of spindle-shaped Schwann cells
- patterns seen microscopically
what are the two patterns seen microscopically of neurilemoma (schwannoma)?
- Antoni A
2. Antoni B
Antoni A
palisaded nuclei arranged around Verocay bodies
Verocay bodies
acellular zones made of reduplicated basement membrane and cytoplasmic processes
Antoni B
- less organized
2. sometimes myxoid
tx of neurilemoma (schwannoma)
- conservative excision
2. usually “shells out” due to dense CT capsule
T/F: neurilemoma (schwannoma) has no tendency to recur
true
T/F: neurilemoma (schwannoma) has an extremely rare malignant transformation
true
neurofibroma
benign tumor of neural fibroblast origin
T/F: over 90% of neurofibroma are solitary
true
what is the remaining 10% of neurofibroma?
remainder are multiple and associated with neurofibromatosis
T/F: neurofibroma is encapsulated
false, UNencapsulated
what is the most common location for a neurofibroma?
skin
clinical features of neurofibroma
- soft
- demarcated, dome-shaped papule or nodule, subcutaneous or submucosal
- non-tender
- can occur centrally within bone
T/F: oral neurofibroma mucosal lesions are not uncommon
true, they’re common
where would neurofibromas occur intraorally?
- tongue
2. buccal mucosa
how does a neurofibroma appear radiographically?
radiolucent
T/F: neurofibroma can appear multilocular on radiographs
false, can be unilocular or multilocular
histopathologic features of neurofibroma
- collection of spindle-shaped cells with wavy nuclei
- mast cells
- lesional tissue mingles with adjacent normal tissue
txx of neurofibroma
conservative excision
prognosis of neurofibroma
generally good, especially small, superficial lesions
T/F: it is uncommon for neurofibroma to transform to malignant peripheral nerve sheath tumor
true
T/F: neurofibromatosis is a common hereditary condition
true
T/F: neurofibromatosis has a variable number of neurofibromas, schwannomas
true
what are the 3 types of neurofibromatosis?
- Type 1
- Type 2
- Schwannomatosis
Type 1 (NF1) neurofibromatosis
mainly neurofibromas
what does Type I neurofibromatosis cause?
Von Recklinghausen disease of the skin (majority of neurofibromatosis cases)
Type 2 (NF2) neurofibromatosis
mainly schwannomas
Schwannomatosis neurofibromatosis
mainly schwannomas
T/F: type 1 neurofibromatosis is autosomal recessive
false, dominant
a parent with NF1 has what percent chance of having an affected child?
50%
what percent of new mutations “de novo” does the affected child with type 1 neurofibromatosis have?
~50%
T/F: type 1 neurofibromatosis has a low variable gene expression
false, highly variable (mild to severe)
what manifestatiosn does type 1 neurofibromatosis have?
- cutaneous
- oral
- ocular
cutaneous manifestations of type 1 neurofibromatosis
- Café-au-lait spots
- 6 or more, greater than 1.5 cm in adult
- multiple neurofibromas (small and discrete)
- plexiform neurofibroma
- pathognomonic for NF1
- axillary or inguinal freckling
Café-au-lait spots
light brown macules, smooth borders “coast of California”
what is a massive, pendulous neurofibroma in type 1 neurofibromatosis called?
plexiform neurofibroma
what does a plexiform neurofibroma feel like?
“a bag of worms”
where are oral manifestations of type 1 neurofibromatosis neurofibromas affect?
- tongue
- gingiva
- bone
T/F: enlargement fungiform papillae reported for type 1 neurofibromatosis but specificity for NF1 unknown
true
ocular manifestations of type 1 neurofibromatosis
Lisch nodules of the iris
Lisch nodules of the iris
clear yellow to brown aggregates of dendritic melanocytes within iris
diagnosis of type 1 neurofibromatosis require how many criteria?
2 or more
summary of diagnostic criteria for type 1 neurofibromatosis
- Six or more light brown spots on the skin (“café-au-lait” spots), measuring more
than 5 millimeters in diameter in children or more than 15 millimeters across in
adolescents and adults - Two or more neurofibromas, or one plexiform neurofibroma (a neurofibroma that
involves many nerves) - Freckling in the area of the armpit or the groin
- Two or more growths on the iris of the eye (known as Lisch nodules or iris
hamartomas) - A tumor on the optic nerve (called an optic nerve glioma)
- Abnormal development of the spine (scoliosis), the temple (sphenoid) bone of the
skull, or the tibia (one of the long bones of the shin) - A parent, sibling, or child with NF1
tx for NF1
- remove traumatized neurofibromas or disfiguring lesions
- genetic counseling
- follow up due to potential of malignant transformation
prognosis of NF1
fair to guarded
what happens to the prognosis of NF1 if malignant transformation occurs?
prognosis is poor (usually a malignant peripheral nerve sheath tumor)
T/F: melanotic neuroectodermal tumor of infancy
(MNTI) is common
false, rare
MNTI are of what origin?
neural crest
when does MNTI develop?
usually develops in the 1st year of life
where does MNTI occur?
anterior maxilla
what color is MNTI?
brown or black
T/F: MNTI grows slowly
false, rapidly
T/F: most MNTI are benign
true (7% act in a malignant fashion)
radiographic features of MNTI
- radiographic appearance is characteristic
- radiolucency anterior maxilla
- deciduous maxillary incisor pushed labially appearing as “tooth floating in space”
histopathologic features of MNTI
- proliferation of small, dark, neuroectodermal-appearing cells nested in aggregates
- surrounded by plump epithelioid cells with vesicular nuclei, which produce melanin
tx of MNTI
conservative excision usually curative
prognosis of MNTI
good
T/F: there has been sporadic reports of aggressive behavior of MNTI after tx
true
T/F: there should be clinical follow-up if MNTI is prudent
true
where does granular cell tumor affect?
any cutaneous surface
which site is the most common for granular cell tumor?
tongue
locations for granular cell tumor intraorally
- tongue
- buccal mucosa
- floor of mouth
clinical features of granular cell tumor
- slow-growing
- demarcated, subcutaneous or submuscosal
- non-tender
- most <1 cm
histopathologic features of granular cell tumor
- collection of mesenchymal cells with granular-appearing cytoplasm
- pseudoepitheliomatous hyperplasia (PEH) present
what stain is used for neural differentiation in granular cell tumor?
S-100(+)
why is granular cell tumor often mistaken for SCC histopathologically?
pseudoepitheliomatous hyperplasia (PEH) in granular cell tumor
tx of granular cell tumor
conservative excision usually curative
prognosis of granular cell tumor
excellent
what causes congenital epulis?
undermined histogenesis
when and where are congenital epulis found?
found at birth on maxillary ridge
congenital GCT
congenital epulis on tongue (rare)
who is affected by congenital epulis?
females
where do congenital epulis appear in relation to midline?
lateral to midline
clinical features of congenital epulis
- smooth surface
- often pedunculated
- vary in size
T/F: congenital epulis are common
false, rare
histopathologic features of congenital epulis
- proliferation of cells with granular cytoplasm
- atrophy of rete ridges
- no PEH
- S-100(-); does not stain for neural differentiation with IHC
tx for congenital epulis
- conservative excision
2. some reports of spontaneous resolution without surgery
prognosis of congenital epulis
excellent
T/F: congenital epulis have a tendency to recur
false, no tendency to recur
blood benign mesencymal tumors
- hemangioma
2. vascular malformations
vascular malformations
- Port wine stain
2. encephalotrigeminal angiomatosis
T/F: hemangioma may be a vascular hamartoma rather than a neoplasm
true
hamartoma
overgrowth of tissue normally present in the site
hemangioma
benign tumor of infancy
what is the most common tumor of childhood?
hemangioma
T/F: most hemangioma are recognized at birth
false, most are NOT recognized at birth
when do hemangioma arise?
by 8 weeks
what is the except of a hemangioma arising by 8 weeks of life?
non-involuting congenital hemangioma
non-involuting congenital hemangioma
- fully developed at birth
2. does not involute
which site is frequently affected by hemangioma?
head and neck
what is the most common intraoral site for hemangioma?
tongue
what color is hemangioma?
usually red but color can range through various shades of purple depending on caliber and depth of vessels
clinical features of hemangioma
- most elevated, but macular lesions seen
- variable in size
- rapid growth
- gradual involution
tx for hemangioma
- “watchful” neglect unless problematic or life-threatening (normally involute)
- B-blocker, steroids, cryotherapy, embolization, lasers or use of sclerosing agents if cosmetically unacceptable
what is the tx for hemangioma if lesion impairs vision?
tx as soon as possible
when should a child be tx’d for hemangioma?
wait until child is at least 6 years of age
what is the tx if the hemangioma lesion is problematic and/or will require surgery anyway?
surgical excision
