Benign Mesenchymal Tumors - part II Flashcards

1
Q

lipoma

A

benign tumor of adipose tissue (fat)

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2
Q

T/F: lipoma occurs in children

A

false, adults

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3
Q

clinical features of lipoma

A
  1. slow-growing
  2. non-tender
  3. soft, doughy
  4. occasionally found intraorally
  5. yellow if close to the surface
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4
Q

where is lipoma commonly found?

A

in head and neck

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5
Q

histopathologic features of lipoma

A

demarcated or encapsulated collection of mature fat cells (adipocytes)

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6
Q

tx of lipoma

A

enucleation or conservative surgical excision

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7
Q

T/F: lipoma has little to no tendency to recur

A

true

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8
Q

T/F: lipoma has evidence of malignant transformation

A

false, no evidence

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9
Q

NERVE benign mesenchymal tumors

A
  1. traumatic neuroma
  2. neurilemoma (schwannoma)
  3. neurofibroma
  4. melanotic neuroectodermal tumor of infancy
  5. granular cell tumor
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10
Q

T/F: traumatic neuroma is NOT a true neoplasm

A

true

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11
Q

what causes a traumatic neuroma?

A
  1. uncommon rxn to sectioning of a nerve

2. reactive proliferation of n. tissue after damage of the n. bundle

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12
Q

what is the nerve in a traumatic neuroma trying to do?

A

attempts to regenerate and reestablish innervation of the distal segment; encounters scar tissue

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13
Q

clinical features of traumatic neuroma

A
  1. smooth-surfaced
  2. dome-shaped papule or nodule, usually <1 cm
  3. ± tender to palpation
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14
Q

approximately how many oral traumatic neuromas are painful?

A

only ~ 1/4 to 1/3 oral traumatic neuromas are painful

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15
Q

T/F: pain from oral traumatic neuromas can be intermittent or constant

A

true

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16
Q

T/F: pain from oral traumatic neuromas can be mild tenderness to burning severe radiating pain

A

true

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17
Q

common intraoral sites for traumatic neuroma

A
  1. tongue

2. buccal vestibule, especially mental foramen area

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18
Q

T/F: a traumatic neuroma on the buccal vestibule especially in the mental foramen area will be painful when impinged upon by a denture

A

true

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19
Q

histopathologic features of traumatic neuroma

A

tangled mass of peripheral nerve fibers in a collagenous background

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20
Q

tx for traumatic neuroma

A

surgical excision, including a small portion of the proximal nerve bundle

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21
Q

prognosis of traumatic neuroma

A

good

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22
Q

T/F: pain from a traumatic neuroma may persist or return at a later date even after being tx’d

A

true

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23
Q

T/F: recurrence of a traumatic neuroma is common

A

false, not common

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24
Q

neurilemoma (schwannoma)

A

benign tumor of schwann cell origin (surround axons of peripheral nerves)

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25
Q

who is affected by neurilemoma (schwannoma)?

A

most in adults, but any age

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26
Q

where does neurilemoma (schwannoma) occur?

A

occur anywhere in the body

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27
Q

clinical features of neurilemoma (schwannoma)

A
  1. slow-growing
  2. solitary
  3. encapsulated
  4. rubbery-firm
  5. non-tender
  6. may cause expansion
  7. may push on the nerve or other structures
  8. can occur centrally within bone
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28
Q

where are the most common intraoral locations for neurilemoma (schwannoma)?

A
  1. lips
  2. tongue
  3. buccal mucosa
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29
Q

how does neurilemoma (schwannoma) appear on radiographs?

A

radiolucent

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30
Q

T/F: neurilemoma (schwannoma) appears unilocular in radiographs

A

false, unilocular AND multilocular

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31
Q

histopathologic features of neurilemoma (schwannoma)

A
  1. well-developed capsule
  2. benign proliferation of spindle-shaped Schwann cells
  3. patterns seen microscopically
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32
Q

what are the two patterns seen microscopically of neurilemoma (schwannoma)?

A
  1. Antoni A

2. Antoni B

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33
Q

Antoni A

A

palisaded nuclei arranged around Verocay bodies

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34
Q

Verocay bodies

A

acellular zones made of reduplicated basement membrane and cytoplasmic processes

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35
Q

Antoni B

A
  1. less organized

2. sometimes myxoid

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36
Q

tx of neurilemoma (schwannoma)

A
  1. conservative excision

2. usually “shells out” due to dense CT capsule

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37
Q

T/F: neurilemoma (schwannoma) has no tendency to recur

A

true

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38
Q

T/F: neurilemoma (schwannoma) has an extremely rare malignant transformation

A

true

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39
Q

neurofibroma

A

benign tumor of neural fibroblast origin

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40
Q

T/F: over 90% of neurofibroma are solitary

A

true

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41
Q

what is the remaining 10% of neurofibroma?

A

remainder are multiple and associated with neurofibromatosis

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42
Q

T/F: neurofibroma is encapsulated

A

false, UNencapsulated

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43
Q

what is the most common location for a neurofibroma?

A

skin

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44
Q

clinical features of neurofibroma

A
  1. soft
  2. demarcated, dome-shaped papule or nodule, subcutaneous or submucosal
  3. non-tender
  4. can occur centrally within bone
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45
Q

T/F: oral neurofibroma mucosal lesions are not uncommon

A

true, they’re common

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46
Q

where would neurofibromas occur intraorally?

A
  1. tongue

2. buccal mucosa

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47
Q

how does a neurofibroma appear radiographically?

A

radiolucent

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48
Q

T/F: neurofibroma can appear multilocular on radiographs

A

false, can be unilocular or multilocular

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49
Q

histopathologic features of neurofibroma

A
  1. collection of spindle-shaped cells with wavy nuclei
  2. mast cells
  3. lesional tissue mingles with adjacent normal tissue
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50
Q

txx of neurofibroma

A

conservative excision

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51
Q

prognosis of neurofibroma

A

generally good, especially small, superficial lesions

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52
Q

T/F: it is uncommon for neurofibroma to transform to malignant peripheral nerve sheath tumor

A

true

53
Q

T/F: neurofibromatosis is a common hereditary condition

A

true

54
Q

T/F: neurofibromatosis has a variable number of neurofibromas, schwannomas

A

true

55
Q

what are the 3 types of neurofibromatosis?

A
  1. Type 1
  2. Type 2
  3. Schwannomatosis
56
Q

Type 1 (NF1) neurofibromatosis

A

mainly neurofibromas

57
Q

what does Type I neurofibromatosis cause?

A

Von Recklinghausen disease of the skin (majority of neurofibromatosis cases)

58
Q

Type 2 (NF2) neurofibromatosis

A

mainly schwannomas

59
Q

Schwannomatosis neurofibromatosis

A

mainly schwannomas

60
Q

T/F: type 1 neurofibromatosis is autosomal recessive

A

false, dominant

61
Q

a parent with NF1 has what percent chance of having an affected child?

A

50%

62
Q

what percent of new mutations “de novo” does the affected child with type 1 neurofibromatosis have?

A

~50%

63
Q

T/F: type 1 neurofibromatosis has a low variable gene expression

A

false, highly variable (mild to severe)

64
Q

what manifestatiosn does type 1 neurofibromatosis have?

A
  1. cutaneous
  2. oral
  3. ocular
65
Q

cutaneous manifestations of type 1 neurofibromatosis

A
  1. Café-au-lait spots
  2. 6 or more, greater than 1.5 cm in adult
  3. multiple neurofibromas (small and discrete)
  4. plexiform neurofibroma
  5. pathognomonic for NF1
  6. axillary or inguinal freckling
66
Q

Café-au-lait spots

A

light brown macules, smooth borders “coast of California”

67
Q

what is a massive, pendulous neurofibroma in type 1 neurofibromatosis called?

A

plexiform neurofibroma

68
Q

what does a plexiform neurofibroma feel like?

A

“a bag of worms”

69
Q

where are oral manifestations of type 1 neurofibromatosis neurofibromas affect?

A
  1. tongue
  2. gingiva
  3. bone
70
Q

T/F: enlargement fungiform papillae reported for type 1 neurofibromatosis but specificity for NF1 unknown

A

true

71
Q

ocular manifestations of type 1 neurofibromatosis

A

Lisch nodules of the iris

72
Q

Lisch nodules of the iris

A

clear yellow to brown aggregates of dendritic melanocytes within iris

73
Q

diagnosis of type 1 neurofibromatosis require how many criteria?

A

2 or more

74
Q

summary of diagnostic criteria for type 1 neurofibromatosis

A
  1. 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
  2. Two or more neurofibromas, or one plexiform neurofibroma (a neurofibroma that
    involves many nerves)
  3. Freckling in the area of the armpit or the groin
  4. Two or more growths on the iris of the eye (known as Lisch nodules or iris
    hamartomas)
  5. A tumor on the optic nerve (called an optic nerve glioma)
  6. Abnormal development of the spine (scoliosis), the temple (sphenoid) bone of the
    skull, or the tibia (one of the long bones of the shin)
  7. A parent, sibling, or child with NF1
75
Q

tx for NF1

A
  1. remove traumatized neurofibromas or disfiguring lesions
  2. genetic counseling
  3. follow up due to potential of malignant transformation
76
Q

prognosis of NF1

A

fair to guarded

77
Q

what happens to the prognosis of NF1 if malignant transformation occurs?

A

prognosis is poor (usually a malignant peripheral nerve sheath tumor)

78
Q

T/F: melanotic neuroectodermal tumor of infancy

(MNTI) is common

A

false, rare

79
Q

MNTI are of what origin?

A

neural crest

80
Q

when does MNTI develop?

A

usually develops in the 1st year of life

81
Q

where does MNTI occur?

A

anterior maxilla

82
Q

what color is MNTI?

A

brown or black

83
Q

T/F: MNTI grows slowly

A

false, rapidly

84
Q

T/F: most MNTI are benign

A

true (7% act in a malignant fashion)

85
Q

radiographic features of MNTI

A
  1. radiographic appearance is characteristic
  2. radiolucency anterior maxilla
  3. deciduous maxillary incisor pushed labially appearing as “tooth floating in space”
86
Q

histopathologic features of MNTI

A
  1. proliferation of small, dark, neuroectodermal-appearing cells nested in aggregates
  2. surrounded by plump epithelioid cells with vesicular nuclei, which produce melanin
87
Q

tx of MNTI

A

conservative excision usually curative

88
Q

prognosis of MNTI

A

good

89
Q

T/F: there has been sporadic reports of aggressive behavior of MNTI after tx

A

true

90
Q

T/F: there should be clinical follow-up if MNTI is prudent

A

true

91
Q

where does granular cell tumor affect?

A

any cutaneous surface

92
Q

which site is the most common for granular cell tumor?

A

tongue

93
Q

locations for granular cell tumor intraorally

A
  1. tongue
  2. buccal mucosa
  3. floor of mouth
94
Q

clinical features of granular cell tumor

A
  1. slow-growing
  2. demarcated, subcutaneous or submuscosal
  3. non-tender
  4. most <1 cm
95
Q

histopathologic features of granular cell tumor

A
  1. collection of mesenchymal cells with granular-appearing cytoplasm
  2. pseudoepitheliomatous hyperplasia (PEH) present
96
Q

what stain is used for neural differentiation in granular cell tumor?

A

S-100(+)

97
Q

why is granular cell tumor often mistaken for SCC histopathologically?

A

pseudoepitheliomatous hyperplasia (PEH) in granular cell tumor

98
Q

tx of granular cell tumor

A

conservative excision usually curative

99
Q

prognosis of granular cell tumor

A

excellent

100
Q

what causes congenital epulis?

A

undermined histogenesis

101
Q

when and where are congenital epulis found?

A

found at birth on maxillary ridge

102
Q

congenital GCT

A

congenital epulis on tongue (rare)

103
Q

who is affected by congenital epulis?

A

females

104
Q

where do congenital epulis appear in relation to midline?

A

lateral to midline

105
Q

clinical features of congenital epulis

A
  1. smooth surface
  2. often pedunculated
  3. vary in size
106
Q

T/F: congenital epulis are common

A

false, rare

107
Q

histopathologic features of congenital epulis

A
  1. proliferation of cells with granular cytoplasm
  2. atrophy of rete ridges
  3. no PEH
  4. S-100(-); does not stain for neural differentiation with IHC
108
Q

tx for congenital epulis

A
  1. conservative excision

2. some reports of spontaneous resolution without surgery

109
Q

prognosis of congenital epulis

A

excellent

110
Q

T/F: congenital epulis have a tendency to recur

A

false, no tendency to recur

111
Q

blood benign mesencymal tumors

A
  1. hemangioma

2. vascular malformations

112
Q

vascular malformations

A
  1. Port wine stain

2. encephalotrigeminal angiomatosis

113
Q

T/F: hemangioma may be a vascular hamartoma rather than a neoplasm

A

true

114
Q

hamartoma

A

overgrowth of tissue normally present in the site

115
Q

hemangioma

A

benign tumor of infancy

116
Q

what is the most common tumor of childhood?

A

hemangioma

117
Q

T/F: most hemangioma are recognized at birth

A

false, most are NOT recognized at birth

118
Q

when do hemangioma arise?

A

by 8 weeks

119
Q

what is the except of a hemangioma arising by 8 weeks of life?

A

non-involuting congenital hemangioma

120
Q

non-involuting congenital hemangioma

A
  1. fully developed at birth

2. does not involute

121
Q

which site is frequently affected by hemangioma?

A

head and neck

122
Q

what is the most common intraoral site for hemangioma?

A

tongue

123
Q

what color is hemangioma?

A

usually red but color can range through various shades of purple depending on caliber and depth of vessels

124
Q

clinical features of hemangioma

A
  1. most elevated, but macular lesions seen
  2. variable in size
  3. rapid growth
  4. gradual involution
125
Q

tx for hemangioma

A
  1. “watchful” neglect unless problematic or life-threatening (normally involute)
  2. B-blocker, steroids, cryotherapy, embolization, lasers or use of sclerosing agents if cosmetically unacceptable
126
Q

what is the tx for hemangioma if lesion impairs vision?

A

tx as soon as possible

127
Q

when should a child be tx’d for hemangioma?

A

wait until child is at least 6 years of age

128
Q

what is the tx if the hemangioma lesion is problematic and/or will require surgery anyway?

A

surgical excision