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OP - Yield > 12: Soft tissue > Flashcards

12: Soft tissue Flashcards

1
Q

surface of giant cell fibroma

A

papillary

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2
Q

bilateral giant cell fibromas behind MD canines – treatment?

A

retrocuspid papilla, disappears with age

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3
Q

retrocuspid papilla histo

A

giant cell fibroma

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4
Q

frenal tag

A

fibrous hyperplasia, most frequent on maxillary labial frenum

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5
Q

epulis fissuratum

A

hyperplasia of fibrous tissue with ill-fitting denture

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6
Q

fibrous hyperplasia with ill-fitting denture

A

epulis fissuratum

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7
Q

inflammatory papillary hyperplasia

A

usually under denture; mouth breathers or deep palatal vault

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8
Q

palate of mouth breathers

A

inflammatory papillary hyperplasia

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9
Q

benign fibrous histiocytoma histo

A

dermatofibroma

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10
Q

dermatofibroma in mouth

A

benign fibrous histiocytoma

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11
Q

other names for benign fibrous histiocytoma

A

sclerosing hemangioma, fibroxanthoma, nodular subepidermal ffibrosis

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12
Q

most common site of benign fibrous histiocytoma

A

skin of extremities

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13
Q

histo of benign fibrous histiocytoma

A

short intersecting fascicles: storiform (straw mat) or cartwheel pattern; fibroblasts, histiocytes, Touton giant cells, xanthoma cells, lymphocytes, HPC like areas

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14
Q

short intersecting fascicles: storiform (straw mat) or cartwheel pattern

A

benign fibrous histiocytoma

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15
Q

benign fibrous histiocytoma IHC

A

factor XIIIa+, Cd34 - (vs DFSP XIIIa and CD34+)

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16
Q

variants of benign fibrous histiocytoma

A

cellular (higher recurrence) and eputhelioid (mimics Spitz nevus)

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17
Q

which benign fibrous histiocytoma has higher recurrence

A

cellular

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18
Q

touton giant cells look

A

“wreath-like” nuclei around scalloped edge of the cell like a florette

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19
Q

“wreath-like” nuclei around scalloped edge of the cell like a florette

A

touton giant cells

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20
Q

juvenile xanthogranuloma histo

A

sheets of histiocytes (xantho), touton giant cells, +/- eos

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21
Q

juvenile xanthogranuloma IHC

A

CD68, antitrypsin + but S100 and CD1a negative

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22
Q

reticulohistiocytoma aka

A

solitary epithlioid histiocytoma

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23
Q

reticulohistiocytoma IHC

A

CD163+, CD68+

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24
Q

reticulohistiocytoma histo

A

large epithelioid histiocytes with glassy cytoplasm, lymphocytes, neutrophils, giant cells

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25
Q

generalized reticulohistiocytoma

A

multiple skin lesions and granulomatous polyarthritis

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26
Q

xanthoma histo

A

localized collection of tissue histiocytes containing lipid (not a true tumor)

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27
Q

localized collection of tissue histiocytes containing lipid

A

xanthoma

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28
Q

xanthoma systemic conditions

A

most primary and some secondary hyperlipoproteinemias

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29
Q

xanthomas of eyelid

A

xanthelasma

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30
Q

lipid levels in xanthelasma

A

normal

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31
Q

fibrous proliferations intermediate between benign and fibrosarc

A

fibromatosis

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32
Q

fibromatosis entities

A

desmoid tumor, juvenile aggressive fibromatosis, extra-abdominal fibromatosis

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33
Q

fibromatosis in bone

A

desmoplastic fibroma

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34
Q

desmoplastic fibroma

A

fibromatosis in bone

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35
Q

conditions with higher risj for fibromatosis

A

familial adenomatous polyposis and gardner

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36
Q

fibromatosis histo

A

spindle cells infiltrate muscle and fat; cells run parallel to vessels and collagen runs alongsid each cell; abindant collagen )little or no cell-too-cell contact); no hyperchromasia; multinucleated giant cells: atrophic skeletal muscle remnants at periphery of lesion

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37
Q

fibromatosis IHC

A

vimentin, SMA, MSA, beta catenin

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38
Q

sternocleidomastoid tumor

A

fibromatosis colli, most common type, few weeks after birth

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39
Q

most common type of fibromatosis

A

sternocleidomastoid tumor aka fibromatosis colli

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40
Q

juvenile hyaline fibromatosis

A

hereditary; muliple cutaneous papiles/nodules/masses, gingival hyperplasia, joint contracures, osteolytic defects

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41
Q

juvenile hyaline fibromatosis histo

A

cords of spindle cells embedded in homogeneous eosinophilic matrix

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42
Q

prliferation of myofibroblasts

A

myofibroma/myofibromatosis

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43
Q

multicentric myofibromatosis

A

neonates/infants with tumors of skin, bone, visceral organs

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44
Q

histo look of myofibroma

A

may appear biphasic: darker central area and lighter pripheral areas; nofules or whorls of elongated spindle cells, myxoid stroma, staghprn vessels, chondroid areas; may have smooth muslce/fibroblastc features, HPC-like pattern, local infiltration

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45
Q

myofibroma IHC

A

vimentin, SMA, PTAH +; desmin S100 -

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46
Q

nodular fasciitis demographic

A

adults 20-40yo, classically rapid growth, 50% with pain

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47
Q

most nodular fasciitis location

A

upper extremities, trunk, head and neck

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48
Q

nodular fasciitis histo

A

red spindle cells in fascicles and bundles; feathery “tissue culture” appearance; keloid-like collagen fibers; inflammation, hemosiderin, extravasated RBC

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49
Q

feathery “tissue culture” look

A

nodular fasciitis

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50
Q

nodular fasciitic IHC

A

KP-1+

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51
Q

keloid natural history and predilection

A

abnormal wound healing in genetically predisposed patients; dark-skinned; hypertrophic scar confined to original wound site

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52
Q

systemic associations for keloid

A

Ehlers-Danlos, scleroderma, Rubinstein-Taybi syndrome

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53
Q

keloid histo

A

haphazard, thick, glassy, deeply eosinophilic collagen fibers

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54
Q

inflammatory myofibroblastic tumor location and cause

A

most common in lung, overexpression of ALK kinase

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55
Q

inflammatory myofibroblastic tumor IHC

A

MSA, SMA, desmin, ALK-1

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56
Q

oral focal mucinosis cause

A

overproduction of hyaluronic acid by fibroblasts (Alcian blue+)

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57
Q

oral focal mucinosis location and predilection

A

75% gingiva, young females

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58
Q

locations for PG

A

75% gingiva (poor hygiene), also tongue, cheek, lips (trauma related)

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59
Q

PG in pregnancy cause and name

A

increased progesterone/estrogen, granuloma gravidarum

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60
Q

PG in extraction socket

A

epulis granulomatosa

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61
Q

epulis granulomatosa

A

PG in extraction socket

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62
Q

peripheral giant cell granuloma clinical look and location

A

blue-purple mass, exclusive to gingiva or alveolar ridge

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63
Q

peripheral ossifying fibroma look and location, demographic

A

pink or red, exclusively gingiva, more anterior maxilla, teenagers (females), incisor-cuspid area

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64
Q

most common mesenchymal neoplasm

A

lipoma

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65
Q

location for lpoma

A

trunk and proximal extremities, more in obese people

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66
Q

metaplasia in lipoma

A

central ctlg or osseous

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67
Q

highest recurrence rate among lipomas

A

intramuscular 2/2 infiltrative growth

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68
Q

histo look of spindle cell lipoma

A

bland spindle cells, myxoid changes, ropy collagen bundles, scattered mast cells, mature adipocytes

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69
Q

IHC lipoma

A

CD34+

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70
Q

lipoblastoma histo

A

lipoblasts and adipocyte with fibrous connective tissue septa

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71
Q

lipoblasts and adipocyte with fibrous connective tissue septa

A

lipoblastoma

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72
Q

age for lipoblastoma

A

< 3 yo (almost exclusively childrins)

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73
Q

hibernoma origin

A

vestigial remnants of brown fat

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74
Q

which neoplasm arises from vestigial remnants of brown fat

A

hibernoma

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75
Q

hibernoma histo look

A

multivacuolated cells (brown fat, like in hibernating animals)

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76
Q

hibernoma IHC

A

S100+, Cd34 –

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77
Q

hibernoma molecular

A

11q13 and 11q21 rearrangement

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78
Q

11q13 and 11q21 rearrangement neoplasm

A

hibernoma

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79
Q

traumatic neuroma location

A

mental foramen, tongue, lower lip, often with trauma

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80
Q

special complication of parotid surgeries

A

traumatic neuroma of greater auricular nerve in 10%

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81
Q

pain in traumatic neuroma

A

25-33%

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82
Q

palisaded encapsulated neuroma aka

A

solitary circumscribed neuroma

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83
Q

solitary circumscribed neuroma aka

A

palisaded encapsulated neuroma

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84
Q

palisaded encapsulated neuroma location

A

90% face (nose and cheeks)

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85
Q

special artifacts for palisaded encapsulated neuroma

A

cracking and peeling

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86
Q

cracking and peeling characteristics artifacts of what

A

palisaded encapsulated neuroma

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87
Q

neurilemoma syndrome assoc

A

NF2

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88
Q

NF2 assoc with what mesenchymal neoplasm

A

neurilemoma

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89
Q

NF2 inheritance, gene, neoplasms

A

AD, MERLIN (schwannomin) gene mutation (chr 22). b/l neurilemomas of vestibular nerve (acoustic neuromas), neurilemmomas of peripheral nerves, meningiomas and ependymomas of CNS

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90
Q

multiple neurilemomas but no vestibular tumors

A

schwannomatosis

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91
Q

schwannomatosis vs NF2

A

NF2 acoustic neuromas

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92
Q

carney syndrome

A

psammomatous melanocytic schwannomas

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93
Q

psammomatous melanocytic schwannomas syndrome

A

carney syndrome

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94
Q

verocay bodies

A

reduplicated basement membrane and cytoplasmic processes

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95
Q

reduplicated basement membrane and cytoplasmic processes

A

verocay bodies

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96
Q

ancient schwannoma features

A

hemorrhage, vessel hyalinization, pleomorphic cells, verocay bodies, xanthomatous changes, cysts, fibrosis and calcification

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97
Q

schwannoma vs neurofibroma IHC

A

S100 stronger in schwannoma

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98
Q

neurites in schwannoma vs neurofibroma

A

absent in schwannoma, present in NF

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99
Q

most common peripheral nerve neoplasm

A

neurofibroma

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100
Q

special cell in neurofibromas

A

mast cells

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101
Q

many mast cells in spindly lesion

A

prolly neurofibroma

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102
Q

histo look of NF

A

shredded carrot

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103
Q

shredded carrot

A

NF

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104
Q

NF IHC

A

S100 scattered

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105
Q

neurofibromatosis aka

A

von Recklinghausen disease of the skin

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106
Q

von Recklinghausen disease of the skin aka

A

neurofibromatosis

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107
Q

NF1 inheritance, gene, chromosome, product

A

50% AD, 50% new mutation; NF1 gene on chr 17, neurofibromin protein

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108
Q

NF1 vs NF2 gene, chromosome, product

A

NF1: NF1 gene on chr 17, neurofibromin; NF2: MERLIN (schwannomin) gene mutation (chr 22)

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109
Q

large baggy neurofibromas

A

elephantiasus neuromatosa in NF1

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110
Q

elephantiasus neuromatosa

A

large baggy neurofibromas in NF1

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111
Q

pathognomonic feel of neurofibroma in NF1

A

plexiform, bag of worms, especially in trunk

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112
Q

bag of worms skin lesion esp trunk

A

pathognomonic plexiform feel of neurofibroma in NF1

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113
Q

NF Dx

A

cafe au ait (6+), axillary freckling (Crowe’s sign), brown pigmented spots on the iris (Lisch nodules), optic glioma

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114
Q

optic glioma

A

NF1

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115
Q

axillary freckling in NF1

A

Crowe’s sign

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116
Q

Crowe’s sign

A

axillary freckling in NF1

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117
Q

Lisch nodules

A

brown pigmented spots on the iris in NF1

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118
Q

brown pigmented iris spots

A

Lisch nodules, NF1

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119
Q

most common oral finding in NF1

A

enlarged fungiform papillae in up to 50% of patients

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120
Q

most common medical problem in NF1

A

HTN

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121
Q

malignant transformation in NF

A

~5% of NF transform to malignant peripheral nerve sheath tumors; others: RMS, leukemia, pheochromocytoma, Wilms tumor

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122
Q

order of neural lesions occurrence

A

neuroma > schwannoma, NF > ancient schwannoma > plexiform NF

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123
Q

ganglioneuroma age location

A

average age 6y, most common in mediastinum

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124
Q

GI ganglioneuroma look and association

A

polypoid; associated with Cowden, tuberous sclerosis, juvenile polyposis, NF1, MEN2B

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125
Q

ganglioneuroma histo

A

scattered clusters of ganglion cells in a background of schwann cell bundles

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126
Q

ganglion cell look

A

large pink cytoplasm and 1-3 nuclei

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127
Q

neurothekeoma aka

A

nerve sheath myxoma

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128
Q

never sheath myxoma aja

A

neurothekeoma

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129
Q

neurothekeoma histo look

A

lobules of cells and myxoid stroma (hyaluronic acid or sulfated acid)

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130
Q

neurothekeoma IHC

A

S100 and PGP9.5+

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131
Q

cellular neurothekeoma

A

cellular, nuclear atypia, mitosis, extends into fat/mm/vessels

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132
Q

neurothekeoma ddx and how

A

focal mucinosis and myxoid neurofibroma – lobulation is key difference

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133
Q

perineurioma differentiation

A

most cells show perineural differentiation

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134
Q

perineurioma forms

A

intraneural, extraneural )soft tissue), sclerosing, reticaulr

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135
Q

intraneural perineurioma histo look

A

tiny “onion bulbs”, EMA+, S100+

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136
Q

ossifying fibromyxoid tumor of soft parts origin and location

A

possibly neuroectodermal, 70% in extrmities

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137
Q

ossifying fibromyxoid tumor of soft parts histo

A

lobules of uniform, round to fusiform cells in nests and cords, fibromyxoid stroma, incomplete shell of metaplastic hypocellular lamellar bone

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138
Q

ossifying fibromyxoid tumor of soft parts IHC

A

70% are vimentin and S100+; also desmin, Leu7, NSE, GFAP, SMA

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139
Q

granular cell tumor location

A

tongue > cheek, rarely parotid

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140
Q

granular cell tumor multiplicity demographic

A

black

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141
Q

granular cell tumor IHC

A

S100+ (schwann origin??), NK1C3, CD68, Leu7, NSE, MBP; GFAP and neurofilament –

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142
Q

examples of epulides

A

1- Giant cell epulis = PGCG
2- Ossifying fibroid epulis = peripheral ossifying fibroma
3- Congenital epulis

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143
Q

polyp on hard palate under denture

A

Leaf –like or fibroepithelial polyp

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144
Q

Granuloma gravidarum

A

Pyogenic granuloma

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145
Q

Proteus syndrome aka, presentation

A

(Wiedmann Syndrome ): overgrowth and atypical bone development, often
accompanied by tumors over half the body. Mistaken for NF1

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146
Q

other names for granular cell tumor

A

=Granular myoblastoma= Granular cell schwannoma

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147
Q

PHACES syndrome

A 
Large segmental cervicofacial hemangioma
Posterior fossa brain anomaly (Dandy – walker malformation )
Hemangioma
Arterial anomalies
Cardiac defects
Eye anomalies
Sternal cleft or supraumbilical raphe
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148
Q

Kasabach-Merritt phenomenon:

A

hemangioma thrombocytopenia syndrome
 Tufted hemangioma
 Kaposiform hemangioendothelioma

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149
Q

hemangioma thrombocytopenia syndrome

A

Kasabach-Merritt phenomenon:
 Tufted hemangioma
 Kaposiform hemangioendothelioma

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150
Q

Non hereditary developmental condition of hamartomatous vascular proliferation

A

Sturge- Weber Syndrome; Encaphalotrigeminal Angiomatosis; Sturge -Weber Angiomatosis

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151
Q

portwine stain aka

A

nevus flammeus

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152
Q

nevus flammeus aka

A

portwine stain

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153
Q

Sturge Weber mutation

A

GNAQ 9q21

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154
Q

Noonan syndromw

A

o Heart defect
o Web neck
o Flat nose
o Pectum excavatum

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155
Q

fibrous histiocytoma not in the mouth

A

dermatofibroma

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156
Q

dermatofibroma in the mouth

A

Fibrous histiocytoma;

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157
Q

solitary fibrous tumor IHC

A

CD34+, BCL-2, STAT6

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158
Q

cafe au lait spots

A

Coast of Maine = FD

Coats of California = NF

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159
Q

fibromatosis in bone

A

desmoplastic fibroma

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160
Q

fibromatosis syndrome

A

gardner

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161
Q

head and neck fibromatosis

A

Juvenile aggressive fibromatosis or extra-abdominal desmoids

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162
Q

fibromatosis recurrence

A

30%

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163
Q

criteria for neurofibromatosis type 1 (7)

A
  1. 6 ≥ café au lait macules – prepubertal > 5mm / adults >15 mm
  2. 2 ≥ NF or 1 plexiform
  3. Freckling in the axillary region ( Crow sign)
  4. Optic glioma
  5. 2 ≥ Lische nodules ( iris hamartoma)
  6. Osseous lesions
  7. A first degree relative
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164
Q

MNTI: preilection, location, radio look, labs, recurrence

A

 Male predilection
 Anterior maxilla but other sites have been reported
 Sunray radiograph
 Lab : High levels of urinary vanillylmandelic acid (VMA)
 20% recurrence

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165
Q

MNTI IHC

A

 + CK, HMB-45, NSE, CD 56, Synaptophysin in some cases

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166
Q

increased vanillylmandelic acid entities

A

o MNET
o MTC
o Pheochromocytoma
o Neuroblastoma

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167
Q

paraganglioma in head and neck syndromes

A

NF1, MEN 2, von Hippel-lindau

syndrome

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168
Q

sustentacular cells in paragangliomas IHC

A

S100+

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169
Q

paraganglioma mutation and highest rate of malignancy

A

PGL1-4 (SDHB) / Type 4 has highest rate of malignancy 13% -23%

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170
Q

von hippel lindau chromosome and presentation

A

3p; Inherited disorder, formation of tumors and fluid-filled sacs (cysts) in many different
parts of the body. Tumors may be either noncancerous or cancerous and most
frequently appear during young adulthood.

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171
Q

tumors with von hippel lindau

A

angiomatosis, hemangioblastomas, pheochromocytoma, renal cell carcinoma,
pancreatic cysts (pancreatic serous cystadenoma), endolymphatic sac tumor, and
bilateral papillary cystadenomas of the epididymis (men) or broad ligament of the
uterus (women

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172
Q

VHL signs and symptoms

A

headaches, problems with balance and walking, dizziness,

weakness of the limbs, vision problems, and high blood pressure

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173
Q 
favored site: 
Adult rhabdomyoma 
Granular cell tumor 
Hibernoma 
Paraganglioma
A

Adult rhabdomyoma H&N
Granular cell tumor skin, tongue
Hibernoma interscapular
Paraganglioma extra-adrenal ganglioma

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174
Q 
electron microscopy: 
Adult rhabdomyoma 
Granular cell tumor 
Hibernoma 
Paraganglioma
A

Adult rhabdomyoma think/thick filaments
Granular cell tumor phagolysosomes
Hibernoma m/ch
Paraganglioma neurosecretory granules

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175
Q 
S100: 
Adult rhabdomyoma 
Granular cell tumor 
Hibernoma 
Paraganglioma
A

Adult rhabdomyoma rare, focal
Granular cell tumor diffuse
Hibernoma diffuse
Paraganglioma sustentacular cells

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176
Q 
MSA: 
Adult rhabdomyoma 
Granular cell tumor 
Hibernoma 
Paraganglioma
A

Adult rhabdomyoma diffuse
Granular cell tumor neg
Hibernoma neg
Paraganglioma neg

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177
Q 
Chromogranin: 
Adult rhabdomyoma 
Granular cell tumor 
Hibernoma 
Paraganglioma
A

Adult rhabdomyoma neg
Granular cell tumor neg
Hibernoma neg
Paraganglioma diffuse

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178
Q

congenital epulis: sex, location, special cells

A

congenital granular cell lesion: 90% F, Midline, Maxilla

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179
Q

hemangioma of infancy special IHC

A

GLUT1

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180
Q

nasopharyngeal angiofibroma special radio

A

Anterior bowing of posterior wall of maxillary sinus

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181
Q

Anterior bowing of posterior wall of maxillary sinus

A

nasopharyngeal angiofibroma

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182
Q

nasopharyngeal angiofibroma: age, location, recurrence

A 
 Male
 Pterygopalatine fossa
 Sphenopalatine foramen
 Anterior bowing of posterior wall of maxillary sinus
 Recurrence 20 %-40%
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183
Q

lymphangiomas look

A

frog eggs or tapioca

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184
Q

frog eggs or tapioca

A

lymphangioma

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185
Q

macro vs microcystic lymphangioma

A

Macrocystic ( cystic hygroma) : 2cm > , 4% in blacks

 Microcystic: 2cm < / Mixed

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186
Q

cystic hygroma

A

macrocystic (>2cm) lemphangioma, 4% in blacks

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187
Q

spider web

A

rhabdomyoma

188
Q

Batson plexus:

A

Valveless vertebral venous plexus

189
Q

origin of congenital epulis of newborn

A

likely myofibroblastic

190
Q

congenital epulis of newborn aka

A

congenital granular cell lesion

191
Q

congenital granular cell lesion aka

A

congenital epulis of newborn

192
Q

gender, location for congenital epulis of newborn

A

10% multiple, 90% females, Mx>md ib akveikar ridge (rarely on tongue also)

193
Q

congenital epulis of newborn IHC

A

S100 - (vs granular cell tumor), KP-1+, vimentin+; no pseudoepitheliomatous hyperplasia (unlike granular cell tumor)

194
Q

pseudoepitheliomatous hyperplasia granular cell tumor vs granular cell lesion of newborn

A

yes in GCT, not in newborn epulis

195
Q

extracranial meningioma origin

A

ectopic arachnoid lining cells

196
Q

ectopic arachnoid lining cells give rise to

A

extracranial meningioma

197
Q

extracranial meningioma types, location, origin, histo

A

1 - congenital - skin of scalp, forehead, paravertebral; abnormal neural tube closure; intermediate between meningocele an dmeningioma (aka meningeal hamartoma)
2 - adults - close to sensory organs (eye ear nose) - syncytial pattern, swirling whorled balls of cells, collagenous septa, psammoma bodies; CK+

198
Q

type 1 extracranial meningioma location, origin, histo, aka

A

1 - congenital - skin of scalp, forehead, paravertebral; abnormal neural tube closure; intermediate between meningocele an dmeningioma (aka meningeal hamartoma)

199
Q

type 2 extracranial meningioma location, origin, histo, IHC

A

2 - adults - close to sensory organs (eye ear nose) - syncytial pattern, swirling whorled balls of cells, collagenous septa, psammoma bodies; CK+

200
Q

congenital displacement of neuroglial tissue aka

A

glial heterotopia, nasal glioma, glial hamartoma, heterotopic glial tumor; variant of encephalocele

201
Q

glial heterotopia location, clinical, age

A

6-% subQ tissue of nose, 30% nasal cavity; polypoid mass in nose of infant, gros with infant

202
Q

glial heterotopia in mouth

A

glial choristoma

203
Q

glial choristoma histo, IHC

A

glial heterotopia; mats of glial tissue with astocytes; neuronal elements absent; GFAP+ (glial fibrillary acidic proterin), S100+

204
Q

encephalocele vs glial heterotopia

A

encephalocele connects to CNS via defect in cribriform plate; when in nose, same as glial heterotopia

205
Q

encephalocele histo

A

mixture of astrocytes, glial fibers, and neuronal elements (latter absent in glial heterotopia)

206
Q

MEN1 aka, findings

A

Werner sybdrome: 3P: benign tumors of parathyroid, pancreas, and pitutary

207
Q

benign tumors of parathyroid, pancreas, and pitutary which MEN

A

MEN1 aka Werner syndrome

208
Q

MEN2A aka and findings

A

Sipple syndrome, pheochromocytoma and medullary thyroid ca

209
Q

pheochromocytoma and medullary thyroid ca whih MEN

A

MEN2A aka sipple syndrome

210
Q

MEN2B findings and mutation

A

Pheochromocytoma (50%), medullary thyroid ca (90%), mucosal neuromas; RET mutation (chrom 10); protuberant lips in narrow face, eversion of eyelids; marfanoid body (thin long limbs and muscle wasting)

211
Q

mucosal neuromas which MEN

A

MEN2B

212
Q

protuberant lips in narrow face, eversion of eyelids which MEN

A

MEN2B

213
Q

neuromas in MEN2B location

A

lips, anterior tongue, bilatrally on commissures

214
Q

marfanoid body which MEN

A

MEN2B

215
Q

medullary thyroid ca labs

A

increased serum or urinary calcitonin

216
Q

increased serum or urinary calcitonin labs

A

medullary thyroid ca

217
Q

pheochromocytoma labs

A

increased vanillylmandelic acid and epi/norepi ratio

218
Q

increased vanillylmandelic acid and epi/norepi ratio which conditions

A

pheochromocytoma, MNTI, neuroblastoma

219
Q

MNTI labs

A

increased vanillylmandelic acid and epi/norepi ratio

220
Q

neuroblastoma labs

A

increased vanillylmandelic acid and epi/norepi ratio

221
Q

MNTI origin, age, location, gender, xray look

A

neural crest origin, <1 yo, Mx, M>F, sunray pattern on xray

222
Q

MNTI histo and IHC

A

○ Large epithelioid cells CK+, HMB45+, neurospecific enolase +
○ Smaller cells neurospecific enolase +, CD56+, +/- other neuroendocrine markers

223
Q

biphasic: NSE and CD56+ small round cells; CK+ epithelioid cells

A

MNTI

224
Q

what are paraganglia

A

chemoreceptors that detect changes i blood pH and O tension

225
Q

paraganglioma aka

A

chemodectoma

226
Q

paraganglioma origin

A

neural crest

227
Q

carotid body paraganglioma origin, gender, condition

A

neural crest, may arise in response to hypoxia; more in females at higher altitudes

228
Q

cartoid body paraganglioma clinical

A

deep mass below angle of MD with pharyngeal swelling; Fontaine’s sign: lesion moved side to side but no vertical movement

229
Q

Fontaine’s sign

A

lesion moved side to side but no vertical movement – carotid body paraganglioma

230
Q

lesion moved side to side but no vertical movement

A

Fontaine’s sign, carotid body paraganglioma

231
Q

% rule for carotid body paraganglioma (3)

A

10% multlifocal, 10% familial history (genomic imprinting), 10% metastasize

232
Q

carotid body paraganglioma histo and IHC

A

zellballen, highly vascular; chief cells (type 1) – synapto/chromo; sustentacular cells (type 2) S1–+

233
Q

carney’s triad

A

extra-adrenal paraganglioma., gastric leiomyosarcoma, pulmonary chondroma

234
Q

when is carotid body paraganglioma malignant

A

when mets are present

235
Q

jugulotympanic paraganglioma aka

A

glomus jugulare

236
Q

glomus jugulare aka

A

jugulotympanic paraganglioma

237
Q

jugulotympanic paraganglioma location and origin

A

paraganglia or auricular branch of vagal nerve or tympanic branch of glossopharyngeal nerve; temporal bone and middle ear;; glomus jugulare – when arises from jugular bulb;; glomus tympanicum – involves middle ear

238
Q

glomus jugulare

A

jugulotympanic paraganglioma in jugular bulb

239
Q

glomus tympanicum

A

jugulotympanic paraganglioma in middle ear

240
Q

most common neoplasm of middle ear

A

jugulotympanic paraganglioma/glomus tympanicum

241
Q

vagal body tumor aka

A

vagal paraganglioma

242
Q

vagal paraganglioma aka

A

vagal body tumor

243
Q

vagal paraganglioma findings and location

A

high cervical masses between mastoid process and angle of jaw; above carotid bifurcation without widening of bifurcation point

244
Q

high cervical masses between mastoid process and angle of jaw; above carotid bifurcation without widening of bifurcation point

A

vagal paragnglioma

245
Q

glomus tumor origin and function

A

glomus body: regulates temperature, is an AV shunt

246
Q

arterial segment of glomus body

A

Sucquet-Hoyer

247
Q

glomus body composition

A

varying proportions of glomus cells, blood vessels, and smooth muscle

248
Q

glomus tumor clinical, location

A

papulonodular lesions, red/pink/blue, extremely painful;; acral distribution (hand, foot, forearm)

249
Q

glomus tumor types

A

solid, glomangioma, glomangiomyoma (60%), infiltrating, glomangiosarcoma

250
Q

glomangioma histo

A

cookie cutter cells (ptominent cell borders); round, centrally placed, almost neuroendocrine like; cells around vascular lakes

251
Q

glomangiomyoma histo

A

smooth muscle cells near vascular spaces blending with glomus cells

252
Q

hemangioma vs vascular malformation

A

hemangioma arises during first 8 wks, involutes; vascular malformation present at birth, no involution

253
Q

most common tumor of infance

A

hemangioma

254
Q

hemangioma gender, location

A

most common tumor of infancy, F:M 5:1, 60% H&N

255
Q

PHACES syndrome

A

Posterior fossa brain anomaly (Dandy Walker), Hemangioma, Arterial anomalies, Cardiac defects, Eye anomalies, Stenral cleft/supraumbilical raphe

256
Q

Kasabach Merritt phenomenon

A

thrombocytopenia and hemorrhage due to platelets being trapped in tufted hemangioma/or kaposiform hemangioendothelioma

257
Q

thrombocytopenia and hemorrhage due to platelets being trapped in tufted hemangioma/or kaposiform hemangioendothelioma

A

Kasabach Merritt phenomenon

258
Q

intrabony hemangioma radio and clinical

A

bruit/pulsation; multi or unilocular; radiolucent or sunburst

259
Q

hemangioma histo types

A

juvenile or cellular (aka juvenile hemangioendothelioma), capillary, cavernous

260
Q

most common slow growing tumor of infants

A

juvenile hemangioendothelioma aka jivenile/cellular hemangioma

261
Q

hemangioma of infancy IHC

A

GLUT1+ (negative in vascular malformations)

262
Q

intravascular papillary endothelial hyperplasia aka

A

Masson’s tumor, Masson’s hemangioma

263
Q

papillary endothelial hyperplasia origin and histo

A

reactive pseudoneoplastic proliferation of endothelial cells associated with thrombosis; dilated vascular channels with endolthelial-lined papillary circles and stroma

264
Q

reactive pseudoneoplastic proliferation of endothelial cells associated with thrombosis; dilated vascular channels with endolthelial-lined papillary circles and stroma

A

papillary endothelial hyperplasia aka Masson’s tumor

265
Q

Sturge Weber aka

A

Sturge Weber sybdrome or angiomatosis, encephalotrigeminal angiomatosis

266
Q

Sturge Weber clinical

A

port wine stain (nevus flammeus), leptomeningeal angiomas, seizzures, mental retardaton

267
Q

sturge weber intraoral

A

gingiva may become hyperplastic or with PG(from vascular component or use of phenytoin for seizures)

268
Q

sturge weber skull film

A

tramline calcifications

269
Q

tramline calcifications on skull film

A

sturge weber

270
Q

fibrous hyperplasia on maxillary labial frenum

A

frenal tag

271
Q

frenal tag

A

fibrous hyperplasia on maxillary labial frenum

272
Q

retrocuspid papilla

A

fibroma on gingiva lingual to MD canines, disappears with age

273
Q

fibroma on gingiva lingual to MD canines

A

retrocuspid papilla

274
Q

leaflike denture fibroma

A

fibroepithelial polyp on hard palate beneath denture, with serrated edges

275
Q

polyp on hard palate beneath denture

A

leaflike denture fibroma or fibropithelial polyp with serrated edges

276
Q

polyp with serrated edges

A

leaflike denture fibroma

277
Q

fibromatosis of head and neck

A

juvenila aggressive fibromatosis or extra-abominal desmoid tumors

278
Q

higher risk conditions for fibromatosis

A

familial adenomatous polyposis and Gardner

279
Q

familial adenomatous polyposis and Gardner higher risk for

A

fibromatosis

280
Q

multicentric myofibroma

A

myofibromatosis; infants with tumors of skin, bone, viscera

281
Q

infants with tumors of skin, bone, viscera

A

multicentric myofibromatosis

282
Q

oral focal mucinosis location and chemistry

A

increased hyaluronic acid, 75% on gingiva

283
Q

PG in extraction socket

A

epulis granulomatosa

284
Q

epulis granulomatosa

A

PG in extraction socket

285
Q

lipoma with higher recurrence rate

A

intramuscular

286
Q

risk in surgery for PA

A

traumatic neuroma in 10%

287
Q

traumatic neuroma association

A

10% of surgeries for PA

288
Q

schwannoma condition association

A

NF2

289
Q

NF2 inheritance, genetics, chromosome, tumors

A

AD, MERLIN gene mutation (chr 22); acoustic neuromas, neurilemomas of peripheral nerves, meningiomas, ependymomas of CNS

290
Q

NF1 inheritance, genetics, chromosome

A

50% AD, 50% new mutation; NF1 gene on chrom 17, neurofibromin protein

291
Q

chrom 17 which NF

A

1 – neurofibromin

292
Q

chrom 22 which NF

A

2 – MERLIN

293
Q

MERLIN gene

A

NF2

294
Q

large baggy neurofibromas special name

A

elephantiasis neuromatosa

295
Q

bag of worms

A

plexiform neurofibroma

296
Q

plexiform neurofibroma special feeling

A

bag of worms

297
Q

elephantiasis neuromatosa

A

large baggy neurofibromas

298
Q

diagnostic criteria for NF1

A

6+ cafe au lait, axillary freckling (Crowe’s sign), iris hamartoma )Lisch nodules), optic glioma

299
Q

Lisch nodules are

A

iris hamartomas; NF1

300
Q

freckling in NF1

A

Crowe’s sign

301
Q

Crowe’s sign

A

axillary freckling in NF1

302
Q

iris hamartomas in NF1

A

Lisch nodules

303
Q

NF1 syndrome associations

A

Noonan syndrome and CGCG

304
Q

most common problem in NF1

A

HTN

305
Q

MPNST in NF1

A

5% of NF

306
Q

anterior bowing of posterior wall of mx sinus

A

nasopharyngeal angiofibroma

307
Q

nasopharyngeal angiofibroma special radio

A

anterior bowing of posterior wall of mx sinus

308
Q

leiomyoma histo types

A

solid, angioleiomyoma, epithlioid leiomyoma (leiomyoblastoma)

309
Q

leiomyoblastoma aka

A

epithlioid leiomyoma

310
Q

epithlioid leiomyoma aka

A

leiomyoblastoma

311
Q

special stain in rhabdomyoma

A

PTAH

312
Q

MPNST and NF1

A

50% of MPNSTs are in pts with NF1

313
Q

MPNST radio

A

widening of MD canal or mental foramen

314
Q

widening of MD canal or mental foramen

A

suspect MPNST

315
Q

triton tumor

A

MPSNT + malignant skeletal muscle

316
Q

MPSNT + malignant skeletal muscle

A

triton tumor

317
Q

olfactory neuroblastoma location

A

nasal cavity close to cribifrom plate

318
Q

botryoid rhabdomyosarcoma histo

A

cambium layers: zone of increased cellularity below mucosa

319
Q

zone of increased cellularity below mucosa in botryoid rhabdomyosarc

A

cambium layers

320
Q

alevolar rhabdomyosarcs translocations

A

PAX3-FKHR and PAX7-FKHR

321
Q

PAX3-FKHR

A

alveolar rhabdomyosarc

322
Q

PAX7-FKHR

A

alveolar rhabdomyosarc

323
Q

embryonal rhabdomyosarc generics

A

loss of heterozygosity 11p15

324
Q

cowden syndrome

A

multiple trichilemmomas, adnexal hamartomas and nevi, hamartomatous intestinal polyps, breast and other malignancies

325
Q

multiple trichilemmomas, adnexal hamartomas and nevi, hamartomatous intestinal polyps, breast and other malignancies

A

cowden syndrome

326
Q

loss of heterozygosity 11p15

A

embryonal rhabdomyosarc

327
Q

alveolar soft part sarc genetics

A

x17 translocation ASPL-TFE3 fusion protein

328
Q

x17 translocation

A

alveolar soft part sarc

329
Q

ASPL-TFE3 fusion protein

A

alveolar soft part sarc

330
Q

synovial sarc genetics

A

x18 translocation SSx-SS18

331
Q

x18 translocation

A

synovial sarc

332
Q

SSx-SS18

A

synovial sarc

333
Q

synovial sarc IHC

A

CK+, EMA+

334
Q

batson plexus

A

vertebral plexus that bypasses lung filtration (explains distant mets in jaws but not in lungs)

335
Q

distant mets in jaws but not in lungs

A

batson plexus – vertebral plexus that bypasses lung filtration

336
Q

vertebral plexus that bypasses lung filtration

A

batson plexus (explains distant mets in jaws but not in lungs)

337
Q

men soft tissue mets

A

lung, renal, melanoma; prostate goes to bone

338
Q

women soft tissue mets

A

breast (IHC ER PR), genital, lung, bone, kidney

339
Q

Klippel Trenaunay Weber syndrome

A

multiple facial hemangiomas, vascular masses with enlargement of extremities, ocular disorders

340
Q

multiple facial hemangiomas, vascular masses with enlargement of extremities, ocular disorders

A

Klippel Trenaunay Weber syndrome

341
Q

Klippel Trenaunay Weber syndrome oral findings

A

premature tooth eruption and bony overgrowth –> malocclusion

342
Q

nasopharyngeal angiofibroma age gender location

A

exlusively in young males (10-17yo), pterygopalatine fossa

343
Q

lesion of pterygopalatine fossa exclusively in young males

A

nasopharyngeal angiofibroma

344
Q

nasopharyngeal angiofibroma imagina

A

anterior bowing of posterior wall of mx sinus, angiogram useful

345
Q

anterior bowing of posterior wall of mx sinus

A

nasopharyngeal angiofibroma

346
Q

hemangiopericytoma aka

A

solitary fibrous tumor

347
Q

solitary fibrous tumor aka

A

hemangiopericytoma

348
Q

hemangiopericytoma location in mouth and in general

A

75% cheek; common on pleura

349
Q

hemangiopericytoma pattern

A

tightly packed cells around staghorn vessels

350
Q

patternless pattern: spindle cells in short fascicles

A

solitary fibrous tumor/hemangiopericytoma

351
Q

solitary fibrous tumor histo

A

remember aka hemangiopericytoma ~ alternating hyper/hypocellular zone; hypocellular with prominent hyalinized collagen bundles; bands of dense collagen separate individual cells; myxoid bluish background around islands of pleomorphic bland cells; HPC like areas with staghorns

352
Q

solitary fibrous tumor IHC

A

CD34+, CD99+, bcl-2+, SMA -, STAT6

353
Q

tightly packed cells around staghorn vessels

A

hemangiopericytoma (=SFT) like pattern

354
Q

glomangiopericytoma aka

A

sinonasal hemangiopericytoma, glomus tumor, HPC-like tumor; distinct from soft tissue hemangiopericytome

355
Q

sinonasal hemangiopericytoma aka

A

glomangiopericytoma, glomus tumor, HPC - like tumor; distinct from soft tissue hemangiopericytome

356
Q

glomagiopericytoma histo

A

remember aka sinonasal hemangiopericytoma ~ monomorphic spindle to ovoid cells with lightly eosinophilic cytoplasm and bland nuclei forming short fascicles or a storiform/whorled/palisaded pattern; tumor cells aggregate around staghorn vessels

357
Q

PEComa = ; examples, association

A

perivascular epithelioid cell neoplasm; derived from perivascular epithelioid cells. eg renal angiomyolipoma, lymphangiomyomatosis, clear cell sihar tumor of lung; associated with tuberous sclerosis complex

358
Q

tuberous sclerosis classic organ tumors

A

renal angiomyolipoma, cardiacrhabdomyoma; subependymal giant cell astrocytoma

359
Q

renal angiomyolipoma type of tumor and syndrome

A

PEComa ~ tuberous sclerosis

360
Q

PEComa IHC, histo

A

melanocytic and muscle markers, can look like granular cell tumor

361
Q

lymphangiomyomatosis what is it and demographic

A

PEComa, tuberous sclerosis association, exclusively in women of child-bearing age

362
Q

lymphangioma types

A

simplex (capillary), cavernous, cystic (cystic hygroma)

363
Q

lymphangioma location, age

A

50-75% head and neck, 90% <2yo

364
Q

cystic lymphangioma location

A

most in posterior triangle of neck; anterior lesions more complications

365
Q

oral lympahngiomas location and look

A

anterior 2/3 of tongue, frog eggs or tapioca pudding

366
Q

neonates and lymphangiomas?

A

4% of black babies, esp boys, have bilateral lymphangiomas on mandibular alveolar ridge

367
Q

leiomyoma locationas

A

most in uterus, GI tract, skin

368
Q

leiomyoma histo types

A

solid, angioleiomyoma, epithelioid leiomyoma (leiomyoblastoma)

369
Q

leiomyoma staining

A

Masson red muscle, SMA+, MSA+

370
Q

rhabdomyoma types and locations

A

adult (pharynx, larynx, FOM, soft palate, base of tongue) and fetal (face, periauricular)

371
Q

adult rhabdomyoma look and location

A

pharynx, larynx, FOM, soft palate, base of tongue; spider-web ~ peripheral vacuoles with filaments

372
Q

fetal rhabdomyoma look and location

A

face, periauricular ~ haphazard spindle cells (muscle looking) in a myxoid stroma

373
Q

rhabdomyoma IHC

A

myoglobin, desmin, MSA, PTAH + (PTAH myofibrils stain purple)

374
Q

PTAH staining

A

purple myofibrils and rhabdomyoma

375
Q

normal tissue in abnormal location

A

choristoma

376
Q

excess tissue in normal location

A

hamartoma

377
Q

hamartoma vs choristoma

A

hamartoma excess tissue in normal location; choristoma normal tisue in abnormal location

378
Q

osseous and cartilaginous choristoma location gender

A

85% posterior tongue, 70% women

379
Q

ectomesenchymal chondromycoid tumor location, histo, IHC

A

always anterior tongue; well defined multilobulated; spindle to round cells in a myxoid or chondroid background; GFAP+

380
Q

multinucleate cell angiohistiocytoma origin and presentation

A

chronic inflammatory disorder of unknown cause; multiple firm, red-purple, dome shaped, coalescing/linearly arranged papules on skin of limbs

381
Q

multiple firm, red-purple, dome shaped, coalescing/linearly arranged papules on skin of limbs

A

multinucleate cell angiohistiocytoma, chronic inflammatory disorder of unknown cause

382
Q

multinucleate cell angiohistiocytoma histo, IHC

A

Histo: proliferation of small blood vessels with unusual dendritic cells and multinucleate cells; IHC endothelial - factor VIII-RA, CD34, CD31; dendritic - factor XIIIa, lysozyme, a1 antichymotrypsin, vimentin, CD68; giant cells - vimentin only

383
Q

fibrosarcoma location and diagnosis

A

diagnosis of exclusion; most in deep soft tissues of lower extremities, especially thigh and knee

384
Q

fibrosarcoma histo

A

herrinbone: fascicles of spindle cells

385
Q

infantile fibrosarcoma aka and age

A

congenital fibrosarcoma, aggressive infantile fibromatosis; congenital or within first year of life

386
Q

infantile fibrosarcoma histo

A

identical to adult but more prominent HPC-like growth

387
Q

infantile fibrosarcoma genetics

A

12 15 translocation –> ETV6-NTRK3 fusion protein

388
Q

12 15 translocation

A

ETV6-NTRK3 fusion protein

secretory Ca

389
Q

ETV6-NTRK3 fusion protein

A

secretory carcinoma, 12 15 translocation

390
Q

dermatofibrosarcoma protuberans genetics

A

translocation 17, 22 (Col11A1-PDGFbeta fusion and increase in PDGFbeta chain)

391
Q

translocation 17, 22

A

dermatofibrosarcoma protuberans (Col11A1-PDGFbeta fusion and increase in PDGFbeta chain)

392
Q

Col11A1-PDGFbeta

A

increase in PDGFbeta chain, dermatofibrosarcoma protuberans, translocation 17, 22

393
Q

increase in PDGFbeta chain,

A

dermatofibrosarcoma protuberans, translocation 17, 22; Col11A1-PDGFbeta fusion

394
Q

dermatofibrosarcoma distribution

A

bathing suit

395
Q

dermatofibrosarcoma protuberans histo and IHC

A

monotonous haphazard storiform arrangement of spindle and stellate cells that infiltrate and entrap adnexa and adipose; low mitoses; IHC: Cd34+, factor XIIIa neg (vs BFH, CD34-, XIIIa+)

396
Q

pigmented dermatofibrosarcoma protuberans

A

Bednar’s tumor

397
Q

Bednar’s tumor

A

pigmented dermatofibrosarcoma protuberans

398
Q

giant cell fibroblastoma

A

juvenile form of dermatofibrosarcoma protuberans

399
Q

juvenile form of dermatofibrosarcoma protuberans

A

giant cell fibroblastoma

400
Q

malignant fibrous histiocytoma age and pattern

A

older patients, storiform pattern

401
Q

most commo soft tissue sarcoma (%, age?)

A

liposarcoma - 20% of all malignancies in adults

402
Q

liposarcoma location

A

most in thigh, retroperitoneum, inguinal

403
Q

liposarcoma types

A

well diff (most common in oral cavity), myxoid/round cell, pleomorphic, dedifferentiated

404
Q

most common liposarcoma in mouth

A

well diff

405
Q

worst prognosis of liposarcoma

A

pleomorphic

406
Q

malignant peripheral nerve sheath tumor vs NF1

A

50% of MPNSTs are in NF1 pts; pts with MPNST and NF1 are 10y younget (35 vw 45) and worse prognosis than without NF1

407
Q

MPNST location

A

proximal extremities and trunk

408
Q

MPNST xray

A

widening of MD canal or mental forament

409
Q

malignant triton tumor

A

MPNST + malignant skeletal muscle

410
Q

MPNST + malignant skeletal muscle

A

malignant triton tumor

411
Q

MPNST histo and IHC

A

low-power: distinctive perivascular tumor cells surrounded by necrosis: S100+ in only 50% of caes

412
Q

angiosarcoma location and better prognosis

A

most in scalp and forehear; early lesion resembles a bruise; better prognosis for oral and salivary gland tumors

413
Q

hemangioendothelioma

A

intermedite between hemangioma and angiosarcoma

414
Q

angiosarcoma IHC

A

CD31+, factor VIII+; CD34 less consistnet

415
Q

angiosarcoma environmental risk factors

A

PVC vinyl chloride carcinogen; radiation (increased incidence in women with history of breast cancer

416
Q

stewart-treves syndrome

A

angiosarc with chronic lymphedema

417
Q

angiosarc with chronic lymphedema

A

stewart-treves syndrome

418
Q

kaposi sarcoma types

A

classic, endemic (benign, aggresive, florid, lymphadenopathic), iatrogenic, AIDS related

419
Q

kaposi stages

A

patch, plaque, nodular

420
Q

patch stage of kaposi

A

normal structures admixed with tumor proliferation, promontory sign

421
Q

leiomyosarcoma location

A

most in uterine wall and GI tract

422
Q

epithelioid leiomyosarcoma histo

A

entirely of rounded cells

423
Q

leiomyosarcoma mutation

A

some associated with RBI mutation

424
Q

RBU mutation

A

leiomyosarcoma (some)

425
Q

leiomyosarcoma special stain

A

PAS shows glycogen within the cells; cell cytoplasm red on Masson trichrome; MSA (HHF35), SMA, desmin, H-caldesmon, SMMS (smooth muscle myosin)

426
Q

rhandomyosarcoma frequency

A

60% of soft tissue sarcomas in children

427
Q

types of rhabdomyosarcomas and ages

A

embryonal (NOS, botryoid, spindle) - <10 yo; alveolar (10-25yo), pleomorphic >40yo

428
Q

most common sarcoma in children

A

rhabdomyosarcoma

429
Q

embryonal rhabdomyosarc histo

A

round, spindle, and strap cells

430
Q

rhabdomyosarcoma location

A

head and neck: face/orbit; and GU tract; oral rhabdomyosarc - palate and maxillary sinus

431
Q

rhabdomyoblasts histo

A

small cells with dark nucleo and depply pink cytoplasm

432
Q

botryoid rhabdomyosarc

A

exophytic polypoid growth in a cavity (mouth, vag(

433
Q

exophytic polypoid rhabdomyosarc in a cavity (mouth, vag)

A

botryoid, type of embryonal

434
Q

cambium layers in rhabdomyosarc

A

zone of increased cellularity just below mucosa in botryoid

435
Q

zone of increased cellularity just below mucosa in botryoid rhabdomyosarc

A

cambium layers

436
Q

alveolar rhabdomyosarc genetics

A

PAX3-FKHR and PAX7-FKHR translocation

437
Q

PAX3-FKHR

A

alveolar rhabdomyosarc

438
Q

PAX7-FKHR

A

alveolar rhabdomyosarc

439
Q

embryonal rhabdomyosarc genetics

A

11p15 LOH

440
Q

11p15 LOH

A

embryonal rhabdomyosarc

441
Q

rhabdomyosarc IHC

A

myogenin, myoD1, HHF-35

442
Q

alveolar soft part sarcoma geneics

A

X;17 translocation, ASPL-TFE3 fusion protein

443
Q

ASPL-TFE3 fusion protein

A

alveolar soft part sarcoma, X;17 translocation

444
Q

X;17 translocation

A

alveolar soft part sarcoma, ASPL-TFE3 fusion protein

445
Q

alveolar soft part sarcoma age sex location

A

young patients – orbit and tongue, more females; adults – lower extremities, more males

446
Q

alveolar soft part sarcoma histo and IHC

A

discohesive cells in nests, minimal atypia, vascular inastion; TFE3+ only IHC marker;; CRYSTALS! crystals are PAS+, diastase resistant; latticework pattern on EM

447
Q

crystals in alveolar soft part sarcoma

A

crystals are PAS+, diastase resistant; latticework pattern on EM

448
Q

synovial sarcoma genetics

A

X;18 translocation - SYT gene chr 18 and SSX1/SSX2 on gene X; –> SYT/SSX fusion mRNA can be detected by RT-PCR or FISH

449
Q

X:18 translocation

A

synovial sarcoma; SYT gene chr 18 and SSX1/SSX2 on gene X; –> SYT/SSX fusion mRNA can be detected by RT-PCR or FISH

450
Q

SYT-SSX fusion mRNA

A

X;18 translocation; synovial sarcoma

451
Q

synovial sarcoma location

A

most near large joints and bursae of extremities; head and neck: paravertebral and parapharyngeal mass

452
Q

synovial sarcoma histo and IHC

A

classically biphasic: spindle cells ~ fibrosarcoma + epithelial cells around glandlike spaces or in nests/cords/whorls.
“slit-oma” calcifications in 30%, staghorn vessels;;; CK+, EMA+, CD99+

453
Q

follicular dendritic cell sarcoma origin and location

A

arises from dendritic (antigen-presenting) cells; predilection for LN of neck, axilla, mediastinum

454
Q

follicular dendritic cells histo, IHC

A

syncytial appearing spindly cells in fascicles or whorls; CD21, CD35+

455
Q

ewing sarcoma aka

A

primitive neuroectodermal tumor

456
Q

primitive neuroectodermal tumor aka

A

ewing sarcoma

457
Q

ewing sarcoma demographics and presentation

A

usually whites <20yo, mandible. fever, increased ESR, leukocytosis

458
Q

ewing sarcoma genetics

A

11,22 translocation. MIC2 gene product. CD99+. FISH against translocation more specific

459
Q

11, 22 translocation

A

Ewing sarcoma, MIC2 gene product (CD99+ but FISH against translocation more specific)

460
Q

MIC2 gene product

A

Ewing sarcoma, 11, 22 translocation. (CD99+ but FISH against translocation more specific)

461
Q

periosteal reaction in ewing

A

onionskin – long bones, not common in jaws

462
Q

atypical ewing sarcoma

A

larger cells

463
Q

ewing sarcoma histo

A

small undifferentiated round blue cells, probably neuroectodermal. 75% contain glycogen granules in cytoplasm.

464
Q

ewing sarcoma locations with poorer prognosis

A

pelvic and proximal lesions

465
Q

most common locations for mets to oral soft tissues

A

gingiva ~50% (looks like PG) and tongue 25%

OP - Yield (19 decks)

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