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OP - Yield > 14: Bone pathology > Flashcards

14: Bone pathology Flashcards

1
Q

osteogenesis imperfecta aka

A

brittle bone disease

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2
Q

brittle bone disease aka

A

osteogenesis imperfecta

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3
Q

osteogenesis imperfecta type 1 presentation

A

most common, blue sclera, joint laxity, hearing loss (hypoacusis), kyphoscoliosis

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4
Q

osteogenesis imperfecta type 2 presentation

A

Most severe , prenatal, respiratory, DEATH

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5
Q

most common osteogenesis imperfecta

A

type 1, blue sclera, joint laxity, hearing loss

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6
Q

most severe osteogenesis imperfecta

A

type 2, prenatal, respiratory, DEATH

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7
Q

osteogenesis imperfecta type 3 presentation

A

mod-severe, popcorn calcification, opalescent teeth

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8
Q

osteogenesis imperfecta type 4 presentation

A

mild-mod, opalescent teeth

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9
Q

blue sclera and opalescent teeth

A

osteogenesis imperfecta

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10
Q

wormian bone

A

small sutures found in a mosaic pattern;; osteogenesis imperfecta, cleidocranial dysplasia

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11
Q

osteopetrosis aka

A

Albers-Schonberg

Marble bone

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12
Q

Albers-Schonberg

aka

A

osteopetrosis, marble bone

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13
Q

marble bone aka

A

Albers-Schonberg

osteopetrosis

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14
Q

osteopetrosis mechanism and presentation

A

High density bone

Normocytic anemia, granulocytopenia
Broad face, hypertelorism, snub nose, frontal bossing
Failure of tooth eruption
Erlenmeyer flask defect in distal femur

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15
Q

osteopetrosis types

A

1-AR infantile “ malignant” type
2-AR intermediate
3-AR dominant adult “ benign”

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16
Q

Erlenmeyer flask defect in distal femur

A 
CHONG
 C: craniometaphyseal dysplasias
 H: haemoglobinopathies
o thalassemia
o sickle cell disease
 O: osteopetrosis
 N: Niemann-Pick disease
 G: Gaucher disease
Lead GNOME
 Lead: poisoning
 G: Gaucher disease
 N: Niemann-Pick disease
 O: osteopetrosis
 M: metaphyseal dysplasia (Pyle disease) and craniometaphyseal dysplasia
 E: 'emalological, e.g. thalassemia
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17
Q

Cleidocranial dysplasia aka and genetics

A

(Cleidocranial dysostosis) RUNX2 (CBFA1)

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18
Q

RUNX2 (CBFA1)

A

Cleidocranial dysplasia

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19
Q

pagets disease aka

A

(Osteitis Deformans)

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20
Q

pagets disease clinical

A

Simian (monkey like) Bowing deformity
Leontiasis Ossea: Lion face in extreme cases alveolar ridge expands
Osteoporosis Circumscripta: Large RL in skull “Cotton wool”
Beard “Lincoln sign”: Bone scan uptake from condyle to condyle
Mosaic pattern or jigsaw
Tam O’shanter sign “ Scottish hat cranium”

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21
Q

cotton wool

A

pagets

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22
Q

lincoln sign

A

pagets on scintigraphy uptake

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23
Q

mosaic pattern/jigsaw histo

A

pagets

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24
Q

leontiasis ossea

A

lion face alveolar ridge expands pagets

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25
Q

etiologic agent possibly in pagets?

A

paramyxovirus

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26
Q

paramyxovirus implicated in

A

paget, measles, mumps

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27
Q

cherubism genetics

A

SH3BP2 gene on chromosome 4p14

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28
Q

SH3BP2 gene

A

chromosome 4p14 cherubism

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29
Q

chromosome 4p14

A

SH3BP2 gene cherubism

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30
Q

massive osteolysis aka

A

Gorham disease; Vanishing bone disease; Idiopathic osteolysis

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31
Q

Gorham disease aka

A

massive osteolysis; Vanishing bone disease; Idiopathic osteolysis

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32
Q

Vanishing bone disease aka

A

massive osteolysis Gorham disease Idiopathic osteolysis

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33
Q

massive osteolysis etiology

A

50% recall prior trauma

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34
Q

fibrous dysplasia syndromes

A

Jaffe-Lichtenstein
McCune-Albright
Mazabraud

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35
Q

fibrous dysplasia genetics

A

GNAS-Guanine nucleotide binding protein α subunit ( postzygotic)

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36
Q

GNAS mutation

A

fibrous dysplasia

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37
Q

ground glass look

A

fibrous dysplasia

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38
Q

fibrous dysplasia location

A

70-80% cases /maxilla / posterior

Craniofacial fibrous dysplasia : maxillary lesions involve adjacent structures

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39
Q

polyostotic fibrous dysplasia age and mutation

A

Most diagnosed before 10 yrs

FGFR23

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40
Q

mazabraud

A

fibrous dysplasia + intramuscular myxoma

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41
Q

fibrous dysplasia + intramuscular myxoma

A

mazabraud

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42
Q

jaffe lichtenstenin

A

Polyostotic FD + café au lait

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43
Q

Polyostotic FD + café au lait

A

jaffe lichtenstein

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44
Q

Polyostotic FD + café au lait + endocrinopathies

A

Mccune Albright

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45
Q

Mccune Albright

A

Polyostotic FD + café au lait + endocrinopathies (sexual precocity, hyperpara, hyperthyroid, hypercortisol)

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46
Q

endocrinopathies in mccune albright

A

(sexual precocity, hyperpara, hyperthyroid, hypercortisol)

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47
Q

gnatho diaphyseal dysplasia inheritance, genetics

A

AD, GDD1 (TMEM16E) ~ similar to gigantiform cementoma

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48
Q

GDD1

A

gnatho diaphyseal dysplasia (TMEM16E)

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49
Q

(TMEM16E)

A

GDD1 gnatho diaphyseal dysplasia

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50
Q

gnatho diaphyseal dysplasia presentation

A

Diffused FOL of jaw
Psammomatoid component
Bone fragility
Bowing of bone

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51
Q

ossifying fibroma genetics, syndrome, characteristic radio/histo findings

A

HRPT 2 a few reported cases
Mutation also seen in Hyperparathyroidism – jaw tumor syndrome
Downward bowing of the inferior cortex / brush borders

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52
Q

HRPT 2

A

a few ossifying fibromas, also seen in Hyperparathyroidism – jaw tumor syndrome

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53
Q

Downward bowing of the inferior cortex

A

ossifying fibroma radio

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54
Q

brush borders of cementicles

A

ossifying fibroma histo

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55
Q

juvenile ossifying fibroma types, location, age

A 
Psammomatoid is more common in craniofacial skeleton paranasal and orbit
Orbital psammomatoid JOF (X:2)
Trabecular younger age group
Trabecular more common in jaw
Maxilla
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56
Q

psammomatoid juvenile ossifying fibroma genetics

A

(X:2)

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57
Q

(X:2)

A

psammomatoid juvenile ossifying fibroma

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58
Q

Gardner syndrome

A

Adenomatous polyposis coli (APC) 5q21 = β Catenin / Wnt pathway /

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59
Q

Adenomatous polyposis coli syndrome association

A

Garnder, (APC) 5q21 = β Catenin / Wnt pathway /

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60
Q

5q21

A

ademonatoud polyposis coli (APC) 5q21 = β Catenin / Wnt pathway / ~ Garnder syndrome

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61
Q

β Catenin / Wnt pathway /

A

Garnder, (APC) 5q21~ Adenomatous polyposis coli

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62
Q

Gardner syndrome polyp behavior

A

Almost 100 % malignant transformation of polyps

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63
Q

Gardner syndrome presentation

A 
Almost 100 % malignant transformation of polyps
 Multiple osteoma
 Odontoma
 Epidermoid cyst
 Cutaneous findings such as lipoma, fibroma, neurofibroma
 Desmoid tumor
 Supernumerary teeth
 Ocular fundus
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64
Q

multiple osteomas syndrome

A 
Gardner; also Almost 100 % malignant transformation of polyps
 Multiple osteoma
 Odontoma
 Epidermoid cyst
 Cutaneous findings such as lipoma, fibroma, neurofibroma
 Desmoid tumor
 Supernumerary teeth
 Ocular fundus
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65
Q

polyps with 100% malignant transformation

A

Gardner
Adenomatous polyposis coli (APC) 5q21 = β Catenin / Wnt pathway /
 Almost 100 % malignant transformation of polyps
 Multiple osteoma
 Odontoma
 Epidermoid cyst
 Cutaneous findings such as lipoma, fibroma, neurofibroma
 Desmoid tumor
 Supernumerary teeth
 Ocular fundus

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66
Q

chondroma location, genetics

A

Hand and foot

 Isocitrate dehydrogenase 1 ( IDH1) mutation

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67
Q

Isocitrate dehydrogenase 1 ( IDH1) mutation

A

chondroma

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68
Q

multuple chondromas syndromes

A

: Ollier disease & Maffucci Syndrome

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69
Q

ollier vs maffucci

A

ollier more common; unilateral multiple enchondromas in ollier

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70
Q

ollier disease presentation, transformation, radio

A

unilateral distribution of multiple enchondromas (vs maffucci)
 Non-hereditary sporadic
 Short and bowing bones
 skeletal, visceral and brain malignancy
 chondrosarcoma
 snowflake due to calcifications

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71
Q

unilateral distribution of multiple enchondromas

A

ollier

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72
Q

maffucci sybdrome presentation, transformation

A

multiple enchondromas associated with multiple hemangiomas and lymphangiomas
o chondrosarcoma

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73
Q

multiple enchondromas associated with multiple hemangiomas and lymphangiomas
o chondrosarcoma

A

maffucci sybdrome

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74
Q

Loose bodies“ joint mice”

A

Synovial chondromatosis:

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75
Q

Numb chin syndrome:

A

Metastatic disease

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76
Q

osteosarcoma periosteal reaction, radio

A

Codman triangle * also in Ewing sarcoma*
 Symmetrical Widening of periodontal ligament “ Garrington sign”
 Spiking “ root resorption”
 Cernia’s sign: molar distalization

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77
Q

Codman triangle

A

osteosarc and ewing periosteal reaction

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78
Q

Garrington sign”

A

Symmetrical Widening of periodontal ligament in osteosarc

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79
Q

Cernia’s sign:

A

molar distalization in osteosarc

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80
Q

osteosarc genetics

A

P53, RB1, Ch # 21

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81
Q

P53, RB1, Ch # 21

A

osteosarc

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82
Q

Ewing sarc genetics

A

t (11:22) EWSR-FLI1

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83
Q

EWSR-FLI1

A

Ewing sarc t (11;22)

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84
Q

t (11;22)

A

EWSR-FLI1 Ewing sarc

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85
Q

Onion skin periosteal reaction

A

1-Ewing sarcoma
2-Garres
3-Periosteal osteosarcoma

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86
Q

Aneurysmal bone cyst (ABC) genetics

A

USP6 on chr 7

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87
Q

t(16;17)(q22.p13)

A

Aneurysmal bone cyst (ABC)

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88
Q

Chondroblastoma genetics

A

H3F3A & H3F3B

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89
Q

H3F3A & H3F3B

A

Chondroblastoma and Giant cell tumor of bone

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90
Q

Giant cell tumor of bone genetics

A

H3F3A & H3F3B

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91
Q

Fibrous Dysplasia (FD) genetics

A

GNAS1

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92
Q

GNAS1

A

Fibrous Dysplasia (FD)

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93
Q

Cherubism genetics

A

SH3BP2

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94
Q

SH3BP2

A

cherubism

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95
Q

Noonan Syndrome genetics

A

PTPN11,SOS1,RAF1

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96
Q

PTPN11

A

Noonan or Leopard syndrome

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97
Q

SOS1

A

Noonan

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98
Q

RAF1

A

Noonan

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99
Q

Leopard Syndrome genetics

A

PTPN11

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100
Q

Craniofacial cutaneous syndrome genetics

A

BRAF,MAP2K1

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101
Q

BRAF

A

Cardiofaciocutaneous syndrome is a disorder that affects many parts of the body, particularly the heart (cardio-), facial features (facio-), and the skin and hair (cutaneous).

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102
Q

MAP2K1

A

Craniofacial cutaneous syndrome

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103
Q

Chondroma genetics

A

IDH1

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104
Q

IDH1

A

chondroma

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105
Q

Ossifying fibroma genetics

A

HRPT2 in some cases

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106
Q

osteogenesis imperfecta genetics

A

COL1A1 or COL1A2 –> impairment of collage type 1 maturation

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107
Q

COL1A1 or COL1A2 mutation

A

impairment of collage type 1 maturation; osteogenesis imperfecta

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108
Q

impairment of collage type 1 maturation

A

COL1A1 or COL1A2 –> osteogenesis imperfecta

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109
Q

most common type of inherited bone disease

A

osteogenesis imperfecta

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110
Q

osteogenesis imperfecta presentation

A

bone fragility, blue sclera, opalescent teeth, hearing loss, joint hyperextensibility

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111
Q

bone fragility, blue sclera, opalescent teeth, hearing loss, joint hyperextensibility

A

osteogenesis imperfecta

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112
Q

opalescent teeth look

A

blue to brown translucency, may have shell teeth - thin dentin, normal enamel

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113
Q

blue to brown translucency of teeth

A

opalescent teeth, osteogenesis imperfecta

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114
Q

shell teeth

A

thin dentin, normal enamel; osteogenesis or dentinogenesis imperfecta

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115
Q

wormian bones

A

10+ sutural bones arranged in mosaic pattern

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116
Q

sutural bones in mosaic pattern

A

wormian

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117
Q

osteogenesis imperfecta types

A

1 - most common/mildestm blue sclera; 2 - most severe/stillborn; 3 most severe perinatal, no blue sclera; 4 - moderate adults, no blue sclera

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5
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118
Q

osteogenesis without blue sclera

A

3 and 4 (arguably 2 also because stillborn)

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119
Q

most common osteogenesis imperfecta

A

1

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120
Q

most severe osteogenesis imperfecta

A

2 – stillborn

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121
Q

what fails in osteopetrosis

A

osteoclasts fail –> increase in bone density

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122
Q

infantile osteopterosis aka

A

malignant; marrow failure, bone fractures, cranial nerve compression

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123
Q

infantile osteopetrosis look and radio

A

broad face, snub nose, frontal bossing, deafness, blindness, facial paralysis, osteomyelitis;; xray diffuse sclerosis of bone

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124
Q

broad face, snub nose, frontal bossing, deafness, blindness, facial paralysis, osteomyelitis

A

infantile osteopetrosis

125
Q

marrow failure which osteopterosis

A

infantile/malignant

126
Q

transient osteopetrosis presentation and treatment

A

diffuse sclerosis and marrow failure, resolves without therapy

127
Q

histo osteopetrosis

A

bone deposition in marrow space, absence of howship lacunae

128
Q

bone deposition in marrow space, absence of howship lacunae

A

osteopetrosis

129
Q

CBFA1 aka and function

A

RUNX2, chr 6, cleidocranial dysplasia; guides osteoblasts/bone formation

130
Q

clavicle in cleidocranial dysplasia

A

malformed: absence, hypoplasia –> droopy shoulders

131
Q

cleidocranial dysplasia presentation

A

short stature; frontal/parietal bossing; long swan neck, hypertelorism, depressed nasal bridge

132
Q

short stature; frontal/parietal bossing; long swan neck, hypertelorism, depressed nasal bridge

A

cleidocranial dysplasia

133
Q

cleidocranial skull and dental

A

delayed closure of sutures; supernumerary teeth (60+)m unerupted teeth, cleft or high arched palate

134
Q

focal osteoporotic marrow defect demographics

A

increaed marrow; most posterior MD in F

135
Q

idiopathic osteosclerosis location

A

90% MD, posterior, usually root apex

136
Q

radiopacity assoc with nonvital tooth

A

condensing osteitis or focal sclerosing chronic osteomyelitis

137
Q

idiopathic osteosclerosis vs osteoma

A

osteoma grows and cortical expansion

138
Q

gorham stout what is bone replaced with; cause?

A

vessels then dense fibrous tissue

cause: hyperactivity of bone; angiomatosis (multicentric proliferation of blood or lymphatics)

139
Q

mutation in pagets

A

SQSTM1 aka p62 – NFkB pathway;; also VCM (valosin containing protein)

140
Q

SQSTM1 mutation aka and what

A

pagets, aka p62 – NFkB pathway

141
Q

p62 mutation aka and what

A

SQSTM1. pagets, NFkB pathway

142
Q

pagets, inclusion body myopathy, and frontotemporal dementia mutation

A

valosin-containing protein (VCP) – NFkB pathway

143
Q

valosin-containing protein

A

pagets, inclusion body myopathy, and frontotemporal dementia mutation – NFkB pathway

144
Q

pagets more common mono or polyostotic

145
Q

common complaint in pagets

A

bone pain and limited mobility

146
Q

most commonly affected bones in pagets

A

lumbar vertebrae, pelvis, skull, femur

147
Q

simian stance in pagets why

A

bowing deformity of weight bearing bones

148
Q

bowing deformity of weight bearing bones in pagets called

A

simian stance

149
Q

mx enlargement –> spacing of teeth of denture too tight

A

pagets; leontiasis ossea

150
Q

leontiasis ossea what is

A

enlargement of middle 1/3 of face in pagets

151
Q

osteoporosis circumscripta

A

large RL in skull of pagets (early phase)

152
Q

early pagets skull radio

A

large RL – osteoporosis circumscripta

153
Q

osteoblastic phase of pagets look

A

cotton wool - patchy sclerotic areas, hypercementosis of teeth

154
Q

ddx COD vs pagets

A

if clinical expansion think Pagets

155
Q

histo of pagets

A

reversal lines with jigsaw or mosaic looking bone; highly vascular stroma

156
Q

reversal lines with jigsaw or mosaic looking bone

A

pagets; highly vascular stroma

157
Q

labs in pagets

A

elevated serum alk phos; normal Ca and P; increased hydroxyproline in urine

158
Q

increased hydroxyproline in urine

159
Q

pagets complications

A 
hypercementosis --> difficult exo
hemorrhage when active/lytic
osteomyelitis whn late
osteosarc )suspect if worsening pain, new mass or sudden frx)
giant cell tumors
160
Q

CGCG demographic and location

A

F < 30yo anteiror MD crossing midline

161
Q

nonaggressive vs aggressive CGCG

A

non – most cases, no symptoms, slow growth

aggressive - painm rapid growth

162
Q

CGCG areas seen in what other lesions

A

ABC, central odontogenic fibroma

163
Q

CGCG in hyperparathyroidism

A

Brown tumor

164
Q

Brown tumor

A

CGCG like in hyperparathyroidism

165
Q

multifocal CGCG concerns

A

cherubism in children, hyperparathyroidism

166
Q

CGCG histo

A

spindle cells and giant cells

167
Q

CGCG recurrence; when higher?

A

15-20%; more if more cellular or more spread out giant cells

168
Q

non-sx treatment of aggressive CGCG

A

steroids, calcitonin, IFN apha 2a

169
Q

giant cell tumor location, histo, recurrence, malignancy

A

epiphyses of long tubular bones, locally aggressive; histo = CGCG; higher recurrence than jaw; malignant transform in 10% of cases

170
Q

cherubism inheritance, sex, genetics

A

AD, 50-70% penetrance F. 100% penetrance M, SH3BP2 mutation (4p16)

171
Q

SH3BP2

A

4p16 cherubism

172
Q

4p16

A

SH3BP2 cherubism

173
Q

eyes turned up to heaven why and what

A

orbital cherubism, wide exposed sclera below iris

174
Q

CGCG of cherubism bs CGCG of hyperparathyroid histo

A

cherubism: eosinophilic cuffing around blood vessels

175
Q

eosinophilic cuffing around blood vessels seen in

A

cherubism, pulse granulomas, schwannomas

176
Q

multiple giant cell lesions syndromes

A

Ramon, Jaffe Campanacci, Noonan-like

177
Q

Ramon syndrome presentation

A

cherubism + gingival fibromatosis

178
Q

cherubism + gingival fibromatosis

A

Ramon syndrome

179
Q

simple bone cyst look during surgery

A

empty or fluid containing cavity, no epithelium

180
Q

simple bone cyst location, age, jaws

A

most in long bones; jaw cases in pts 10-20yo; usually unilateral, posterior MD

181
Q

characteristic simple bone cyst xray

A

scalloping between roots

182
Q

scalloping between roots on xray

A

characteristic simple bone cyst

183
Q

simple bone cyst assoc with other lesions

A

florid COD

184
Q

what can florid COD be associated with

A

simple bone cysts

185
Q

anurysmal bone cyst genetics

A

17;16 translocation

186
Q

17;16 translocation

A

aneurysmal bone cyst

187
Q

aneurysmal bone cyst age

188
Q

ballooning or blow-out distortion of bone contour

A

aneurysmal bone cyst

189
Q

blood soaked sponge

A

aneurysmal bone cyst

190
Q

aneurysmal bone cyst histo

A

spaces filled with blood, cellular fibroblastic stroma, lacelike calcifications

191
Q

spaces filled with blood, cellular fibroblastic stroma, lacelike calcifications

A

aneurysmal bone cyst

192
Q

other lesions often associated with aneurysmal bone cyst

A

CGCG or BFOLs

193
Q

fibrous dysplasia genetics

A

GNAS1 mutation

194
Q

GNAS1 mutation

A

fibrous dysplasia

195
Q

fibrous dysplasia mechanism

A

replacement of bone by connective tissue

196
Q

fibrous dysplasia more commonly mono or poly

A

80% are mono

197
Q

fibrous dysplasia radio look

A

ground glass

198
Q

ground glass radio

A

fibrous dysplasia

199
Q

polyostotic FD + cafe au lait

A

Jaffe Lichtenstein

200
Q

polyostotic FD + cafe au lait + endocrine

A

McCune Alrbight

201
Q

possible endocrine disturbances in McCune albright

A

sexual precocity, putiutary adnoma, hyperPTH

202
Q

polyostotic FD + intramuscular myxomas

A

Mazabraud syndrome (increased risk of osteosarc)

203
Q

Mazabraud syndrome presentation and risks

A

polyostotic FD + intramuscular myxomas

increased risk of osteosarc

204
Q

which polyostotic FD higher risk of osteosarc

205
Q

hockey stick deformity

A

fibrous dysplasia; leg length discrepancy due to involvement of upper femur

206
Q

electrolytes in fibrous dysplasia and why

A

renal phosphate wasting (bone produces FGF23) –> hypophosphatemia

207
Q

cafe au lait in fibrous dysplasia descripror – vs what other?

A

coast of Maine vs coast of Cali in NF

208
Q

chinese characters histo

A

fibrous dysplasia

209
Q

bone trabeculae in fibrous dysplasia

A

monotonous – chinese characters

210
Q

most common BFOL

A

cemento-osseous dysplasia

211
Q

COD vs ossifying fibroma:

sx, histo, hemorrhage

A

OF separate easily from surrounding bone, have to scrape out COD
cementum like particles in COD have retraction artifact – OF more ovoid and brish borders
– COD hemorrhage throughout lesion, OF on periphery

212
Q

dental considerations with CODs

A

prone to necrosis (avoid bx and exo) and osteomyleitis

213
Q

familial gigantiform cementoma inheritance, demographics, age, extent

A

AD, whites, first decade, all four quads

214
Q

familial gigantiform cementoma most similar to what other BFOL

A

florid COD (histo also); ultimately leads to massive sclrotic masses of disorganized mineralized material

215
Q

familial gigantiform cementoma labs

A

increased serum alk phos (declines after surgical removal); anemia – GYN exam for polypoid adenomas

216
Q

ossifying fibroma genetics

A

HRPT2 parafibromin

217
Q

parafibromin

A

ossifying fibroma HRPT2

218
Q

HRPT2

A

parafibromin ossifying fibroma;; also in hyperPTH-jaw tumor syndrome

219
Q

ossifying fibromas characteristic radio

A

downward bowing of md cortex

220
Q

downward bowing of md cortex

A

ossifying fibroma

221
Q

hyperPTH jaw tumor syndrome

A

HRPT2 mutation; patathyroid tumors, OF of jaws, renal cysts, Wilms tumor

222
Q

ossifying fibroma syndrome association

A

hyperPTH jaw tumor syndrome

HRPT2 mutation; patathyroid tumors, OF of jaws, renal cysts, Wilms tumor

223
Q

ossifying fibroma histo

A

hard tissue may be osteoid, bone, or cementum like (vs more uniform in fibrous dysplasia)

224
Q

how is juvenile ossifying fibroma different from regular

A

age of patient, site of involvement, clinical behavior == males, Mx, rapid growth

225
Q

types of juvenile ossifying fibromas

A

trabecular and psammomatoid

226
Q

psammomatoid juvenile ossifying fibroma genetics

A

x;2 translocation

227
Q

x;2 translocation

A

psammomatoid juvenile ossifying fibroma

228
Q

trabecular vs psammomatoid juvenile ossifying fibroma

A

trabecular younger patients; psammomatoid 4x more common; 70% extragnathic (orbit, frontal bone and paranasal sinuses)

229
Q

juvenile ossifying fibroma can be associated with what other entity

A

aneurysmal bone cysts

230
Q

osteoma types-locations

A

periosteal (peripheral/exophytic), endosteal (central), cutis (in muscle or dermis)

paranasal sinuses (usually frontal) more common than jaws

gnathic posterior mandible (lingual surface) or condyle
also coronoid, ramus, angle of md

231
Q

condylar osteoma causes clin and ddx

A

shift of chin; ddx condylar hyperplasia, hemifacial hyperplasia

232
Q

osteoma xray

A

periosteal - sclerotic mass; endosteal – similar to idiopathic osteosclerosis

233
Q

osteoma histo types

A

compact - dense bone with minimal marrow; cancellous – bone trabeculae and marrow

234
Q

gardner syndrome genetics

A

APC (adenomatous polyposis coli)

235
Q

do gardners polyps transform

A

colorectal yes almost 100%; but not small intestine/stomacj

236
Q

gardner findings other than polyps and where

A

osteomas - 3-6, skull, paranasal sinuses, angle of mandible – precede bowel lesions
dental – odontomas, supernumerary and impacted teeth
also epidermoid cysts, thyroid ca, desmoid tumors, and pigmentation of ocular fundus (90%)

237
Q

epidermoid cysts and osteomas

238
Q

osteblastoma vs osteoid osteoma difference

A

osteoblastoma: >2cm, posterior MD, <30yo
osteoid osteoma: <2cm, produce prostaglandins, nocturnal pain alleviated by aspirin, rare in jaws
radio: osteoblastoma RL or mixed, no rim
osteoid osteoma lucent with target like nidus, usually has rim

239
Q

aggressive osteoblastoma aka and how different

A 
aka epithelioid; 
atypical histo (similar to well diff osteosarc), aggressive behavior, >4cm. >30yo, pain
240
Q

osteoid osteoma and osteoblastoma histo

A

reversal lines, blue bone, bland, plump, active osteoblasts

241
Q

cementoblastoma location, age, symptoms, radio

A

attached to root with lucent rim
75% in mandible, 90% in posterior mandible, 50% assoc w 1 molar
75% <30yo
2.3 pain and swelling

242
Q

most severe osteogenesis imperfecta

A

2 most sever –> death; 3 most severe in adults

243
Q

adult osteopterosis aka and forms

A

benign osteopetrosis – one form with nerve compression, two with fractures

244
Q

intermediate osteopetrosis what is?

A

less severe from of infantile disease –> resolves spontaneously

245
Q

CGCG areas in what other lesions

A

ABC and central odontogenic fibroma

246
Q

ABC areas in what other lesions

A

OF or CGCG

247
Q

ollier vs maffucci

A

ollier – multiple chondromas, usually unilateral; maffucci – skeletal chondromatosis and soft tissue angiomas

248
Q

chondromyxoid fibroma genetics

A

chromosome 6 abnormality

249
Q

chromosome 6 abnormality

A

chondromyxoid fibroma

250
Q

synovial chondromatosis aka and what is

A

chondrometaplasia; metaplastic cartilage in synovial membrane

251
Q

desmoplastic fibroma syndomr

A

tuberous sclerosis

252
Q

codman triangle

A

periosteal triangular elevation in osteosarc

253
Q

types of juxtacortical osteosarc

A

aka peripheral; parosteal (in periosteum) and periosteal (at interface with cortex)

254
Q

postradiation sarcomas what are

A

50% osteosarc; 40% fibrosarc

255
Q

ewing translocation

256
Q

11;22 translocation

257
Q

metastatic to bone most common

A

breast, lung, thyroid, prostate, kidney most common primary

258
Q

chondroma locations

A

most in short bones of hands and feet

259
Q

chondromas in jaws arise from

A

cartilaginous rests – anterior maxilla, condyle symphysis, coronoid process

260
Q

ollier disease vs maffucci syndrome

A

ollier: multiple chondromas, usually unilateral
maffucci: multiple chondromas and hemangiomas; phleboliths; skin macules

261
Q

chondromyxoid fibromas genetics and location

demographics and symptoms

A

chromosome 6 abnormality
rare in jaws, usually long bones
usually <30yo, Md, pain and swelling

262
Q

chromosome 6 abnormality

A

chondromyxoid fibromas

263
Q

chondromyxoid fibroma histo

A

lobules of spindle or stellate cells with myxoid or chondroid intercellular substance;; lobules seprated by cellular tissue with spindle or round cells and giant cells

264
Q

lobules of spindle or stellate cells with myxoid or chondroid intercellular substance;; lobules seprated by cellular tissue with spindle or round cells and giant cells

A

chondromyxoid fibroma

265
Q

synovial chondromatosis what is and where and why

A

chondrometaplasia; metaplstic nodule of cartilage in synovial membrane, usually large joints;; rarely in TMJ with nonspecific symptoms
sometimes secondary to other joint conditions (traums, overuse); primary - when no identifiable factor

266
Q

metaplstic nodule of cartilage in synovial membrane

A

synovial chondromatosis

267
Q

stages of synovial chondromatosis

A

1- foci in synovial lining
2 - foci increase and detach, material found in synovial membrane and joint
3 - cartilage only in joint

268
Q

synovial chondromatosis radio finding

A

loose bodies – round irregular radopacities in joint region on xray although this is not specific

269
Q

loose bodies on radio

A

synovial chondromatosis although not specific

270
Q

synovial chondromatosis histo

A

nodules of cartilage within synovium and loose in joint space; may ossify

271
Q

nodules of cartilage within synovium and loose in joint space

A

synovial chondromatosis

272
Q

bone counterpart of fibromatosis and syndrome

A

desmoplastic fibroma; sometimes assoc with tuberous sclerosis

273
Q

desmoplastic fibroma is bony variant of

A

fibromatosis

274
Q

desmoplastic fibroma location

A

posterior md (molar and angle-ascending

275
Q

desmoplastic fibroma histo

A

small fibroblasts and abundant collagen

276
Q

most common non hematopoietic malignancy of bone

277
Q

osteosarc age

A

bimodal: 10-20yo (period of bone growth) and 50+ (pagets or history of radiation)
jaw osteosarc 33 yo (vs 23yo long bones)

278
Q

gnathic osteosarc location

279
Q

gnathic osteosarc radio findings

A

spiking f roots, sunburst, symmetrical PDL widening (tumor infiltration)

280
Q

codman triangle

A

periosteal elevation in osteosarc

281
Q

causes of secondary osteosarc

A

irradiation to bone, fibrous dysplasia, Pagets, bone infarct

282
Q

long bone osteosarc: gender, age, spread

A

M, <20yo, hematogenous

283
Q

types and aka of peripheral osteosarc

A

peripheral = juxtacortical
parosteal — pedunculated, no elevation of periosteum. spindle cells + bone, low grade
periosteal – sessile, elevated periosteum, primitive sarcoma with chondroid and osteoid, mid grade

284
Q

parosteal vs periosteal – which is higher grade

A

parosteal is low grade, periosteal is mid grade

285
Q

which osteosarc pedunculated, no elevation of periosteum. spindle cells + bone, low grade

286
Q

which osteosarc sessile, elevated periosteum, primitive sarcoma with chondroid and osteoid, mid grade

A

periosteal

287
Q

postirradiation sarcoma, how soon, what dose, what types

A

average 14y after radiation, >7000cGy

50% osteosarc, 40% fibrosarc

288
Q

chondrosarc frequency, location, symptoms, age

A

half as common as osteosarc, twice as common as ewing
rare in jaws
mx, painless (vs usually p ainful osteosarc)
>50yo (osteosarc is younger)

289
Q

chondorsarcs of head and neck location

A

rare in jaws; mx

30% are extraosseous (larynx or soft tissue)

290
Q

chondrosarc histo

A

often lobular pattern, periphery of lobules more immature

291
Q

grades of chondrosarc

A

1 - similar to chondroma but look for binucleated chondrocytes
2 - increased cellularity, matrix is myxoid
3 0 highly cellular with spindle cells

292
Q

chondrosarc histo types

A

well diff, mesenchymal, clear cell, periosteal, and dedifferentiated (well diff + fibrosarc)

293
Q

mesenchymal chondrosarc frequency. age

A

10% of all chondrosarcs

most common in jaws; younger patients

294
Q

mesenchynal chondrosarc histo

A

biphasic: spindle/round undff cells with HPC like areas (staghorn vessels)
cartilagenous component variable, usually low grade and well demarcated

295
Q

mesenchymal chondrosar IHC

A

cells are CD99+, cartilagenous component S100+

HEY1-NCOA2 transloc

296
Q

chordoma origin

A

malignant tumor that recapitulates notochord – arises from its remntnats

297
Q

chordoma categories – which is most common

A

spheno occipital, sacro coccygeal (most common), vertebral

298
Q

chordoma in skull location and symptoms

A

clivus and parasellar most common location, usually in midline
symptoms: headache and diplopia – 6th nerve palsy

299
Q

tumor in clivus or parasellar, usually midline, with headache and diplopia 2/2 6th nerve palsy

300
Q

chordoma histo

A

lobular with fibrous septa
lobules consist of solid masses of tumor cells or pools of mucin with tumor cells
physaliferous cells – large cells with central nuclei and vacuolated and reticulated cytoplasm

301
Q

physaliferous cells

A

chordoma!

large cells with central nuclei and vacuolated and reticulated cytoplasm

302
Q

large cells with central nuclei and vacuolated and reticulated cytoplasm in chordoma

A

physaliferous

303
Q

chondroid chordoma

A

contains hyaline type cartilaginous tissue with tumor cells in lacunae

304
Q

chordoma IHC

A

CK. EMA, S100, NSE+

305
Q

most common jaw cancer

A

metastatic carcinoma

306
Q

most common primaries for jaw mets

A

breat lung thyroid prostate kidney

307
Q

jaw mets location

A

80% to mandible

308
Q

numb chin syndrome

A

loss of sensation in lower lip and chin, common in jaw mets

309
Q

loss of sensation in lower lip and chin

A

numb chin syndrome, common in jaw mets

OP - Yield (19 decks)

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