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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
etiologic agent possibly in pagets?
paramyxovirus
26
paramyxovirus implicated in
paget, measles, mumps
27
cherubism genetics
SH3BP2 gene on chromosome 4p14
28
SH3BP2 gene
chromosome 4p14 cherubism
29
chromosome 4p14
SH3BP2 gene cherubism
30
massive osteolysis aka
Gorham disease; Vanishing bone disease; Idiopathic osteolysis
31
Gorham disease aka
massive osteolysis; Vanishing bone disease; Idiopathic osteolysis
32
Vanishing bone disease aka
massive osteolysis Gorham disease Idiopathic osteolysis
33
massive osteolysis etiology
50% recall prior trauma
34
fibrous dysplasia syndromes
Jaffe-Lichtenstein McCune-Albright Mazabraud
35
fibrous dysplasia genetics
GNAS-Guanine nucleotide binding protein α subunit ( postzygotic)
36
GNAS mutation
fibrous dysplasia
37
ground glass look
fibrous dysplasia
38
fibrous dysplasia location
70-80% cases /maxilla / posterior | Craniofacial fibrous dysplasia : maxillary lesions involve adjacent structures
39
polyostotic fibrous dysplasia age and mutation
Most diagnosed before 10 yrs | FGFR23
40
mazabraud
fibrous dysplasia + intramuscular myxoma
41
fibrous dysplasia + intramuscular myxoma
mazabraud
42
jaffe lichtenstenin
Polyostotic FD + café au lait
43
Polyostotic FD + café au lait
jaffe lichtenstein
44
Polyostotic FD + café au lait + endocrinopathies
Mccune Albright
45
Mccune Albright
Polyostotic FD + café au lait + endocrinopathies (sexual precocity, hyperpara, hyperthyroid, hypercortisol)
46
endocrinopathies in mccune albright
(sexual precocity, hyperpara, hyperthyroid, hypercortisol)
47
gnatho diaphyseal dysplasia inheritance, genetics
AD, GDD1 (TMEM16E) ~ similar to gigantiform cementoma
48
GDD1
gnatho diaphyseal dysplasia (TMEM16E)
49
(TMEM16E)
GDD1 gnatho diaphyseal dysplasia
50
gnatho diaphyseal dysplasia presentation
Diffused FOL of jaw Psammomatoid component Bone fragility Bowing of bone
51
ossifying fibroma genetics, syndrome, characteristic radio/histo findings
HRPT 2 a few reported cases Mutation also seen in Hyperparathyroidism – jaw tumor syndrome Downward bowing of the inferior cortex / brush borders
52
HRPT 2
a few ossifying fibromas, also seen in Hyperparathyroidism – jaw tumor syndrome
53
Downward bowing of the inferior cortex
ossifying fibroma radio
54
brush borders of cementicles
ossifying fibroma histo
55
juvenile ossifying fibroma types, location, age
``` 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 ```
56
psammomatoid juvenile ossifying fibroma genetics
(X:2)
57
(X:2)
psammomatoid juvenile ossifying fibroma
58
Gardner syndrome
Adenomatous polyposis coli (APC) 5q21 = β Catenin / Wnt pathway /
59
Adenomatous polyposis coli syndrome association
Garnder, (APC) 5q21 = β Catenin / Wnt pathway /
60
5q21
ademonatoud polyposis coli (APC) 5q21 = β Catenin / Wnt pathway / ~ Garnder syndrome
61
β Catenin / Wnt pathway /
Garnder, (APC) 5q21~ Adenomatous polyposis coli
62
Gardner syndrome polyp behavior
Almost 100 % malignant transformation of polyps
63
Gardner syndrome presentation
``` Almost 100 % malignant transformation of polyps  Multiple osteoma  Odontoma  Epidermoid cyst  Cutaneous findings such as lipoma, fibroma, neurofibroma  Desmoid tumor  Supernumerary teeth  Ocular fundus ```
64
multiple osteomas syndrome
``` 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 ```
65
polyps with 100% malignant transformation
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
66
chondroma location, genetics
Hand and foot |  Isocitrate dehydrogenase 1 ( IDH1) mutation
67
Isocitrate dehydrogenase 1 ( IDH1) mutation
chondroma
68
multuple chondromas syndromes
: Ollier disease & Maffucci Syndrome
69
ollier vs maffucci
ollier more common; unilateral multiple enchondromas in ollier
70
ollier disease presentation, transformation, radio
unilateral distribution of multiple enchondromas (vs maffucci)  Non-hereditary sporadic  Short and bowing bones  skeletal, visceral and brain malignancy  chondrosarcoma  snowflake due to calcifications
71
unilateral distribution of multiple enchondromas
ollier
72
maffucci sybdrome presentation, transformation
multiple enchondromas associated with multiple hemangiomas and lymphangiomas o chondrosarcoma
73
multiple enchondromas associated with multiple hemangiomas and lymphangiomas o chondrosarcoma
maffucci sybdrome
74
Loose bodies“ joint mice”
Synovial chondromatosis:
75
Numb chin syndrome:
Metastatic disease
76
osteosarcoma periosteal reaction, radio
Codman triangle * also in Ewing sarcoma*  Symmetrical Widening of periodontal ligament “ Garrington sign”  Spiking “ root resorption”  Cernia’s sign: molar distalization
77
Codman triangle
osteosarc and ewing periosteal reaction
78
Garrington sign”
Symmetrical Widening of periodontal ligament in osteosarc
79
Cernia’s sign:
molar distalization in osteosarc
80
osteosarc genetics
P53, RB1, Ch # 21
81
P53, RB1, Ch # 21
osteosarc
82
Ewing sarc genetics
t (11:22) EWSR-FLI1
83
EWSR-FLI1
Ewing sarc t (11;22)
84
t (11;22)
EWSR-FLI1 Ewing sarc
85
Onion skin periosteal reaction
1-Ewing sarcoma 2-Garres 3-Periosteal osteosarcoma
86
Aneurysmal bone cyst (ABC) genetics
USP6 on chr 7
87
t(16;17)(q22.p13)
Aneurysmal bone cyst (ABC)
88
Chondroblastoma genetics
H3F3A & H3F3B
89
H3F3A & H3F3B
Chondroblastoma and Giant cell tumor of bone
90
Giant cell tumor of bone genetics
H3F3A & H3F3B
91
Fibrous Dysplasia (FD) genetics
GNAS1
92
GNAS1
Fibrous Dysplasia (FD)
93
Cherubism genetics
SH3BP2
94
SH3BP2
cherubism
95
Noonan Syndrome genetics
PTPN11,SOS1,RAF1
96
PTPN11
Noonan or Leopard syndrome
97
SOS1
Noonan
98
RAF1
Noonan
99
Leopard Syndrome genetics
PTPN11
100
Craniofacial cutaneous syndrome genetics
BRAF,MAP2K1
101
BRAF
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).
102
MAP2K1
Craniofacial cutaneous syndrome
103
Chondroma genetics
IDH1
104
IDH1
chondroma
105
Ossifying fibroma genetics
HRPT2 in some cases
106
osteogenesis imperfecta genetics
COL1A1 or COL1A2 --> impairment of collage type 1 maturation
107
COL1A1 or COL1A2 mutation
impairment of collage type 1 maturation; osteogenesis imperfecta
108
impairment of collage type 1 maturation
COL1A1 or COL1A2 --> osteogenesis imperfecta
109
most common type of inherited bone disease
osteogenesis imperfecta
110
osteogenesis imperfecta presentation
bone fragility, blue sclera, opalescent teeth, hearing loss, joint hyperextensibility
111
bone fragility, blue sclera, opalescent teeth, hearing loss, joint hyperextensibility
osteogenesis imperfecta
112
opalescent teeth look
blue to brown translucency, may have shell teeth - thin dentin, normal enamel
113
blue to brown translucency of teeth
opalescent teeth, osteogenesis imperfecta
114
shell teeth
thin dentin, normal enamel; osteogenesis or dentinogenesis imperfecta
115
wormian bones
10+ sutural bones arranged in mosaic pattern
116
sutural bones in mosaic pattern
wormian
117
osteogenesis imperfecta types
1 - most common/mildestm blue sclera; 2 - most severe/stillborn; 3 most severe perinatal, no blue sclera; 4 - moderate adults, no blue sclera
118
osteogenesis without blue sclera
3 and 4 (arguably 2 also because stillborn)
119
most common osteogenesis imperfecta
1
120
most severe osteogenesis imperfecta
2 -- stillborn
121
what fails in osteopetrosis
osteoclasts fail --> increase in bone density
122
infantile osteopterosis aka
malignant; marrow failure, bone fractures, cranial nerve compression
123
infantile osteopetrosis look and radio
broad face, snub nose, frontal bossing, deafness, blindness, facial paralysis, osteomyelitis;; xray diffuse sclerosis of bone
124
broad face, snub nose, frontal bossing, deafness, blindness, facial paralysis, osteomyelitis
infantile osteopetrosis
125
marrow failure which osteopterosis
infantile/malignant
126
transient osteopetrosis presentation and treatment
diffuse sclerosis and marrow failure, resolves without therapy
127
histo osteopetrosis
bone deposition in marrow space, absence of howship lacunae
128
bone deposition in marrow space, absence of howship lacunae
osteopetrosis
129
CBFA1 aka and function
RUNX2, chr 6, cleidocranial dysplasia; guides osteoblasts/bone formation
130
clavicle in cleidocranial dysplasia
malformed: absence, hypoplasia --> droopy shoulders
131
cleidocranial dysplasia presentation
short stature; frontal/parietal bossing; long swan neck, hypertelorism, depressed nasal bridge
132
short stature; frontal/parietal bossing; long swan neck, hypertelorism, depressed nasal bridge
cleidocranial dysplasia
133
cleidocranial skull and dental
delayed closure of sutures; supernumerary teeth (60+)m unerupted teeth, cleft or high arched palate
134
focal osteoporotic marrow defect demographics
increaed marrow; most posterior MD in F
135
idiopathic osteosclerosis location
90% MD, posterior, usually root apex
136
radiopacity assoc with nonvital tooth
condensing osteitis or focal sclerosing chronic osteomyelitis
137
idiopathic osteosclerosis vs osteoma
osteoma grows and cortical expansion
138
gorham stout what is bone replaced with; cause?
vessels then dense fibrous tissue | cause: hyperactivity of bone; angiomatosis (multicentric proliferation of blood or lymphatics)
139
mutation in pagets
SQSTM1 aka p62 -- NFkB pathway;; also VCM (valosin containing protein)
140
SQSTM1 mutation aka and what
pagets, aka p62 -- NFkB pathway
141
p62 mutation aka and what
SQSTM1. pagets, NFkB pathway
142
pagets, inclusion body myopathy, and frontotemporal dementia mutation
valosin-containing protein (VCP) -- NFkB pathway
143
valosin-containing protein
pagets, inclusion body myopathy, and frontotemporal dementia mutation -- NFkB pathway
144
pagets more common mono or polyostotic
poly
145
common complaint in pagets
bone pain and limited mobility
146
most commonly affected bones in pagets
lumbar vertebrae, pelvis, skull, femur
147
simian stance in pagets why
bowing deformity of weight bearing bones
148
bowing deformity of weight bearing bones in pagets called
simian stance
149
mx enlargement --> spacing of teeth of denture too tight
pagets; leontiasis ossea
150
leontiasis ossea what is
enlargement of middle 1/3 of face in pagets
151
osteoporosis circumscripta
large RL in skull of pagets (early phase)
152
early pagets skull radio
large RL -- osteoporosis circumscripta
153
osteoblastic phase of pagets look
cotton wool - patchy sclerotic areas, hypercementosis of teeth
154
ddx COD vs pagets
if clinical expansion think Pagets
155
histo of pagets
reversal lines with jigsaw or mosaic looking bone; highly vascular stroma
156
reversal lines with jigsaw or mosaic looking bone
pagets; highly vascular stroma
157
labs in pagets
elevated serum alk phos; normal Ca and P; increased hydroxyproline in urine
158
increased hydroxyproline in urine
pagets
159
pagets complications
``` hypercementosis --> difficult exo hemorrhage when active/lytic osteomyelitis whn late osteosarc )suspect if worsening pain, new mass or sudden frx) giant cell tumors ```
160
CGCG demographic and location
F < 30yo anteiror MD crossing midline
161
nonaggressive vs aggressive CGCG
non -- most cases, no symptoms, slow growth | aggressive - painm rapid growth
162
CGCG areas seen in what other lesions
ABC, central odontogenic fibroma
163
CGCG in hyperparathyroidism
Brown tumor
164
Brown tumor
CGCG like in hyperparathyroidism
165
multifocal CGCG concerns
cherubism in children, hyperparathyroidism
166
CGCG histo
spindle cells and giant cells
167
CGCG recurrence; when higher?
15-20%; more if more cellular or more spread out giant cells
168
non-sx treatment of aggressive CGCG
steroids, calcitonin, IFN apha 2a
169
giant cell tumor location, histo, recurrence, malignancy
epiphyses of long tubular bones, locally aggressive; histo = CGCG; higher recurrence than jaw; malignant transform in 10% of cases
170
cherubism inheritance, sex, genetics
AD, 50-70% penetrance F. 100% penetrance M, SH3BP2 mutation (4p16)
171
SH3BP2
4p16 cherubism
172
4p16
SH3BP2 cherubism
173
eyes turned up to heaven why and what
orbital cherubism, wide exposed sclera below iris
174
CGCG of cherubism bs CGCG of hyperparathyroid histo
cherubism: eosinophilic cuffing around blood vessels
175
eosinophilic cuffing around blood vessels seen in
cherubism, pulse granulomas, schwannomas
176
multiple giant cell lesions syndromes
Ramon, Jaffe Campanacci, Noonan-like
177
Ramon syndrome presentation
cherubism + gingival fibromatosis
178
cherubism + gingival fibromatosis
Ramon syndrome
179
simple bone cyst look during surgery
empty or fluid containing cavity, no epithelium
180
simple bone cyst location, age, jaws
most in long bones; jaw cases in pts 10-20yo; usually unilateral, posterior MD
181
characteristic simple bone cyst xray
scalloping between roots
182
scalloping between roots on xray
characteristic simple bone cyst
183
simple bone cyst assoc with other lesions
florid COD
184
what can florid COD be associated with
simple bone cysts
185
anurysmal bone cyst genetics
17;16 translocation
186
17;16 translocation
aneurysmal bone cyst
187
aneurysmal bone cyst age
teenagers
188
ballooning or blow-out distortion of bone contour
aneurysmal bone cyst
189
blood soaked sponge
aneurysmal bone cyst
190
aneurysmal bone cyst histo
spaces filled with blood, cellular fibroblastic stroma, lacelike calcifications
191
spaces filled with blood, cellular fibroblastic stroma, lacelike calcifications
aneurysmal bone cyst
192
other lesions often associated with aneurysmal bone cyst
CGCG or BFOLs
193
fibrous dysplasia genetics
GNAS1 mutation
194
GNAS1 mutation
fibrous dysplasia
195
fibrous dysplasia mechanism
replacement of bone by connective tissue
196
fibrous dysplasia more commonly mono or poly
80% are mono
197
fibrous dysplasia radio look
ground glass
198
ground glass radio
fibrous dysplasia
199
polyostotic FD + cafe au lait
Jaffe Lichtenstein
200
polyostotic FD + cafe au lait + endocrine
McCune Alrbight
201
possible endocrine disturbances in McCune albright
sexual precocity, putiutary adnoma, hyperPTH
202
polyostotic FD + intramuscular myxomas
Mazabraud syndrome (increased risk of osteosarc)
203
Mazabraud syndrome presentation and risks
polyostotic FD + intramuscular myxomas | increased risk of osteosarc
204
which polyostotic FD higher risk of osteosarc
Mazabraud
205
hockey stick deformity
fibrous dysplasia; leg length discrepancy due to involvement of upper femur
206
electrolytes in fibrous dysplasia and why
renal phosphate wasting (bone produces FGF23) --> hypophosphatemia
207
cafe au lait in fibrous dysplasia descripror -- vs what other?
coast of Maine vs coast of Cali in NF
208
chinese characters histo
fibrous dysplasia
209
bone trabeculae in fibrous dysplasia
monotonous -- chinese characters
210
most common BFOL
cemento-osseous dysplasia
211
COD vs ossifying fibroma: | sx, histo, hemorrhage
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
dental considerations with CODs
prone to necrosis (avoid bx and exo) and osteomyleitis
213
familial gigantiform cementoma inheritance, demographics, age, extent
AD, whites, first decade, all four quads
214
familial gigantiform cementoma most similar to what other BFOL
florid COD (histo also); ultimately leads to massive sclrotic masses of disorganized mineralized material
215
familial gigantiform cementoma labs
increased serum alk phos (declines after surgical removal); anemia -- GYN exam for polypoid adenomas
216
ossifying fibroma genetics
HRPT2 parafibromin
217
parafibromin
ossifying fibroma HRPT2
218
HRPT2
parafibromin ossifying fibroma;; also in hyperPTH-jaw tumor syndrome
219
ossifying fibromas characteristic radio
downward bowing of md cortex
220
downward bowing of md cortex
ossifying fibroma
221
hyperPTH jaw tumor syndrome
HRPT2 mutation; patathyroid tumors, OF of jaws, renal cysts, Wilms tumor
222
ossifying fibroma syndrome association
hyperPTH jaw tumor syndrome | HRPT2 mutation; patathyroid tumors, OF of jaws, renal cysts, Wilms tumor
223
ossifying fibroma histo
hard tissue may be osteoid, bone, or cementum like (vs more uniform in fibrous dysplasia)
224
how is juvenile ossifying fibroma different from regular
age of patient, site of involvement, clinical behavior == males, Mx, rapid growth
225
types of juvenile ossifying fibromas
trabecular and psammomatoid
226
psammomatoid juvenile ossifying fibroma genetics
x;2 translocation
227
x;2 translocation
psammomatoid juvenile ossifying fibroma
228
trabecular vs psammomatoid juvenile ossifying fibroma
trabecular younger patients; psammomatoid 4x more common; 70% extragnathic (orbit, frontal bone and paranasal sinuses)
229
juvenile ossifying fibroma can be associated with what other entity
aneurysmal bone cysts
230
osteoma types-locations
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
condylar osteoma causes clin and ddx
shift of chin; ddx condylar hyperplasia, hemifacial hyperplasia
232
osteoma xray
periosteal - sclerotic mass; endosteal -- similar to idiopathic osteosclerosis
233
osteoma histo types
compact - dense bone with minimal marrow; cancellous -- bone trabeculae and marrow
234
gardner syndrome genetics
APC (adenomatous polyposis coli)
235
do gardners polyps transform
colorectal yes almost 100%; but not small intestine/stomacj
236
gardner findings other than polyps and where
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
epidermoid cysts and osteomas
gardner
238
osteblastoma vs osteoid osteoma difference
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
aggressive osteoblastoma aka and how different
``` aka epithelioid; atypical histo (similar to well diff osteosarc), aggressive behavior, >4cm. >30yo, pain ```
240
osteoid osteoma and osteoblastoma histo
reversal lines, blue bone, bland, plump, active osteoblasts
241
cementoblastoma location, age, symptoms, radio
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
most severe osteogenesis imperfecta
2 most sever --> death; 3 most severe in adults
243
adult osteopterosis aka and forms
benign osteopetrosis -- one form with nerve compression, two with fractures
244
intermediate osteopetrosis what is?
less severe from of infantile disease --> resolves spontaneously
245
CGCG areas in what other lesions
ABC and central odontogenic fibroma
246
ABC areas in what other lesions
OF or CGCG
247
ollier vs maffucci
ollier -- multiple chondromas, usually unilateral; maffucci -- skeletal chondromatosis and soft tissue angiomas
248
chondromyxoid fibroma genetics
chromosome 6 abnormality
249
chromosome 6 abnormality
chondromyxoid fibroma
250
synovial chondromatosis aka and what is
chondrometaplasia; metaplastic cartilage in synovial membrane
251
desmoplastic fibroma syndomr
tuberous sclerosis
252
codman triangle
periosteal triangular elevation in osteosarc
253
types of juxtacortical osteosarc
aka peripheral; parosteal (in periosteum) and periosteal (at interface with cortex)
254
postradiation sarcomas what are
50% osteosarc; 40% fibrosarc
255
ewing translocation
11;22
256
11;22 translocation
ewing
257
metastatic to bone most common
breast, lung, thyroid, prostate, kidney most common primary
258
chondroma locations
most in short bones of hands and feet
259
chondromas in jaws arise from
cartilaginous rests -- anterior maxilla, condyle symphysis, coronoid process
260
ollier disease vs maffucci syndrome
ollier: multiple chondromas, usually unilateral maffucci: multiple chondromas and hemangiomas; phleboliths; skin macules
261
chondromyxoid fibromas genetics and location | demographics and symptoms
chromosome 6 abnormality rare in jaws, usually long bones usually <30yo, Md, pain and swelling
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chromosome 6 abnormality
chondromyxoid fibromas
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chondromyxoid fibroma histo
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
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
chondromyxoid fibroma
265
synovial chondromatosis what is and where and why
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
metaplstic nodule of cartilage in synovial membrane
synovial chondromatosis
267
stages of synovial chondromatosis
1- foci in synovial lining 2 - foci increase and detach, material found in synovial membrane and joint 3 - cartilage only in joint
268
synovial chondromatosis radio finding
loose bodies -- round irregular radopacities in joint region on xray although this is not specific
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loose bodies on radio
synovial chondromatosis although not specific
270
synovial chondromatosis histo
nodules of cartilage within synovium and loose in joint space; may ossify
271
nodules of cartilage within synovium and loose in joint space
synovial chondromatosis
272
bone counterpart of fibromatosis and syndrome
desmoplastic fibroma; sometimes assoc with tuberous sclerosis
273
desmoplastic fibroma is bony variant of
fibromatosis
274
desmoplastic fibroma location
posterior md (molar and angle-ascending
275
desmoplastic fibroma histo
small fibroblasts and abundant collagen
276
most common non hematopoietic malignancy of bone
osteosarc
277
osteosarc age
bimodal: 10-20yo (period of bone growth) and 50+ (pagets or history of radiation) jaw osteosarc 33 yo (vs 23yo long bones)
278
gnathic osteosarc location
mx = md
279
gnathic osteosarc radio findings
spiking f roots, sunburst, symmetrical PDL widening (tumor infiltration)
280
codman triangle
periosteal elevation in osteosarc
281
causes of secondary osteosarc
irradiation to bone, fibrous dysplasia, Pagets, bone infarct
282
long bone osteosarc: gender, age, spread
M, <20yo, hematogenous
283
types and aka of peripheral osteosarc
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
parosteal vs periosteal -- which is higher grade
parosteal is low grade, periosteal is mid grade
285
which osteosarc pedunculated, no elevation of periosteum. spindle cells + bone, low grade
parosteal
286
which osteosarc sessile, elevated periosteum, primitive sarcoma with chondroid and osteoid, mid grade
periosteal
287
postirradiation sarcoma, how soon, what dose, what types
average 14y after radiation, >7000cGy | 50% osteosarc, 40% fibrosarc
288
chondrosarc frequency, location, symptoms, age
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
chondorsarcs of head and neck location
rare in jaws; mx | 30% are extraosseous (larynx or soft tissue)
290
chondrosarc histo
often lobular pattern, periphery of lobules more immature
291
grades of chondrosarc
1 - similar to chondroma but look for binucleated chondrocytes 2 - increased cellularity, matrix is myxoid 3 0 highly cellular with spindle cells
292
chondrosarc histo types
well diff, mesenchymal, clear cell, periosteal, and dedifferentiated (well diff + fibrosarc)
293
mesenchymal chondrosarc frequency. age
10% of all chondrosarcs | most common in jaws; younger patients
294
mesenchynal chondrosarc histo
biphasic: spindle/round undff cells with HPC like areas (staghorn vessels) cartilagenous component variable, usually low grade and well demarcated
295
mesenchymal chondrosar IHC
cells are CD99+, cartilagenous component S100+ | HEY1-NCOA2 transloc
296
chordoma origin
malignant tumor that recapitulates notochord -- arises from its remntnats
297
chordoma categories -- which is most common
spheno occipital, sacro coccygeal (most common), vertebral
298
chordoma in skull location and symptoms
clivus and parasellar most common location, usually in midline symptoms: headache and diplopia -- 6th nerve palsy
299
tumor in clivus or parasellar, usually midline, with headache and diplopia 2/2 6th nerve palsy
chordoma
300
chordoma histo
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
physaliferous cells
chordoma! | large cells with central nuclei and vacuolated and reticulated cytoplasm
302
large cells with central nuclei and vacuolated and reticulated cytoplasm in chordoma
physaliferous
303
chondroid chordoma
contains hyaline type cartilaginous tissue with tumor cells in lacunae
304
chordoma IHC
CK. EMA, S100, NSE+
305
most common jaw cancer
metastatic carcinoma
306
most common primaries for jaw mets
breat lung thyroid prostate kidney
307
jaw mets location
80% to mandible
308
numb chin syndrome
loss of sensation in lower lip and chin, common in jaw mets
309
loss of sensation in lower lip and chin
numb chin syndrome, common in jaw mets

OP - Yield (19 decks)

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