bones, joints, & soft tissue tomors Flashcards
(205 cards)
1
Q
What is Dysostosis?
A
developmental anomaly of bone
- abnormal mesenchymal migration
- defective ossification of fetal cartilage
- sporadic or part of syndrome
- homebox gene alterations
2
Q
Types of Dysostosis?
A
Aplasia, supernumerary digits, abnormal fusion of bones
3
Q
What is Dysplasia?
A
mutations interfere with growth or homeostasis (dwarfism)
4
Q
Bone dysplasia is called?
A
osteodysplasia
5
Q
Cartilage dysplasia is called?
A
chondrodysplasia
6
Q
Klippel-Feil syndrome
A
Sprengel’s deformity is always associated with it.
Congenital fusion of any 2 cervical vertebrae
7
Q
Osteogenesis imperfecta aka….
A
brittle bone disease
8
Q
Osteogenesis imperfecta is a mutation of what?
A
type I collagen
- austosomal dominant
- premature breakdown of collagen
9
Q
In Osteogenesis imperfecta the ECM is affected where?
A
eyes, inner ear bones, skin, joints, teeth
10
Q
Range in severity in Osteogenesis imperfecta?
A
Type I: normal lifespan
Type II: lethal in utero (cerebral hemorrhage, respiratory failure)
11
Q
MC cause of dwarfism
A
Achondroplasia
12
Q
Achondroplasia mutation
A
(FGFR3) Fibroblast growth factor receptor
- inhibits epiphyseal chondrocytes
13
Q
Is Achondroplasia spontaneous or genetic
A
75% spontaneous
25% autosomal dominant
14
Q
What are the bone-forming tumors?
A
Osteoma, osteoid osteoma and osteoblastoma, osteosarcoma
15
Q
MC on the head and skull
A
Osteoma
16
Q
Common age range for osteomas
A
40-50 years old
17
Q
What type of growth does osteomas have
A
Exophyotic
18
Q
Common age and gender occurrence for osteoid osteomas and osteoblastomas
A
Males are 2x more likely.
10-20 years old
19
Q
Common location and length of osteoid osteomas
A
Femur and <2cm
20
Q
What bone-forming tumor produces nocturnal pain
A
Osteoid osteomas
21
Q
What tumor pain is relieved by aspirin
A
Osteoid osteomas
22
Q
Common location and length of osteoblastomas
A
Vertebrae and 2-6cm
23
Q
Is osteoblastomas localized
A
No
24
Q
Is osteoblastoma pain relieved by aspirin
A
No
25
What is the MC primary bone CA
Osteosarcoma
26
Types of osteosarcomas
Primary and secondary
27
What gender/location is common in primary osteosarcomas
Males 75%.
| <20 years old
28
Common location of primary osteosarcomas
Knee
29
Treatment for primary osteosarcomas
Amputation
30
Type of growth associated with primary osteosarcomas
Periosteal reaction. "Sunburst"
31
Codman triangle is associated with what bone tumor
Osteosarcoma
32
MC Age and location of occurrence for secondary osteosarcomas
>40, pelvis
33
10-20% of secondary osteosarcomas metastasize where?
To the lungs
34
T/F osteosarcomas are really aggressive and are fatal
True
35
Osteosarcomas are usually secondary to what disease?
Paget's disease
36
Changes with Achondroplasia
short proximal extremities, lower extremity, frontal bossing, midface hypoplasia
37
Spinal Achondroplasia
hyper lordosis and kyphosis at birth, bullet vertebrae, scolosis, spinal stenosis.
- foramen magnum stenosis
- Death: BRAIN STEM COMPRESSION
38
Thanatophoric dwarfism
Fatal.
1: 20,000 births
extremely small thorax and shortened long bones
39
Trident hand is associated with
Achondroplasia
40
Osteopetrosis
a group of rare genetic disorders characterized by defective osteoclast-mediated bone resorption.
41
In Osteopetrosis the patient usually has Foraminal Stenosis which causes?
cranial nerve palsies
42
In Osteopetrosis the patients medullary cavity fills which causes?
deranged hematopoiesis
- hepatosplenomegaly
- recurrent infections, anemia
43
Two characteristics of Osteopetrosis
No medulla.
| Erlenmeyer flask deformity.
44
Osteoporosis
an aquired condition characterized by reduced bone mass, and increase porosity
45
Categories of Osteoporosis
1. localized
| 2. generalized to entire skeleton
46
Primary generalized Osteoporosis
MC type: senile, postmenopausal
47
Secondary generalized Osteoporosis
Various causes
- neoplasia, hyperparathyroidism
- nutrient deficiency
- drug exposure: corticosteroids (alcohol, smoking)
48
Osteoporosis MC impacts what type of bone?
trabecular bone (cancellous, spongy)
49
What are the cartilage-forming tumors
Osteochondroma, chondroma aka endochondroma, chondrosarcoma
50
MC gender and age associated with osteochondromas
Males 3x more likely. 10-30 years old
51
MC location of osteochondromas
Knee..... Can be from 1-20cm
52
Are osteochondromas sessile or pedunculated
Both, but MC sessile
53
T/F Endochondromas are in the medullary cavity
True
54
Common age range of endochondromas
20-50 years old
55
Where are solitary lesions located in endochondromas
Metaphysis
56
Multiple lesions in endochondromas are called
Ollier disease
57
MC location of endochondromas
Hands and feet. MC proximal phalanges
58
"O ring sign" is associated with which tumor
Endochondromas
59
MC location of chondrosarcomas
Intermedullary of the girdles of the body
60
Age and gender related to chondrosarcomas
Males 2x, 40-60 years old
61
"Glistening mass" is used to describe what tumor type
Chondrosarcomas
62
Types of chondrosarcomas
Low grade and high grade
63
Which type is MC
Low grade
64
Is low grade chondrosarcomas slow or fast growing
Slow
65
Survival rate for low grade chondrosarcomas
80-90% 5 year survival
66
What % of chondrosarcomas metastasize to the lung
70%
67
An increase in radiolucency means and increase in what
Aggressiveness
68
Are fibrous cortical defect and nonossifying fibroma neoplastic?
No
69
MC location of fibrous cortical defect and nonossifying fibroma
knee.
| MC distal femur, and proximal tibia
70
fibrous cortical defect and nonossifying fibroma are MC asymptomatic or symptomatic
asymptomatic
71
Do fibrous cortical defect and nonossifying fibroma self resolve usually?
yes, in 2-3 years
72
Fibrous Dysplasia
failure of bony differentiation.
| well-circumscribed, intramedullary
73
Types of Fibrous Dysplasia
Monostotic, polyostotic, MCcane Albright syndrome
74
Monostotic Fibrous Dysplasia
single bone involvement
75
MC age and location of Monostotic Fibrous Dysplasia
RIbs and 10-30 years old
76
Does Monostotic Fibrous Dysplasia have any distortion?
minimal distortion
77
Polyostotic Fibrous Dysplasia
multiple bone envolvement
78
Age an MC location of Polyostotic Fibrous Dysplasia
late childhood/adolescence. Femur (MC)
79
Does Polyostotic Fibrous Dysplasia have craniofacial envolvement
yes 50% of the time
80
Classic appearance of Polyostotic Fibrous Dysplasia
"ground glass"
81
Major features of MCcane Albright syndrome
precocious puberty, cafe au lait spots, endocrine HYPER function
82
Ewing sarcoma and primitive neuroectodermal tumor are what types of tumors
malignant
83
MC age, ethnicity, gender of Ewing sarcoma and primitive neuroectodermal tumors
10-20 yrs old, white(9x) males
84
MC location of Ewing sarcoma and primitive neuroectodermal tumors
Long bone diaphysis
| MC femur, pelvis
85
Are Ewing sarcoma and primitive neuroectodermal tumors painful?
yes
86
Ewing sarcoma and primitive neuroectodermal tumo mimics what infections
pyrexia, leukocytosis, increase ESR
87
Structural characteristics of Ewing sarcoma and primitive neuroectodermal tumors
"onion-skinning"
88
5 year survival rate of Ewing sarcoma and primitive neuroectodermal tumors
75%
89
Which one is undifferentiated Ewing sarcoma or primitive neuroectodermal tumor
Ewing Sarcoma
90
What is primitive neuroectodermal tumor
neurally differentiated
91
What is the MC pediatric bone CA
osteosarcoma
92
What is the 2nd MC pediatric bone CA
Ewing sarcoma
93
Ewing sarcoma is what % of primary bone CA
10%
94
Characteristic of primitive neuroectodermal tumor
Homer-Wright rosettes
95
Giant-cell tumors of bone are multinucleated giant cells and considered ___________ like.
osteoclast
96
Positioning of Giant-cell tumors of bone
large, lytic, eccentric
97
MC location of Giant-cell tumors of bone
knee
98
what part of bone do Giant-cell tumors of bone affect
epiphysis/metaphysis
99
are Giant-cell tumors of bone painful?
YES. Arthritis like pain
100
2% of Giant-cell tumors of bone metastasize where
lungs
101
Structural characteristics of Giant-cell tumors of bone
"soap bubble" appearance, thin "shell" of cortex
102
MC age of Giant-cell tumors of bone
20-40 years old
103
What is MC, primary or secondary bone CA
Secondary
104
Mechanisms for the spread for secondary bone CA
1. Direct extension
2. Circulation
3. Infra spinal seeding
105
Sarcomas metastasize how?
Hematogenous
106
Carcinomas metastasize how?
Lymphatics
107
Secondary met to bone is usually lytic or blastic?
Both
108
In adult metastases usually originates from where?
Prostate (blastic)
Breast (lytic)
Lung
109
What are the common sources of bony metastasis in children?
Neuroblastoma, Wilms tumor, osteosarcoma, Ewing sarcoma, rhabfomuosarcoma
110
What is the MC joint disorder?
OA
111
What is the fundamental feature of OA
Degeneration of the articular cartilage.
Decrease in proteoglycans = matrix breakdown
112
Is OA inflammatory?
No
113
What are the secondary changes of OA
Subchondral changes
114
Types of OA
Primary and secondary
115
Primary OA
Insidious onset. No trauma and usually age related
116
Secondary OA
Predisposing injury or deformity is usually the cause.
Trauma (obesity), deformity, systemic disease
117
Secondary OA typically occurs in adults or children
5% of children
118
MC location of OA in females
Knees and hands
119
MC location of OA in men
Hope
120
Steps in OA
1. Exposed subchondral bone, eburnation***
2. Subchondral cyst
3. Residual articular cartilage
121
Heberdens nodes are associated with which type of arthritis
OA
122
What type of disease is RA
Systemic, chronic autoimmune disease
123
MC location of RA
MCP and PIP JOINTS. (Bouchards nodes)
124
Pannus is associated with which type of arthritis
RA
125
Structural characteristics of RA
Ulnar deviation and swan-neck deformity
126
MC gender and age for RA
Females (3-5x)
| Age 10-40
127
Which type of arthritis can have possible ankylosis
RA
128
What type of disease is juvenile rheumatoid arthritis
Autoimmune
129
What joints are affected by JRA
Large joints
130
Common age for JRA
<16, MC 1-6
131
How long to patients with JRA have symptoms
At least 6 weeks
132
Does seronegative spondyloarthropathies have rheumatoid factor?
No
133
HLA-B27 is associated with what disease
Seronegative spondyloarthropathies
134
Clinical entities of seronegative spondyloarthropathies
```
"P.E.A.R."
Psoriatic arthritis
Enteropathic arthritis
Ankylosing spondylitis
Reiters syndrome (reactive arthritis)
```
135
What is another name for ankylosing spondylitis
Marie-strumpell disease
136
MC location of AS
Axial skeleton: inflammation and ankylosis
137
Onset of AS
<40 year olds MC males
138
Characteristics of AS
Morning stiffness: > 30 minutes
| Nocturnal LBP
139
95% of AS are diagnosed with the presence of what
+HLA-B27
140
Gout affects what percent of the population
< 1% and MC males over the age of 30
141
What causes gout
Excessive amounts of Utica acid, and an end product of purine metabolism, within tissues and body fluids
142
Gout is usually marked by what?
Recurrent episodes of acute arthritis, sometimes accompanied by large crystalline aggregates (tophi)
143
Categories of gout
Primary (unknown cause)
| Secondary ( co-morbid disease or drug exposure)
144
Metabolic defect with primary gout
Hyperuricemia
145
Common causes of secondary gout
Leukemia, renal disease, lead poisoning
146
MC location of gout
Hallux (mtp) = podagra
147
Pseudogout is aka
CPPD**
| Calcium pyrophosphate deposition disease
148
MC AGE for pseudogout
>50
149
MC location of Pseudogout
Knee, wrist, shoulder, elbow, ankle
150
Does Pseudogout present inflammation
Yes with 50% destruction
151
Pseudogout is MC sporadic or inherited
Sporadic
152
Altered pyrophosphate metabolism is associated with what disease
Pseudogout
153
Hydroxyapatite deposition disease is aka
Calcific tendonitis
154
What happens in HADD
Hydroxyapatite accumulates in tendons
155
MC location of HADD
Rotator cuff muscles
156
MC microbe involved in infections arthritis
Bacterial
157
How is microbe spread to the joint in infectious arthritis
Hematogenous, trauma, local pathology (osteomyelitis)
158
Two types of infectious arthritis
Suppurative arthritis and Lyme arthritis
159
When a bacteremia seeds to joints it creates a pus forming arthritis called
Suppurative
160
MC location of suppurative arthritis
Knee
161
What bacteria is MC cause of suppurative arthritis in children younger than 2
Haemophilus influenzae
162
What bacteria is MC cause of suppurative arthritis in children older than 2 and adults
Staph aureus
163
What bacteria is MC cause of suppurative arthritis in adolescents and young adults
Neisseria gonorrhae
164
What bacteria is MC related to sickle cell disease
Salmonella spp.
165
Least aggressive bacteria. Subacute course
Neisseria gonorrhoea
166
Lyme arthritis occurs in what percent of untreated Lyme disease cases
60-80%
167
Primary joint neoplasms are rare and MC
Benign
168
T-F tumor like neoplasms are MC than neoplasia
T
169
What is a ganglion
A small myoxiod cyst
170
Location of a ganglion
Wrist: joint capsule/ tendon sheath
171
Ganglion are commonly asymptomatic
True
172
Treatment of ganglions
"Bible therapy"
173
What is a synovial cyst
Benign, synovial herniation through joint capsule
174
What is a bakers cyst
A synovial cyst in the popliteal fossa
175
What are tenosynovial giant cell tumors
Group of benign synovial neoplasms. MC in ages 20-50
176
Is Diffuse TGCT or localized TGCT pain related
Diffuse
177
MC location of diffuse TGCT
Knee
178
TGCT Of tendon sheath
Localized TGCT
179
Soft tissue tumors are MC benign or malignant
Benign (100x) > malignant
180
Soft tissue Sarcomas are what % of diagnosed CA
<1%
181
Location of soft tissue tumors
Any location but MC thighs
182
MC soft tissue tumor in adults
Lipoma
183
Lipoma
Benign tumor of adipocytes
184
Liposaecomas
Malignant tumor of adipocytes
185
Nodular fasciitis
Reactive fibroblastic mass on the palm side of the arm, chest, back. Non neoplastic
186
Myositis ossificans
Reactive metaplastic bone.
| MC in proximal muscle bellies
187
Fibromatosis
Benign locally invasive fibroblastic proliferation. Commonly reoccurs after removal
188
Superficial Fibromatosis
Affect superficial fascia and cause
| Dupuytren contracture and peyronie disease
189
Deep Fibromatosis
MC abdominal. Aggressive, locally destructive and reoccur. Cause Gardner syndrome
190
Gardner syndrome is caused by what gene mutation
APC.
Colon polyps and multiple osteomas are related to this disease
191
Fibrosarcomas have what kind of pattern
Herringbone pattern
192
In fibrosarcomas 25% hematogenous metastasize where
Lung
193
What % of fibrosarcomas recur after excision
50%
194
Virtually all skeletal muscle tumors are benign or malignant
Malignant
195
MC pediatric soft tissue sarcoma
Rhabdomyosarcoma
196
Rhabdomyosarcomas MC occur where
Areas with minimal skeletal muscle
197
T/f two thirds of Rhabdomyosarcomas are cured in kids
True. Adults have a poor prognosis
198
Benign smooth muscle tumors are called
Leiomyomas
199
Leiomyomas are MC where
Uterus (MC "fibroids")
200
Malignant smooth muscle tumors
Leiomyosarcomas
201
Leiomyosarcomas are MC in
Adult female
202
Signature histological shape of Leiomyosarcomas
Cigar shaped
203
Location of synovial sarcomas
<10% are intra-articular. Deep soft tissues near large extremity joints.
MC in knee 60-70%
204
Are synovial sarcomas painful
No
205
MC age for synovial sarcomas
20-40 years old
