Non Neo/Neo Bone Flashcards
1
Q
What is achondroplasia?
A
Autosomal dominant dwarfism
Caused by FGFR3 mutation
Inhibits cartilage proliferation
2
Q
What is osteogenesis imperfecta?
A
Due to mutations that encode TYPE I collagen subunits
3
Q
What is osteomyelitis?
A
Broadly divided into pyogenic and granulomatous histologies
4
Q
What causes pyogenic osteomyelitis?
A
Staph. aureus
5
Q
What causes pyogenic osteomyelitis in neonates?
A
Group B strep, Haemophilus influ
6
Q
What causes pyogenic osteomyelitis in sickle cell patients?
A
Salmonella
7
Q
What are the biggest causes of osteoporosis?
A
Post menopause and senile
8
Q
What is rickets and osteromalacia?
A
Rickets is the children form, osteomalacia is the adult form
Causes defect in BONE MINERALIZATION due to vit D deficiency
9
Q
What causes secondary osteoporosis?
A
Hyperparathyroidism and Chronic renal disease
10
Q
What causes a callus?
A
A healing fracture
11
Q
What may cause Paget disease of bone?
A
Viral infection
Goes through a lytic, mixed, and sclerotic phase
Mosaic pattern of lamellar bone
Sarcoma is the dreaded complication
12
Q
What is fibrous dysplasia?
A
A developmental abnormality of bone
Makes a mass
Monostotic: ONe bone
Polystatic; mutliple bones
Hisotlogy i: Trabeculae of woven bone withou osteoblastic rimming
13
Q
What is McCune-Albright syndrome?
A
GNAS1 mutation
Polyostatic endocrinopathy w/ CAFE au LAIT spots
14
Q
What causes osteoarthritis?
A
Breakdown of articular cartilage
Due to cytokine production
Results in secondary changes, burnishing of bone
subchondral cysts
Osteophytes
15
Q
What causes osteoarthritis?
A
Breakdown of articular cartilage
Due to cytokine production
Results in secondary changes, burnishing of bone
subchondral cysts
Osteophytes
16
Q
What is rheumatoid arthritis?
A
Systemic autoimmune disease
Symmetrical arthritis
Involves multiple joints, especially in hands
17
Q
What are characterisitic pathology features of rheumatoid arthritis?
A
Destructive
Inflammatory synovitis (Pannus)
Necrotizing granulomas (rheumatoid nodules
18
Q
What is rheumatoid factor?
A
Involved in rheumatoid arthritis
IgM directed against Fc portion of IgG
19
Q
What is Gout?
A
Imbalance of urate production and excretion
Caused by monosodium urate crystals
20
Q
What are monosodium urate crystals?
A
Needle-shaped that are negatively birefringent under polarized light
Turn yellow in parallel light
21
Q
When in peak bone mass?
A
20-40
Slow decrease after especially in women
22
Q
What stimulates osteoclasts?
A
Osteoblasts
23
Q
What is thanatotropic dwarfism?
A
Lethal form of dwarfism due to two copies of FGFR3 mutation
24
Q
What is a type I collagen disease?
A
Osteogenesis imperfecta
Mutation in a1 and a2 chain of collagen type I
AD
25
What are the phenotypes of Osteogenesis imperfecta?
Brittle bones/ BLUE SCLERA
Lethal in utero
26
What is osteomyelitis?
Inflammation of bone marrow and bone
Usually infectious
Inflammation is pyogenic or granulomatous
27
What are the hematogenous spread osteomyelitis?
Long bones in children
Vertebra in adults
28
What is direct extension osteomyelitis?
Jaws or Skulls from dental or sinus infection
Toes/feet from diabetic foot ulcers
29
What are the pathogens involved in osteomyelitis?
S. aurues (90%)
Group B strep and Haemophilus in NEONATES
Pseudomonas (IVDU)
Mycobac (Elderly, debilitated, immunocompromised)
Salmonella (Sickle Cell patients)
30
What is seen in histo for chronic osteomyelitis?
Fibrosis and plasma cell infiltration in bone marrow
31
What is seen in tuberculosis osteomyelitis?
Necrotizing granulomatous inflammation
32
What is osteoporosis?
Age related changes
Reduced physical activity
Genetic factors
Nutritional factors
Hormonal influences
33
What are the hormonal influences of osteoporosis?
Estrogen def leads to acc bone loss
Lifetime risk 1 to 2 in @
1 in 40 in MEN
34
What is the mechanism of loss of bone mass in post menopause?
Decreased estrogens causes increased IL-1 which increases osteoclast activity
35
What is secondary osteoporosis?
Rickets (child) osteomalacia (adults)
Defect in mineralization
Due to def in vit D
Hyperparathyroidism: Increased PTH, detected by osteoblasts to stimulate osteoclast activity
Chronic Renal Disease: Hyperphosphatemia causes hyperparathyroidism
36
What is brown tumor of bone?
Aggregates of osteoclasts and spindled stroma
37
How does a fracture repair itself?
Procallus: Organization of hematoma along fracture line
Fibrocartilaginous Callus: Ingrowth of mesenchymal cells
-Bridge gap and differentiate towards cartilage
Osteous callus: Cartilage undergoes osssification to form bony trabeculae
38
What is paget disease of bone?
Collage of matrix madness
Three phases:
- Osteolytic
- Mixed osteoclastic-osteoblastic
- Osteosclerotic
39
What is the etiology of paget disease?
Virus infection by paramyxovirus
40
When does paget disease appear?
After age 40
Pain
Elevated alk phos
Polyostotic 85%
SErious complication is osteosarcoma
41
When does paget disease appear?
After age 40
Pain
Elevated alk phos
Polyostotic 85%
SErious complication is osteosarcoma
42
What is seen on X ray for the sclerotic phase?
Irregular thickening of bone (cortical and trabecular)
43
How is the mosaic pattern of bone easily seen?
Seen best under polarized light
44
What is fibrous dysplasia?
Benign tumor
Three patterns:
- Monostotic 70%: Children, arrests at puberty, impinge on nerves, result in path fx, femur, ribs, jaw most common
Polyostotic without endocrine dysfunction: 27%
McCume-Albright Syndrome: Polyostotic involvement with Coast of Maine cafe-au-lait spots and endocrinopathies
3% due to somatic GNAS1 mutation
Histology: Curvilinear trabeculae of woven bone
45
What is the characteritic of McCune-Albright Syndrome?
Histology is chinese character like
46
What is the most common joint disease?
Osteoarthritis
Wear and tear
80% of people >70
Generally oligoarticular
Heberden nodes (DIP)
Secondary disease occurs due to prveious trauma or congenital deformity
47
What is the pathogenesis of osteoarthritis?
Chondrocytes produce (IL-1/TNF-a) that induce production of metalloproteinases and inhibit collagen and proteoglycan synthesis
48
What is Rheumatoid Arthritis?
Chronic systemic autoimmune disorder
Polyradicular
Severe form of chronic synovitis with secondary destruction of tisue
Presents with PIP or MCP joint involvement
NO DIP
49
What is pathogenesis of Rh arthritis?
Proliferative inflamed synovium (pannus)
25% have extra articular presentation, subQ or Visceral nodules of palisading granulomas w/ fibrinoid necrosis
80% have circulating Rh factor (IgM against Fc of IgG)
50
How do you make the dx of Rh arthritis?
Morning stiffness, arth of >3 joints, Rh nodules, RF, typical radiology (4 of 7)
51
What is the joint deformity of RA?
Ulnar deviation
52
What is gout secondarily caused by?
Leukemia (tumor lysis syndrome), chronic renal disease, Lesch-Nyhan syndrome (HGPRT def)
53
What is a gouty tophus?
A foreign body composed of many giant cells
54
Where do most primary bone tumors arise?
Metaphyses
55
What are osteomas?
Proliferation dense, cortical bone, affecting facial/skull bones
Dx based on radiological finding
56
What are osteoid osteomas?
Pediatric tumor
Small size
57
What are osteoid osteomas?
Pediatric tumor
Small size
58
What is a osteoblastoma?
A osteoid osteoma that is big >2cm
Found in the vertebrae
Produce different pain
NOT RESPONSIVE TO NSAIDS
59
What is a osteosarcoma?
A malignant mesenchymal tumor
Production of malignant osteoid
Predominates in children
Adults if had Paget disease before
60
How do you treat osteosarcoma?
Children: Neoadjuvant chemo (doxorubcine, vincristine,
Resect the tumor (limb sparing or radical)
Responder is 90% necrosis, has good prognosis
If not a responder has a poor prognosis
61
What is an endochondrom?
A benign cartilagenous tumor
Small size, circumscription, tendency to invovle tubular bones
62
What is a osteochondroma?
Bony outgrowth with cartilagenous cap
Cap rarely undergoes mal transformation to chondrosarcoma
63
What is a chondroblastoma?
Benign
Young person
Epiphyseal based
Chicken wire histo
64
What is chondrosarcoma?
Adult cartilage forming sarcoma
Permeative growth
Observance is well-differentiated grade I
65
What is a de differentiated chondrosarcoma?
Abrupt transformation of well diff to a high grade sarcoma
66
What is a giant cell tumor of bone?
A large destructive EPIPHYSEAL based tumor of ADULTS
Mononuclear cells mixed with osteoclast like giant cells
Lesions are treated by curretage but RECUR commonly
67
WHat is a Ewing sarcoma?
EWSR1 and ETS mutation
Diffuse, strong membranous CD99 (MIC2) expression is diagnosis
Use multiagent neoadjuvant approach that results in cure of 2/3 cases
68
What is the most common cause of cancer in bone?
METS
69
What mets are osteoblastic?
Prostate and Breast
70
What mets are mixed osteoblast/lytic?
Lung
71
What mets are lytic?
Kidney, thyroid
72
What mets are lytic?
Kidney, thyroid
73
What is punctate calcification?
Cartilage
74
What is diffuse cloud like calcification?
Osteoid
75
What are benign features?
Sclerotic rim
Lack of cortical bone destruction
Lack of soft tissue extension
Lack of periosteal reaction
76
What are mal features?
Ill defined
Infiltrative borders
77
What are osteoid producing primary tumors?
Osteoma
Osteoid Osteoma
Osteoblastoma
Osteosarcoma
78
What are the features of a osteoid osteoma?
75% of patients are
79
What is seen on xray for a osteoid osteoma?
A cortical, well-circumscribed, radiolucent lesion
80
What is seen in histo?
A nidus of osteoid that is surrouded by dense reactive rim of bony tissue
81
What is seen in histo?
A nidus of osteoid that is surrouded by dense reactive rim of bony tissue
NIDUS: woven bone with osteoblastic rimming
82
What is a osteoblastoma?
A large osteoid osteoma in vertebra most typically
>2cm
No relief with NSAIDS
DULL pain
83
What is a huge relative risk to developing osteosarcoma?
Hereidtary retinoblastoma
84
What is the radiology of a osteosarcoma?
Admixed radiolucent and radiodense lesion
Cortical destruction
Periosteal reaction
Sunburst, codman's triangle
Cumulus appearance
85
What is the histo of osteosarcoma/
High grade mal tumor with atypical osteoblasts
Lay down malignant osteoid
Multiple subtypes
Osteoblastic, chondroblstic, fibroblastic, telangiectatic
86
What is the treatment for osteosarcoma?
Neoadjuvant chemo (doxorubicin, cisplastin, HD methotrexate)
FOllowed by radical surgery
Limb salvage if can
Adults may receive post op chemo
87
Where is the most common mets of osteosarcoma?
To lung but can pluck tumor nodules if it does
88
Where does an osteosarcoma grow on histo?
Tends to grow on a scaffold of preexisting trabeculae
Lacy osteoid wtih atypical cells embedded
89
What is an endochondroma?
Most common intraosseous cartilage tumor
Tubular bones
Hand
Peak incidence 3-5 decade, M=F
Lucent lesions with sclerotic
90
What is an endochondroma?
Most common intraosseous cartilage tumor
Tubular bones
Hand
Peak incidence 3-5 decade, M=F
Lucent lesions with sclerotic
91
What is the treatment for an endochondroma?
Curettage
92
What is the disease defined by multiple enchondromas?
Ollier disease
93
Multiple enchondromas/hemangiomas define?
Maffucci syndrome
94
What is the xray finding of a enchondroma?
Well-circumscribed, lucent lesion with stippled Ca2+ intermixed
95
What is the gross finding of enchondroma?
A glistening blue gray lesion
96
What is the histo of a enchondroma?
It is bland appearing hyaline cartilage
97
What is an osteochondroma?
Bony exostoses with cartilage cap (mushroom cap)
Rarely undergo malignant transformation
98
What is a chondroblastoma?
Giant cell based tumor of adolescents
Epiphyseal based
Chicken wire calcification
99
What is a chondrosarcoma?
Adult tumor (30-60y)
Involves long bones, flat bones of pelvis
Pt's often present with pain
100
What is seen in radiology of chondrosarcoma?
Destructive lesion with cortical loss and usually soft tissue spread
101
What is the histo for a chondrosarcoma?
Well-diff
Resembles normal cartilage with permeative growth pattern
102
What is the grading for a chondrosarcoma?
Grade 1 (resembles normal cartilage): 83% survival
```
Grade II (myxoid)
Grade III (poor diff) These two are 53% 5 year survival
```
103
What is the grading for a chondrosarcoma?
Grade 1 (resembles normal cartilage): 83% survival
```
Grade II (myxoid)
Grade III (poor diff) These two are 53% 5 year survival
```
Mets to Lung
104
What do you see on xray for a chondrosarcoma?
A aggressive looking lesion
105
What is seen in histo for a chondrosarcoma?
Grade I normal appearing cartilage that is infiltrating bony trabeculae
106
What is a de-differentiated chondrosarcoma?
Abrupt transition from a well-differentiated chondrosarcoma to pleomorphic sarcoma
Neoplastic progression
107
What is a giant cell tumor of bone?
Adult Tumor
20-45y
Painful that may present with a pathologic fracture
108
What do you see on radiology for a giant cell tumor?
Lytic, destructive lesion
Involves epiphyses of long bones (>50% around knee)
109
What do you on histology for a giant cell tumor?
Tumor composed of proliferation of neoplastic mononuclear cells amidst neurons
Reactive osteoclast-like giant cells
110
What is the biology of a giant cell tumor?
Benign but locally aggressive, rarely mets
111
What is the treatment of a giant cell tumor of bone?
Curettage, 10-15% recur
112
What is the treatment of a giant cell tumor of bone?
Curettage, 10-15% recur
113
What is Ewing Sarcoma?
Small round cell sarcoma
Balance translocation of EWSR1 with ETS family Tx factor
Novel fusion oncogene
Chromosome 22
114
Where does Ewing Sarcoma hit?
Long bones of children (5-20y)
10-20% extraskeletal
115
What do you see on radiology of Ewing Sarcoma?
Non-specific thickeing of cortical bone
| Due to tumor in medullary cavity of diaphysis
116
What is the histology of Ewing sarcoma?
Sheets of monotonous, primitive appearing blue cells
Scant cytoplasm tends to be clear
Express high levels of membrane glycoprotein (CD99)
117
What does immunohisto detect in Ewing sarcoma?
Membrane glycoprotein CD99
118
What does FISH find in Ewing sarcoma?
A EWSR1 rearrangement
119
What is the treatment of Ewing sarcoma?
Neoadjuvant chemo with surgery
2/3 cured, otherwise lethal
120
What is the most common hematolymphoid tumor of bone?
Myeloma
Primary bone lymphoma also found
121
What are some soft tissue tumors arising in bone?
Angiosarcomas are seen
Use CD31 to stain for this type
