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Robbins Pathology MSK (Chapters 24,25,26) > Chapter 25- Bones, Joints > Flashcards

Chapter 25- Bones, Joints Flashcards

1
Q

What is the meaning of osteoporosis

A

Porous, or thinned out bone

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2
Q

What is the meaning of osteomalacia

A

Weakened, soft bone

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3
Q

What is the meaning of Osteopetrosis

A

Peter=rock, stone bone

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4
Q

What is the meaning of osteogenesis imperfecta

A

Imperfect origin of bone

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5
Q

Osteitis deformans

A

Deforming bone-itis

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6
Q

What is the meaning of osteodystrophy

A

Difficult/bad growing bone

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7
Q

The hardness of the bone is made up of which material

A

Hydroxyapatite

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8
Q

What is contained in bone hydroxyapatite

A

99% of body calcium

85% if phosphorous

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9
Q

What is the source and function of osteopontin

A

Osteoblasts and functions in calcium homeostasis

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10
Q

What are the characteristics of woven bone and which individuals are commonly seen to have it

A
  • Produced rapidly during fetal development, abnormal in adults
  • Seen in fracture repair
  • Arrangement of collagen, with less structural integrity
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11
Q

What is the structure of woven versus lamellar bone

A

Woven bone has more collagen and disorganized

Lamellar bone: parallel collagen and stronger

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12
Q

Which cells in the bone regulate the calcium and phosphate levels

A

Osteocytes

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13
Q

What is the method of bone development in long bones

A

Endochondral ossification

*New bone at the bottom of the growth plate giving longitudinal growth

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14
Q

What is the method of bone growth in flat bones

A

Intramembranous ossification

*new bone placed on preexisting surface, aka appositional growth

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15
Q

When is peak bone mass achieved

A

Early adulthood after cessation of skeletal growth

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16
Q

Rank and RANKL result in which process with regards to bone

A

Breakdown

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17
Q

OPG results in which process with regards to bone

A

Buildup

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18
Q

What is is the pathway/receptor activation involved with RANK

A

NF kappaB

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19
Q

What is the mechanism that OPG works

A

Binds to the RANKL and prevents it from binding to the RANK

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20
Q

What is the process and result of WNT proteins with regards to bones

A

WNT proteins bind to LRP5/6 on osteoblasts and activate Betacatenin and production of OPG

*Results in bone building

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21
Q

What is the process and result of the activation of sclerostin

A

Inhibits the WNT/Beta catenin pathway, Inhibition in the production of OPG

*Results in bone breakdown

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22
Q

What are the compounds that commonly cause the building of bone

A

Testosterone
Estrogen
Vitamin D

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23
Q

What are the common compounds that cause bone breakdown

A

PTH
IL-1
Glucocorticoids

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24
Q

What is the role of M-CSF

A

On osteoclasts and stimulate tyrosine kinase cascade that causes the generation of osteoclasts

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25
Q

What is dystosis of bone

A

Migration and condensation of mesenchyme into cartilage analage

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26
Q

What is the dysplasia of the bone

A

GLobal disorganization of bone and cartilage

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27
Q

What are the genes that can cause dystosis

A

Homeobox genes

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28
Q

What are the common conditions that are classified under dystosis

A

Aplasia (missing bone)
Supernumerary (extra bone)
Syndatcyly or craniosynostosis (abnormal fusion of bone)

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29
Q

What is the gene associated with:

-Short terminal phalanges of thumb and big toe

A

HOXD13

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30
Q

What is the gene associated with:

  • Abnormal clavicle
  • Wormian bones (extra cranial sutures)
  • supernumerary teeth
A

RUNX2

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31
Q

What is the gene associated with:

Achondroplasia, aka causing drawfism

A

FGFR3 gain of function

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32
Q

What are the clinal features of drawfism

A

Normal trunk length

No change in the longevity, intelligence, or reproductive status

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33
Q

What is the gene associated with:

Thanatrophic dysplasia

A

Most lethal form of drawfism

FGF3

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34
Q

What are the clinical features of thanatophoric dysplasia

A
  • Small chest cavity (insufficient respiration)

- microcephalic

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35
Q

What is the gene associated with:

Abnormal bone density

A
  • LPR5 receptor (osteoporosis or osteopetrosis)

- Mutations in RANKL (decreased or missing osteoclasts)

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36
Q

What is the structure affected by osteogenesis imperfecta

A

Type 1 collagen (alpha 1 and 2 chains)

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37
Q

What is the state and type of collagen effect in type 1 osteogenesis imperfecta

A

Decreased amount of type 1 collagen, but the structure is normal

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38
Q

When do most fractures in type 1 osteogenesis imperfecta occur

A

Before puberty, with the decreased frequency as the age increases

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39
Q

What is the lifespan with type 1 osteogenesis imperfecta

A

Normal life span with normal or near normal stature

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40
Q

What are the clinical features of osteogenesis imperfecta type 1

A
  • *Blue/purple/grey tint to sclera
  • Triangular face
  • brittle teeth
  • Hearing loss (in the 20s/30s)
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41
Q

What is the state of the bone deformities in type 1 osteogenesis imperfecta

A

Normal bone with minimal to absent deformities

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42
Q

What is the lifespan in type 2 osteogenesis imperfecta

A

Lethal

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43
Q

What are the clinical features of type 2 osteogenesis Imperfecta

A
  • Lethal due to respiratory issues arising from the small thoracic cavity
  • Numerous fractures and severe bone deformities
  • Small stature with small lungs
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44
Q

What is the state of bone deformity in type 3 osteogenesis imperfecta

A

Bone deformity, usually severe

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45
Q

What is the state and type of cartilage in type 3 osteogenesis imperfecta

A

Collagen type 1 in improperly formed

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46
Q

What are the clinical features of type 3 osteogenesis imperfecta

A
  • *Fractures present at birth, usually revealing healed fractures
  • Blue sclera
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47
Q

What is the status and type of collagen in type 4 osteogenesis imperfecta

A

Collagen type 1 is improperly formed

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48
Q

What are the clinical features of osteogenesis imperfecta type 4

A
  • Inbetween type 1 and 3
  • White or near white sclera
  • shorter than normal stature
  • Fractures before puberty
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49
Q

What is the description of osteopetrosis

A

Aka Marble bone disease or Albee’s-Schoenberg disease

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50
Q

What is the gene and its product mutation in osteopetrosis

A

CLCN7, which encodes the proton pump carbonic anhydrase 2 on osteoclasts and renal tubular cells

*carbonic anhydrase required for proton generation from CO2 and water

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51
Q

What is the result of carbonic anhydrase deficiency in osteopetrosis

A

Inability to acidity the osteoclast reabsorption pit to solubilize hydroxyapitie, results in lack or resorption

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52
Q

What are the physical findings in osteopetrosis

A

Lack a medullary cavity, with bulbous ends of long bones (which look like Erlenmeyer flasks)

-Neuronal foramina are small, which can compress the nerves

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53
Q

What is the inheritance fo the severe infantile type of osteopetrosis

A

Autosomal recessive

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54
Q

What is the inheritance form of the mild type of osteopetrosis

A

Autosomal dominant

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55
Q

What are the clinical findings in those patients with the severe infantile form of osteopetrosis

A
  • Cranial nerve defects (optic atrophy, deafness, facial paralysis)
  • Fractures, anemia, hydrocephaly
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56
Q

What are the clinical findings of the mild from of osteopetrosis

A

Repeated fractures

-Mild cranial nerve defects and anemia

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57
Q

What is the cause of mucopolysaccharidosis

A

Lysosomal storage disease in which there is a deficiency in the enzyme that degrades dermatan sulfate, heparan sulfate and heratan sulfate

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58
Q

What is the enzyme that is commonly affected in mucopolysaccharidoses

A

Acid hydrolase enzymes

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59
Q

What is the result of mucopolysaccaridoses

A

Mucopolysaccharides accumulate in the chondrocytes and in the extracellular space, which results in the death of the cells and defects in the articular cartilage

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60
Q

What are the physical features of a patient with mucopolysaccaridoses

A

Short stature, chest wall abnormalities, and malformed bones

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61
Q

What are the three only causes of primary osteoporosis

A

1) Idiopathic
2) Postmenopausal
3) Senile

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62
Q

What is the definition of osteopenia

A

Decreased bone mass that is 1-2.5 SD below the mean

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63
Q

What is the definition of osteoporosis

A

Bone mass reduction that is at least 2.5 SD below the mean for peak bone mass

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64
Q

How does the diet of a younger female affect her chances of osteoperosis

A

Insufficiency in calcium uptake in the teenage years cause restriction of the peak bone mass, which sets them up for osteoporosis later in life

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65
Q

What is the mechanism in which menopause can cause osteoporosis

A

Estrogen deficiency causes the increased resorption and formation of bone, but there is a higher rate of resorption, leading to the increased turnover of bone

*Deceased amount of estrogen leads to the increased number of RANKL and decreased OPG

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66
Q

What are the physical bone findings in the case of osteoporosis due to menopause

A

Increased osteoclast activity resulting in the thinning of the trabeculae, resulting in microfractures and even the vertebral collapse

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67
Q

What are the physical findings in the case of osteoporosis caused by senile and old age

A

Cortex of the bone is thinned by subperiosteal and endosteal resorption

*Haversian system is widened

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68
Q

What is the test used to look at the bone density

A

DEXA scan (wont show up until there is at least 30% of bone loss)

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69
Q

What is the general process of Paget’s disease

A

Aka osteitis deformans

Increased but disordered and structurally unsound bone mass

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70
Q

What is monostatic Paget’s disease of the bone

A

Involves a single bone

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71
Q

What is the cause of the majority if the cases of Paget’s disease of the bone

A

40-50% are familial

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72
Q

In those sporadic cases of Paget’s disease of the bone, what is the mutation and what is its function

A

SQSTM1 gene, which increased the NFKB and activated osteoclasts

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73
Q

What is the radio graphing finding in the cause of Paget’s disease of the bone

A

“Cotton wool” with increased uptake on a bone scan

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74
Q

What is the average age that someone with Paget’s disease of the bone will be seen

A

70 years old

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75
Q

What is the morphology of the bone in Paget’s disease under histology

A
  • Mosiac like pattern that fits together like a jigsaw puzzle
  • Prominent cement lines
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76
Q

What are the features of Paget’s disease of the bone in the cause of the lytic phase

A

Large osteoclasts withe 100 nuclei

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77
Q

What are the features of Paget’s disease of the bone in the cause of the mixed phase

A

Osteoclasts persist, but there are lots of blasts as well

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78
Q

What are the features of Paget’s disease of the bone in the cause of the final phase

A

Burned out phase, with the osteoslcerotic phase

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79
Q

What are the clinical features of Paget disease

A
  • Chalk stick type fractures of the long bones
  • Hypervasculatiry of the paget bone, leasing to increased warming of the overlying skin and high output heart failure
  • Bowing of limbs
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80
Q

What is the seriously complication of Paget’s disease of the bone

A

High output heart failure due to increased blood flow due to the hypervascularity of the Paget bone

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81
Q

What are the lab values that will be seen in Paget disease of the bone

A

Increased alk phosphatase

Normal calcium and phosphorous

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82
Q

What is the cause of Rickets, and what is the mechanism

A

Vitamin D deficiency in children

-Decreased Deposition of bone in the growth plate

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83
Q

What is the cause of osteomalacia and what is the mechanism

A

Vitamin D deficiency in adults

-None formed during remodeling is undermineralized, leading to increased risk of fractures

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84
Q

What are the clinical features of rickets

A
  • Frontal bossing
  • squared off head
  • *Rachitic rosary of ribs
  • Bowed legs
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85
Q

Which bone condition is present in the case of “railroad tracks” on histology

A

Dissecting osteitis due to hyperparathyroidism

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86
Q

What is the mechanism of disecting osteitis

A

Hyperparathyroidism causes the osteoclasts to tunnel into the bone centrally along the length of the trabeculae

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87
Q

What are the bone abnormalities than can be caused by hyperparathyroidism

A

1) Osteoporosis
2) Dissecting osteoitis
3) Brown tumor
4) osteitis fibrosis cystica

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88
Q

What is the cause of features of a brown bone tumor

A

Severe form is aka Von Reckilinghausen disease of bone

Hyperparathyroidism leading to microfractures as a result of bone loss. The fractures causes the influx of macrophages and ingrowth fibrosis that creates a mass of reactive tissue

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89
Q

What is the mechanism that renal failure causes changes in pH and bone loss

A

Tubular dysfunction leads to renal tubular acidosis, which decreases the pH and dissolves the hydroxyapatite, leading to demineralization of the bone

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90
Q

What type of fracture is:

-Overlying skin is intact

A

Simple

91
Q

What type of fracture is:

-bone communicates with the surface of the skin

A

Compound

92
Q

What type of fracture is:

-bone is fragmented

A

Comminuted

93
Q

What type of fracture is:

-Ends of the bone at the fracture site are not aligned

A

Displaced

94
Q

What type of fracture is:
-Slowly developing fracture that follows a period of increased physical activity in which the bone is subjected to repetitive loads

A

Stress

95
Q

What type of fracture is:

-extending only partially through the bone, which is more common in infants when the bone is soft

A

Greenstick

96
Q

What type of fracture is:

-pathologic

A

Involved bone is weaker due to an underlying disease process, such as a tumor

97
Q

What is the state of the fracture at the end of the first week

A

Soft tissue callus or procallus

*Predominately uncalcified

98
Q

What is the state of a fracture after 2 weeks

A

soft tissue callus is transformed into a bony callus, reaching maximum girth after end to 2nd or third week

99
Q

What is the cause of osteonecrosis

A

Infarction of the bone and marrow

100
Q

What are the most common causes of osteonecrosis

A

Fractures or corticosteroids

*Also bisphosphanates in children (especially the jawbone)

101
Q

What form of osteonecrosis is described as infarcts that cause pain and is initially only associated with activity, then becomes constant

A

Subchondral infarction

102
Q

What is a complication seen with subchondral infarcts

A

They collapse and can cause secondary osteoarthritis

103
Q

Which form of osteonecrosis is associated with small and clinically silent infarcts, but present in the case of patients with sickle cell disease, the bends, or Gaucher’s disease

A

Medullary osteonecrosis

104
Q

In the case of osteonecrosis, which portion of the bone is most affected and why

A

Medullary infarcts are more common with the cortex often not being

*Zcortex has collateral flow to it

105
Q

Subchondral infarcts will have what in the bone

A

triangular or wedge shaped infarcts

106
Q

In the case of osteonecrosis of the bone, what does the dead trabeculae that remain serve as

A

Scaffolding for deposition of new bone, aka creeping substitution

*Most of the time, the pace is too slow to be effective, so there is collapse and sloughing go the articular cartilage

107
Q

What is sequesteum

A

Dead bone following a subperiosteal abcess, commonly becoming a draining sinus and release fragments

108
Q

What is a involucrum

A

Newly deposited bone serves as a shell of living tissue around the segment of dead bone

109
Q

What is a Brodie abscess

A

Small interosseous abcess frequently involving the cortex, which is then walled off by reactive bone

110
Q

Which bone tends to be affected by sclerosing osteomyolytis of Garre and what is it

A

Jaw

-associated with extensive new bone formation that obscures much of the underlying osseous structure

111
Q

Which condition will cause saber shin

A

Congenital syphilis,

*Bone lesion that appears about 5th month of gestation and is a reactive periosteum bone deposition on medial and anterior surface of tibia

112
Q

Which condition is present if there is the formation of saddle nose

A

Syphilis

113
Q

What is the most common benign bone tumor

A

Osteochondroma aka exostosis

114
Q

What are the characteristics of osteochonroma

A

85% are solitary, seen in early adulthood

-Men 3 times more likely

115
Q

What is the gene commonly mutated with osteochondromas

A

EXT1 and EXT2

116
Q

What is the location that osteochondromas tend to occur

A

Metaphysic near a growth plate of tubular bone

117
Q

What is the the associated risk with familiar exostosis

A

Aka osteochondroma

AD and progresses to chondrosarcoma

118
Q

What is the location that chondromas aka enchondromas tend to occur

A

Hands and feet

119
Q

What causes the formation of chondromas/enchondromas

A

Benign hyaline cartilage that forms a solitary meaphyseal lesion

120
Q

What is the location of the enchondral

A

Medullary cavity

121
Q

What is Ollier syndrome

A

Multiple enchondromas

122
Q

What is mafucci syndrome

A

Multiple enchondromas with angiomas

123
Q

What are patients with mafucci syndrome at an increased risk for

A

Chondrosarcomas and other malignancies

124
Q

What is the gene mutation seen in chondromas or enchondromas

A

IDH1 and 2

125
Q

What is the most common malignancy of the bone

A

Osteosarcoma

126
Q

What is the location and age that patients get chondrosarcomas

A

Males in their 40s or older

Axial skeleton

127
Q

What is the location that chondrosarcomas tend to spread

A

Lungs via hematogenous routes

128
Q

What does the chondrosarcomas look like on radiograph

A

Calcified matri appears as foci of flocculent densities

129
Q

What is the main way that chondrosarcomas arise

A

De novo (85%),

From preexisting osteochonadromas or enchondromas fro the rest

130
Q

What genetic abnormalities may be seen that lead to chondrosarcomas

A
  • EXT gene in multiple osteochondroma syndrome

- IDH1/2 in chondromatosis and sporadic chondrosarcomas

131
Q

What is garner syndrome and what is the gene mutation

A

APC

  • Multiple GI adenomas
  • Osteomas
  • thyroid and Desmond tumors, skin cysts
132
Q

What is the patient complaint that seems to arise with osteoid osteoma

A

Painful, usually worse at night, but is relieved by NSAID and aspirin use

133
Q

Osteoid osteoma is caused by the production of what

A

Prostaglandins E2 by osteoblasts

134
Q

What is the age at which osteoid osteomas arise

A

Young men, usually in their teens and early 20s

135
Q

What is the location that osteoid osteomas tend to occur

A

Appendicular skeleton, usually on the femur or tibia

136
Q

What is the difference between an osteoid osteoma and osteoblastoma with regards to size

A

Osteoma <2 cm

Osteoblastoma >2cm

137
Q

What is the radiographic findings in the cause of osteoid osteoma

A

Nidus aka central mineralization surrounded by reactive bone

138
Q

Which condition will have:

-Haphazardly interconnecting trabeculae of woven bone that are rimmed by prominent osteoblasts

A

Osteoid osteoma

139
Q

What are the features of osteoblastoma that are different than osteoid osteoma

A
  • Larger (>2 cm)
  • Involves the posterior spine
  • No bony reaction
  • DOES NOT RESPOND TO ASPIRIN
140
Q

What is the most common location for an osteosarcoma

A

The knee (60%)

141
Q

What is the presentation of an osteosarcoma

A

Painful enlarging mass

142
Q

What is the age group osteosarcoma

A

Bimodal, with 75% younger than 20 years old, usually around the knee

Second group is those with Paget’s or prior radiation (secondary osteosarcoma)

143
Q

Which genetic factors will increase the risk for an osteosarcoma

A
  • Rb gene (70% of cases)

- TP53, Li-Fraumani syndrome

144
Q

What is the radiographic finding in the case of osteosarcoma

A
  • Mixed lytic and blastic mass

- Codman triangle, which is elevation of the periosteum

145
Q

What is the chances of metastasis in an osteosarcoma and what are the common locations

A

Hematogenous LH spread, with 90% to the lungs, bones or brain

146
Q

What is the age and patient population seen to get Ewing sarcomas

A

<20 year olds, with redirection for White patients

147
Q

What are the background for the Ewing sarcoma

A

Small blue round cell tumor

148
Q

What is the genetic component involved in Ewing sarcoma with a neural differentiation

A

PNET (primitive neuroectodermal tumor)

149
Q

What is the clinical presentation of Ewing sarcoma

A

Painful enlarging mass, frequently tender, warm, swollen

-Usually mimics an infection with a fever and increased sedimentation rate

150
Q

What is the periosteal reaction seen in Ewing sarcoma

A

Reactive bone in the onion skin type fashion on X-RAY

151
Q

Which bones are commonly affected by Ewing sarcoma

A

Long bones, especially the femur

*Arises in the medullary cavity and Incase’s the cortex

152
Q

What is the gene and translocation seen in Ewing sarcoma

A

t(11;22)(q24:q12) leading to a EWS-FL11 fusion gene (EWS gene)

153
Q

What is the important prognostic findings in the case of Ewing sarcoma

A

The amount of chemotherapy-induced necrosis

154
Q

What is the gene and histological finding in the cause of Ewing sarcoma with a neural component

A

PNET

-Homer-Wright rosettes with a central fibrillation core

155
Q

What is the prognosis of fibrous dysplasia

A

Benign proliferation of fibrous tissue and bone that does not mature

156
Q

Which form of fibrous dysplasia is more common

A

Monostatic (70%) usually asymptomatic

**Polustatic tends to be crippling

157
Q

Which bone condition will arise with the appearance of ground glass

A

Fibrous dysplasia

158
Q

What is McCune-Albright disease

A
  • Unilateral fibrous dysplasia bone lesions
  • pigmented skin lesions (Cafe au lait) on the side of the deformities
  • Precocious puberty in females
159
Q

What is Mazabraud syndrome

A
  • Fibrous dysplasia (usually polyostotic)
  • multiple skeletal deformities in childhood
  • Soft tissue myxomas (intramuscular)
160
Q

What condition commonly has the histological finding of curvilinear trabeculae, usually described as “Chinese characters”

A

Fibrous dysplasia

161
Q

What is the cell of a giant cell tumor of the bone

A

Aka osteoclastoma

-Multinucleated osteoclast type giant cell

162
Q

Of those malignancies in the bone, what is the common primary location

A

Prostate, breast, kidney, lung

163
Q

What are the metastatic tumors that spread to bone in children

A
  • Neuroblastoma
  • Wilms
  • Osteosarcoma
  • Ewing
  • Rhabdomyosarcoma
164
Q

What are the characteristics of the lytic bone diseases and where are they commonly coming from

A

Bone destroying tumor

-Kidney, lung, GI tract, melanoma

165
Q

Which form of metastatic tumors tend to be blastic

A

Aka bone forming

-Prostatic adenocarcinoma

166
Q

What are the characteristics of the synarthrosis

A

Solid joints, which provide structural integrity and only allow minimal movement
-lots of type 2 collagen

-Lack joint space

**Such as teeth

167
Q

What are the common locations affected by osteoarthritis aka DJD

A
  • Hands and knees in women

- Hips in men

168
Q

What are predisposing conditions that can cause osteoarthritis/DJD

A
  • Joint deformities
  • Trauma
  • Obesity
169
Q

Which conditions can cause secondary osteoarthritis

A

-DM
-Thyroid
-Acromegaly
Charcot

170
Q

What are the clinal patient complaints in the case of osteoarthritis/DJD

A
  • Evening stiffness
  • crepitus
  • gets worse with use
171
Q

What is the name of the nodes in the proximal IP joint

A

Bouchard’s nodes

172
Q

What is the name of the nodes in the distal IP joint

A

Henerden nodes

173
Q

What is the mechanism of disease for osteoarthritis

A
  • Increased in the water content of the Matrix
  • decreased concentration of proteoglycans

*Leads to cracks in the bone matrix

174
Q

What is eburnation

A

Subchondral bone exposure that rubs smooth and leads to bone on bone pain

175
Q

What is the result of osteoarthritis

A
  • Eburnation (bone on bone pain)
  • microfractures and cysts
  • Osteophytes develop
  • Fibrillation on histology

*can lead to nerve root compression and radicular pain

176
Q

What is the common appearance in the case of bone in a patient with osteoarthritis

A

There is the formation of a cyst and some residual cartilage, then there is none, forming the eburnation

177
Q

What are the characteristic features of the joint in rheumatoid arthritis

A
  • Inflammation
  • Pannus
  • Eroding cartilage
  • fibrous and bony ankylosis
178
Q

What are the characteristic features of the joint in osteoarthritis

A
  • Subchondral cysts
  • No ankylosis
  • Subchondral sclerosis
  • Osteophytes
  • Thinned and Fibrillated cartilage
179
Q

What is the method of rheumatoid arthritis (RA)

A

Autoimmune, nonsuppurative proliferation and inflammatory synovitis

-Destruction of the articular cartilage

180
Q

What is ankylosis

A

Stiffness of a joint due to abnormal adhesion and rigidity of the bones of the joint, which may be the result of injury or disease

181
Q

Which joints tend to be affected in RA

A

Symmetrical distribution with small joints affected

182
Q

What is the compound that tends to be elevated in the mechanism of rheumatoid arthritis

A

TNF

183
Q

In the joints during rheumatoid arthritis, what are the factors that tend to be circulating and support a diagnosis

A
  • Antigen antibody complexes that contain citrullinated peptides (CCP’s)
  • Rheumatoid factor (RF)
184
Q

What is the condition of boutonniere and what condition is it seen with

A

Rheumatoid arthritis (RA)

  • Deformity of finger
  • hyperextension of DIP
  • flexion of PIP
185
Q

What is the condition of Swan-neck and what condition is it seen with

A

Rheumatoid arthritis

  • Hyperextension of PIP
  • Flexion of DIP
186
Q

What is the condition seen with RA and ulnar deviation

A
  • Decreased joint space
  • Periarticular bony erosion

-Ulnar deviation of the fingers with radial deviation of the wrist

187
Q

What is pannus with regards to RA

A

Edematous, Thickened, hyperplastic synovium

188
Q

What are rheumatoid nodules

A

Extension surfaces at pressure points get these nodules that can Mimic systemic autoimmune disorders such as SLE or scleroderma

189
Q

What features will wheumatoid nodules have histologically

A

Central area of necrosis with palisading histiocytes

190
Q

What is the HLA match that is associated with ankylosing spondylitis

A

HLA-B27, negative for rheumatoid factor (RF)

191
Q

What is the mechanism of disease for Seronegative spondyloarthropathies

A

Autoimmune, T cell mediated, with no specific Abs present

192
Q

What is the triad for reactive arthritis

A

1) Arthritis
2) Urethritis or cervicitis
3) Conjunctivitis

193
Q

What is the HLA present in psoriatic arthritis

A

HLA-B27

194
Q

What is the radiographic findings i the case of psoriatic arthritis

A

“Pencil in a cup”

-Pitting in the nails

195
Q

What is the common cause of suppurative arthritis in children younger than 2 years old

A

H. Influenza

196
Q

What is the cause of suppurative arthritis in the cause of older kids and adults

A

Staph aureus, gonococcus infection

197
Q

Gout is caused by which compound

A

Monosodium urate (MSU) within and around the joints

198
Q

What is the plasma level of crystals that will cause gout

A

> 6.8mg/dL

199
Q

Which joints tend to be favored in the case of gout

A

Lower temperatures where the crystals can precipitate

200
Q

What is the cytokine that is present in gout

A

IL-1Beta

201
Q

What is gouty tophus

A

Pathomnumonic for gout

202
Q

What is the joint hit with gout first

A

The first metatarsophalangeal joint

203
Q

What is the compound present in the cause of pseudogout

A

Calcium phyrophosphate crystals

204
Q

What are the characteristics of the crystals present in pseudogout

A
  • chalky, white, friable deposits

* Individual crystals are rhomboid and are positively infringe this

205
Q

What is a simple test that can be done to look if it is a ganglion cyst

A

Translucent nodule when shined with a light

*commonly at the wrist as a firm pea sized cyst that lacks cell lining

206
Q

What is the cause of ganglionic cysts

A

Cystic of my oil degeneration, with cyst lacking cell lining

207
Q

What is the location and cause of a Baker cyst

A

Popliteal cyst, usually a cause of RA

208
Q

What are the common locations for soft tissue tumors

A

Arises in extremities, especially the thigh

209
Q

What is the common translocation in the cause of liposarcomas, the gene product and the result of the mutation

A

T(12;16)(q13;p11)

FUS-DDIT3

Arrests Adipocytes differentiation

210
Q

What is the common translocation in the cause ofsynovial sarcoma, the gene product and the result of the mutation

A

T(x;18)(p11;q11)

SS!*

Interrupts cell cycle control

211
Q

What is the common translocation in the cause of rhabdomyosarcoma, the gene product and the result of the mutation

A

T(2;13)(q35;q14)

FOX01

Disrupts skeletal muscle differentiation

212
Q

What is the most common soft tissue tumor of adults

A

Lipomas

213
Q

What are the histological findings in the cause of lipoma

A

Encapsulated mass of normal appearing adipose tissue

214
Q

What is the method that the liposarcomas are able to form

A

They have a potent inhibitor of p53

215
Q

What is the most common type of liposarcoma

A

Myxoid variant

216
Q

What is Gardner syndrome and what is the gene that is commonly mutated

A

FAP, with a germline in the APC gene

This syndrome is at an increased risk for developing fibrous tumors

217
Q

Which condition with have herringbone fascicles

A

Fibrosarcoma

218
Q

What are the clinical features of a fibrosarcoma

A

Dee seated, slow growing mass in the thigh or trunk of young or middle aged adults

219
Q

What are the translocations seen in rhabdomyosarcoma

A

Pax3 (2;13)

Pax7 (1;13)

220
Q

Which patient population is most commonly affected by rhabdomyosarcoma

A

Kids

221
Q

What is the name of the tumor if there is embryonal tissue in the rhabdomyosarcoma

A

Rhabdomyoblasts

*Has cross striations

222
Q

Which tumor has sarcoma botryoides and which layer are they

A

Cambium layer, seen in rhabdomyoblasts

223
Q

What are the conditions that are seen to have leiomyoma

A

Hereditary leiomyomatosis and renal cell carcinoma syndrome (Autosomal dominant)

224
Q

Leiomyoma are of which cell type origin

A

Smooth muscle origin

Robbins Pathology MSK (Chapters 24,25,26) (7 decks)

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