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Path II > Non Neo/Neo Bone > Flashcards

Non Neo/Neo Bone Flashcards

1
Q

What is achondroplasia?

A

Autosomal dominant dwarfism

Caused by FGFR3 mutation

Inhibits cartilage proliferation

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2
Q

What is osteogenesis imperfecta?

A

Due to mutations that encode TYPE I collagen subunits

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3
Q

What is osteomyelitis?

A

Broadly divided into pyogenic and granulomatous histologies

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4
Q

What causes pyogenic osteomyelitis?

A

Staph. aureus

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5
Q

What causes pyogenic osteomyelitis in neonates?

A

Group B strep, Haemophilus influ

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6
Q

What causes pyogenic osteomyelitis in sickle cell patients?

A

Salmonella

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7
Q

What are the biggest causes of osteoporosis?

A

Post menopause and senile

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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

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9
Q

What causes secondary osteoporosis?

A

Hyperparathyroidism and Chronic renal disease

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10
Q

What causes a callus?

A

A healing fracture

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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

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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

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13
Q

What is McCune-Albright syndrome?

A

GNAS1 mutation

Polyostatic endocrinopathy w/ CAFE au LAIT spots

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14
Q

What causes osteoarthritis?

A

Breakdown of articular cartilage

Due to cytokine production

Results in secondary changes, burnishing of bone
subchondral cysts
Osteophytes

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15
Q

What causes osteoarthritis?

A

Breakdown of articular cartilage

Due to cytokine production

Results in secondary changes, burnishing of bone
subchondral cysts
Osteophytes

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16
Q

What is rheumatoid arthritis?

A

Systemic autoimmune disease

Symmetrical arthritis

Involves multiple joints, especially in hands

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17
Q

What are characterisitic pathology features of rheumatoid arthritis?

A

Destructive

Inflammatory synovitis (Pannus)

Necrotizing granulomas (rheumatoid nodules

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18
Q

What is rheumatoid factor?

A

Involved in rheumatoid arthritis

IgM directed against Fc portion of IgG

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19
Q

What is Gout?

A

Imbalance of urate production and excretion

Caused by monosodium urate crystals

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20
Q

What are monosodium urate crystals?

A

Needle-shaped that are negatively birefringent under polarized light

Turn yellow in parallel light

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21
Q

When in peak bone mass?

A

20-40

Slow decrease after especially in women

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22
Q

What stimulates osteoclasts?

A

Osteoblasts

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23
Q

What is thanatotropic dwarfism?

A

Lethal form of dwarfism due to two copies of FGFR3 mutation

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24
Q

What is a type I collagen disease?

A

Osteogenesis imperfecta

Mutation in a1 and a2 chain of collagen type I

AD

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25
Q

What are the phenotypes of Osteogenesis imperfecta?

A

Brittle bones/ BLUE SCLERA

Lethal in utero

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26
Q

What is osteomyelitis?

A

Inflammation of bone marrow and bone

Usually infectious

Inflammation is pyogenic or granulomatous

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27
Q

What are the hematogenous spread osteomyelitis?

A

Long bones in children

Vertebra in adults

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28
Q

What is direct extension osteomyelitis?

A

Jaws or Skulls from dental or sinus infection

Toes/feet from diabetic foot ulcers

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29
Q

What are the pathogens involved in osteomyelitis?

A

S. aurues (90%)

Group B strep and Haemophilus in NEONATES

Pseudomonas (IVDU)

Mycobac (Elderly, debilitated, immunocompromised)

Salmonella (Sickle Cell patients)

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30
Q

What is seen in histo for chronic osteomyelitis?

A

Fibrosis and plasma cell infiltration in bone marrow

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31
Q

What is seen in tuberculosis osteomyelitis?

A

Necrotizing granulomatous inflammation

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32
Q

What is osteoporosis?

A

Age related changes

Reduced physical activity

Genetic factors

Nutritional factors

Hormonal influences

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33
Q

What are the hormonal influences of osteoporosis?

A

Estrogen def leads to acc bone loss

Lifetime risk 1 to 2 in @

1 in 40 in MEN

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34
Q

What is the mechanism of loss of bone mass in post menopause?

A

Decreased estrogens causes increased IL-1 which increases osteoclast activity

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35
Q

What is secondary osteoporosis?

A

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

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36
Q

What is brown tumor of bone?

A

Aggregates of osteoclasts and spindled stroma

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37
Q

How does a fracture repair itself?

A

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

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38
Q

What is paget disease of bone?

A

Collage of matrix madness

Three phases:

  • Osteolytic
  • Mixed osteoclastic-osteoblastic
  • Osteosclerotic
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39
Q

What is the etiology of paget disease?

A

Virus infection by paramyxovirus

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40
Q

When does paget disease appear?

A

After age 40

Pain

Elevated alk phos

Polyostotic 85%

SErious complication is osteosarcoma

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41
Q

When does paget disease appear?

A

After age 40

Pain

Elevated alk phos

Polyostotic 85%

SErious complication is osteosarcoma

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42
Q

What is seen on X ray for the sclerotic phase?

A

Irregular thickening of bone (cortical and trabecular)

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43
Q

How is the mosaic pattern of bone easily seen?

A

Seen best under polarized light

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44
Q

What is fibrous dysplasia?

A

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

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45
Q

What is the characteritic of McCune-Albright Syndrome?

A

Histology is chinese character like

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46
Q

What is the most common joint disease?

A

Osteoarthritis

Wear and tear

80% of people >70

Generally oligoarticular

Heberden nodes (DIP)

Secondary disease occurs due to prveious trauma or congenital deformity

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47
Q

What is the pathogenesis of osteoarthritis?

A

Chondrocytes produce (IL-1/TNF-a) that induce production of metalloproteinases and inhibit collagen and proteoglycan synthesis

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48
Q

What is Rheumatoid Arthritis?

A

Chronic systemic autoimmune disorder

Polyradicular

Severe form of chronic synovitis with secondary destruction of tisue

Presents with PIP or MCP joint involvement

NO DIP

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49
Q

What is pathogenesis of Rh arthritis?

A

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
Q

How do you make the dx of Rh arthritis?

A

Morning stiffness, arth of >3 joints, Rh nodules, RF, typical radiology (4 of 7)

51
Q

What is the joint deformity of RA?

A

Ulnar deviation

52
Q

What is gout secondarily caused by?

A

Leukemia (tumor lysis syndrome), chronic renal disease, Lesch-Nyhan syndrome (HGPRT def)

53
Q

What is a gouty tophus?

A

A foreign body composed of many giant cells

54
Q

Where do most primary bone tumors arise?

A

Metaphyses

55
Q

What are osteomas?

A

Proliferation dense, cortical bone, affecting facial/skull bones

Dx based on radiological finding

56
Q

What are osteoid osteomas?

A

Pediatric tumor

Small size

57
Q

What are osteoid osteomas?

A

Pediatric tumor

Small size

58
Q

What is a osteoblastoma?

A

A osteoid osteoma that is big >2cm

Found in the vertebrae

Produce different pain

NOT RESPONSIVE TO NSAIDS

59
Q

What is a osteosarcoma?

A

A malignant mesenchymal tumor

Production of malignant osteoid

Predominates in children

Adults if had Paget disease before

60
Q

How do you treat osteosarcoma?

A

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
Q

What is an endochondrom?

A

A benign cartilagenous tumor

Small size, circumscription, tendency to invovle tubular bones

62
Q

What is a osteochondroma?

A

Bony outgrowth with cartilagenous cap

Cap rarely undergoes mal transformation to chondrosarcoma

63
Q

What is a chondroblastoma?

A

Benign

Young person

Epiphyseal based

Chicken wire histo

64
Q

What is chondrosarcoma?

A

Adult cartilage forming sarcoma

Permeative growth

Observance is well-differentiated grade I

65
Q

What is a de differentiated chondrosarcoma?

A

Abrupt transformation of well diff to a high grade sarcoma

66
Q

What is a giant cell tumor of bone?

A

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
Q

WHat is a Ewing sarcoma?

A

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
Q

What is the most common cause of cancer in bone?

A

METS

69
Q

What mets are osteoblastic?

A

Prostate and Breast

70
Q

What mets are mixed osteoblast/lytic?

A

Lung

71
Q

What mets are lytic?

A

Kidney, thyroid

72
Q

What mets are lytic?

A

Kidney, thyroid

73
Q

What is punctate calcification?

A

Cartilage

74
Q

What is diffuse cloud like calcification?

A

Osteoid

75
Q

What are benign features?

A

Sclerotic rim

Lack of cortical bone destruction

Lack of soft tissue extension

Lack of periosteal reaction

76
Q

What are mal features?

A

Ill defined

Infiltrative borders

77
Q

What are osteoid producing primary tumors?

A

Osteoma

Osteoid Osteoma

Osteoblastoma

Osteosarcoma

78
Q

What are the features of a osteoid osteoma?

A

75% of patients are

79
Q

What is seen on xray for a osteoid osteoma?

A

A cortical, well-circumscribed, radiolucent lesion

80
Q

What is seen in histo?

A

A nidus of osteoid that is surrouded by dense reactive rim of bony tissue

81
Q

What is seen in histo?

A

A nidus of osteoid that is surrouded by dense reactive rim of bony tissue

NIDUS: woven bone with osteoblastic rimming

82
Q

What is a osteoblastoma?

A

A large osteoid osteoma in vertebra most typically

> 2cm

No relief with NSAIDS

DULL pain

83
Q

What is a huge relative risk to developing osteosarcoma?

A

Hereidtary retinoblastoma

84
Q

What is the radiology of a osteosarcoma?

A

Admixed radiolucent and radiodense lesion

Cortical destruction

Periosteal reaction

Sunburst, codman’s triangle

Cumulus appearance

85
Q

What is the histo of osteosarcoma/

A

High grade mal tumor with atypical osteoblasts

Lay down malignant osteoid

Multiple subtypes
Osteoblastic, chondroblstic, fibroblastic, telangiectatic

86
Q

What is the treatment for osteosarcoma?

A

Neoadjuvant chemo (doxorubicin, cisplastin, HD methotrexate)

FOllowed by radical surgery
Limb salvage if can

Adults may receive post op chemo

87
Q

Where is the most common mets of osteosarcoma?

A

To lung but can pluck tumor nodules if it does

88
Q

Where does an osteosarcoma grow on histo?

A

Tends to grow on a scaffold of preexisting trabeculae

Lacy osteoid wtih atypical cells embedded

89
Q

What is an endochondroma?

A

Most common intraosseous cartilage tumor

Tubular bones

Hand

Peak incidence 3-5 decade, M=F

Lucent lesions with sclerotic

90
Q

What is an endochondroma?

A

Most common intraosseous cartilage tumor

Tubular bones

Hand

Peak incidence 3-5 decade, M=F

Lucent lesions with sclerotic

91
Q

What is the treatment for an endochondroma?

A

Curettage

92
Q

What is the disease defined by multiple enchondromas?

A

Ollier disease

93
Q

Multiple enchondromas/hemangiomas define?

A

Maffucci syndrome

94
Q

What is the xray finding of a enchondroma?

A

Well-circumscribed, lucent lesion with stippled Ca2+ intermixed

95
Q

What is the gross finding of enchondroma?

A

A glistening blue gray lesion

96
Q

What is the histo of a enchondroma?

A

It is bland appearing hyaline cartilage

97
Q

What is an osteochondroma?

A

Bony exostoses with cartilage cap (mushroom cap)

Rarely undergo malignant transformation

98
Q

What is a chondroblastoma?

A

Giant cell based tumor of adolescents

Epiphyseal based

Chicken wire calcification

99
Q

What is a chondrosarcoma?

A

Adult tumor (30-60y)

Involves long bones, flat bones of pelvis

Pt’s often present with pain

100
Q

What is seen in radiology of chondrosarcoma?

A

Destructive lesion with cortical loss and usually soft tissue spread

101
Q

What is the histo for a chondrosarcoma?

A

Well-diff

Resembles normal cartilage with permeative growth pattern

102
Q

What is the grading for a chondrosarcoma?

A

Grade 1 (resembles normal cartilage): 83% survival

Grade II (myxoid)
Grade III (poor diff) These two are 53% 5 year survival
103
Q

What is the grading for a chondrosarcoma?

A

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
Q

What do you see on xray for a chondrosarcoma?

A

A aggressive looking lesion

105
Q

What is seen in histo for a chondrosarcoma?

A

Grade I normal appearing cartilage that is infiltrating bony trabeculae

106
Q

What is a de-differentiated chondrosarcoma?

A

Abrupt transition from a well-differentiated chondrosarcoma to pleomorphic sarcoma

Neoplastic progression

107
Q

What is a giant cell tumor of bone?

A

Adult Tumor

20-45y

Painful that may present with a pathologic fracture

108
Q

What do you see on radiology for a giant cell tumor?

A

Lytic, destructive lesion

Involves epiphyses of long bones (>50% around knee)

109
Q

What do you on histology for a giant cell tumor?

A

Tumor composed of proliferation of neoplastic mononuclear cells amidst neurons

Reactive osteoclast-like giant cells

110
Q

What is the biology of a giant cell tumor?

A

Benign but locally aggressive, rarely mets

111
Q

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

A

Curettage, 10-15% recur

112
Q

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

A

Curettage, 10-15% recur

113
Q

What is Ewing Sarcoma?

A

Small round cell sarcoma

Balance translocation of EWSR1 with ETS family Tx factor
Novel fusion oncogene

Chromosome 22

114
Q

Where does Ewing Sarcoma hit?

A

Long bones of children (5-20y)

10-20% extraskeletal

115
Q

What do you see on radiology of Ewing Sarcoma?

A

Non-specific thickeing of cortical bone

Due to tumor in medullary cavity of diaphysis

116
Q

What is the histology of Ewing sarcoma?

A

Sheets of monotonous, primitive appearing blue cells

Scant cytoplasm tends to be clear
Express high levels of membrane glycoprotein (CD99)

117
Q

What does immunohisto detect in Ewing sarcoma?

A

Membrane glycoprotein CD99

118
Q

What does FISH find in Ewing sarcoma?

A

A EWSR1 rearrangement

119
Q

What is the treatment of Ewing sarcoma?

A

Neoadjuvant chemo with surgery

2/3 cured, otherwise lethal

120
Q

What is the most common hematolymphoid tumor of bone?

A

Myeloma

Primary bone lymphoma also found

121
Q

What are some soft tissue tumors arising in bone?

A

Angiosarcomas are seen

Use CD31 to stain for this type

Path II (11 decks)

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