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Ortho > Pathology > Flashcards

Pathology Flashcards

1
Q

Achondroplasia is the reduction in proliferation of __1_ in developing growth plate; failure of normal ____2_____ formation

A
  1. chondrocytes

2. epiphyseal cartilage

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2
Q

Cause of achondroplasia (mutation?)

A

defect in signal transduction - point mutation involving gene coding of FGFR3 in cell signaling

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3
Q

what zones in achondroplasia are narrowed and disorganized –> no columns

A

zones of proliferation and hypertrophy

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4
Q

abnormality in OI

A

type I collage synthesis - mutations in genes that code for alpha1 and alpha2 chains

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5
Q

how is OI inherited

A

AD

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6
Q

For major subtypes of OI - which one is which type?

  1. lethal - multiple fracture in utero
  2. survive fractures in infancy but have progressive deformity
  3. increased risk of fracture but no deformity
A
  1. type II
  2. type III and IV
  3. type I
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7
Q

type of OI where you would see blue sclera, increased risk of fractures, and dental and hearing problems

A

type I

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8
Q

disease causes by decreased osteoclastic bone resorption –> diffuse skeletal sclerosis

abnormally thickened, heavily mineralized, abnormally brittle bone

A

Osteopetrosis

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9
Q

what osteopetrosis form is malignant and which is benign?

A

AR = malignant

AD = benign

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10
Q

what may you see clinically with osteopetrosis?

A

increase incidence of fractures

anemia

thrombocytopenia

increase susceptibility of infections

cranial nerve roots may be compressed –> cranial nerve palsy

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11
Q

What may be a helpful treatment for someone with osteopetrosis

A

bone marrow transplant

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12
Q

disorder where the cortex and trabeculae are thinned, but remain of normal composition

quantitative loss of bone

A

osteoporosis

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13
Q

what is primary osteoporosis related to?

A

age

menopause

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14
Q

Osteoporosis is worse in areas containing abundant ___ bone such as weight-bearing bones

A

trabecular

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15
Q

Osteoporosis will have a increase in _1__ of bone due to decrease in bone mass. There will be fewer and thinner _2__; do not join to form trabeculae

A
  1. Porosity

2. bony spicules

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16
Q

What are the 2 main vertebral fracture patterns of osteoporotic bone?

  1. anterior column of vertebral body is compressed leading to kyphosis, but usually with preservation of the spinal cord.
  2. an axial load disrupts the anterior, middle, and/or posterior columns leading to disruption of the spinal cord
A
  1. Wedge fractures

2. burst fractures

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17
Q

Rickets/osteomalacia is defective _________ caused by vitamin D deficiency

A

matrix mineralization

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18
Q

function of vitamin d

A

maintain adequate levels of calcium and phosphate for bone mineralization

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19
Q

What are the effects rickets has on children?

A
  1. heaping of unmineralized matrix; inadequate provisional calcification of epiphyseal cartilage
  2. softening of bones –> fractures and deformities
  3. inadequate maturation of cartilage
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20
Q

Most common causes of rickets/osteomalacia

A

Malabsorption syndrom

Inactive activation of vitamin D

(also: dietary deficiency of vit. D, inadequate vit. D synthesis, inborn error of vitamin D metabolism

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21
Q

generalized pain, fractures, mottled areas of lucency (pseudofractures) in bones are symptoms of what?

A

osteomalacia

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22
Q

Symptoms of rickets

A

loss of rigidity including:

  1. bowing of legs, 2. enlarged costochondral junctions (rachitic rosary),
  2. pigeon breast deformity,
  3. kyphosis,
  4. frontal bossing
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23
Q

what disorder produced bone cysts and brown tumors

A

hyperparathyroidism

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24
Q

what does PTH do

A
  1. activates osteoclast to resorb born, mobilizes calcium
  2. increase resorption of calcium by renal tubules
  3. increase synthesis of active Vit. D by kidneys –> enhanced calcium absorption from gut
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25
Q

what happens if have hyperparathyroidism

A

significant skeletal changes secondary to abnormal osteoclastic activity to increase serum calcium and phosphate

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26
Q

term describes collectively all of the skeletal chainges of chronic renal disease

A

renal osteodystrophy

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27
Q

pathophysiology of renal osteodystropy

A

First: renal failure –> decrease 1,25-(OH)2-D3 –> hypocalcemia –> secondary hyperparathyroidism

Second: osteitis fibrosa cystica

metabolic acidosis, iron and aluminum deposits in bone interfering with matrix mineralization –> osteomalacia

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28
Q

disorder of bone maintenance - acceleration of bone turnover –> increase bone mass of poorly structures and weak bone

  1. aggressive osteoclast-mediated bone resorption
  2. imperfect osteoblast-mediated bone repair
A

Paget disease

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29
Q

what virus like particles and antigens were identified in osteoclasts of Paget disease

A

Paramyxovirus

30
Q

what part of the bone dose Ewing sarcoma occur

A

diaphysis

31
Q

what part of the bone does chondrosarcoma occur

A

metaphysis

32
Q

what part of the bone does osteosarcoma occur

A

metaphysis

33
Q

what part of the bone does giant cell tumor occur

A

epiphysis

34
Q

rare benign tumor of mature bone - asymptomatic

A

osteoma

35
Q

site of osteoma

A

cortical bones of skull and face

36
Q

Xray - bone tumors where central area of tumor (nidus) is radiolucent, surrounded by rim of sclerotic bone

A

osteoid osteoma

osteoblastoma

37
Q

How do osteoid osteoma and osteoblastoma differ (have identical histology) in regards to pain and size?

A

Osteoblastoma

  • giant osteoid osteoma (>2 cm)
  • painful
  • not relieved with aspirin

Osteoid osteoma

  • smaller
  • painful
  • relieved with aspirin
38
Q

where is osteoblastoma found?

A

vertebral column

39
Q

age osteoblastoma affects

A

teens-twenties

40
Q

what is this the histology of?

  • interlacing trabeculae of woven bone surrounded by osteoblasts; loose CT stroma (no BM elements); may see giant cells
A

Osteoblastoma

Osteoid osteoma

41
Q

malignant mesenchymal tumor; neoplastic cells produce osteoid; sometimes makes cartilage

A

osteosarcoma

42
Q

bone tumor with bimodal age distribution: most occur <20 but small peak in elderly with predisposing conditions

A

osteosarcoma

43
Q

where does osteosarcoma usually metastisize to? (AGGRESSIVE)

A

lungs

also bone, liver, other

44
Q

Treatment for osteosarcoma?

A

pre-op chemo

radical surgery or limb salvage surgery and chemo

45
Q

What kind of bone tumor:

large destructive mixed blastic and lytic mass with infiltrating margins, Codman triangle

destroys cortex, extends inwards –> marrow cavity extends outwards –> adjacent soft tissues

A

osteosarcoma

46
Q

what bones are osteosarcomas most often found

A

knee

47
Q

elevation of periosteum to produce an angle between surface of involved bone

What bone tumor would you see this in?

A

Codman triangle

Osteosarcoma

48
Q

what age would you see osteochondroma; what gender?

A

young (10-30)

males (x3)

49
Q

what tumor would you expect if the xray showed polypoid growth (mushroom-shaped)

A

osteochondroma

50
Q

how do you treat osteochondroma

A

excision

51
Q

Benign tumor of hyaline cartilage, well circumscribed

A

chondroma

in medullary cavity called enchondromas

52
Q

what is it called if there are multiple enchondromas

A

Ollier disease

53
Q

common site of condromas

A

solitary, metaphyses of small bones of hands and feet

54
Q

X-ray shows well defined bone with stippled calcium - what is it?

A

chondroma

55
Q

chondromas affect what age

A

young - 20 to 40

56
Q

Microscopy: mature, hypocellular cartilage with bland chondrocytes

what is it?

A

chondroma

57
Q

when is there a worse prognosis for chondroma

A

if multiple chondroma syndrome (Ollier) - 1/3 develop chondrosarcoma

58
Q

where are chondrosarcomas found

A

pelvic bones, axial skeleton

59
Q

what determines the grade (1-3) of chondrosarcoma?

A

cellularity

degree of nuclear atypia

mitotic activity

60
Q

small round blue tumors

primitive neural tumors derived from a precursor multipotent mesenchymal stem cell

A

Ewing sarcoma (EWS)

PNET

61
Q

how do EWS and PNET differ

A

Both have neural phenotypes and identical chromosome translocation

EWS –> undifferentiated
PNET –> neural differentiation

62
Q

sheets of uniform, undifferentiated small round blue cells

A

Ewing sarcoma

63
Q

Homer wright rosettes with central fibrillary cores

A

PNET

64
Q

Numerous osteoclast-like multinucleated giant cells mixed with mononuclear stromal cells

A

giant cell tumor of bone (osteoclastoma)

65
Q

where do most giant cell tumor of bone arise

A

epiphyses of long bones around knee; proximal humerus and distal radius

66
Q

age of giant cell tumor of bone

A

20-55 (mature bone)

67
Q

what tumor of the bone may present with arthritis symptoms

A

giant cell tumor of bone

68
Q

age group for fibrous dysplasia

A

young 10-30

69
Q

what does xray look like for fibrous dysplasia

A

radiolucent in diaphysis

70
Q

Morphology is showing curvilinear spicules of immature woven bone (chinese characters) surrounded by fibroblastic proliferation

what is it

A

fibrous dysplasia

71
Q

what does fibrous dysplasia result from

A

somatic mutation occurring during embryonic development

72
Q

what bone tumors are malignant

A

All sarcomas

giant cell tumor

Ortho (9 decks)

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