bone pathology Flashcards

1
Q

bone forming cells

A

osteoprogenitor cells
osteoblasts
osteocytes (quiescent /mature osteblast)

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2
Q

bone composition

A

type of connective tissue
(35%) 1/3 organic - cells/collagen and osteoid (proteinaceous matrix)
(65%) 2/3 inorganic - calcium hydroxyapatite

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3
Q

calcium hydroxyapatite

A

bones inorganic matrix - gives the hardness properties
storehouse for 99% of bodies calcium
85% pf phosporus
65% of body’s sodium and magnesium

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4
Q

is osteoid unmineralized or mineralized

A

unmineralized - organic

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5
Q

microscopically two types of bones

A

woven bone -

lamellar bone

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6
Q

what activates /regulates osteoclasts?

A

PARACRINE MECHANISM REGULATING OSTEOCLASTS –> with RANK receptor and RANK ligand –> interaction causes activation of transcription factors that activate the precursor cell to differentiate into an osteoclasts

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7
Q

T/F osteoblasts have a receptor for RANK

A

this is true - so we see a balance in the paracrine signaling

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8
Q

osteoprotegrin

A

decreases the osteclasts formation by preventing Rank ligans from interacting with the receptor

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9
Q

receptors on osteoblasts

A

for hormones like PTH. vitamin D, estrogen, cytokines, growth factors, and ECM matrix proteins

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10
Q

epiphysis

A

ends of the long bones where we can see growth plate and have production of bone

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11
Q

metaphysis

A

flairing of the epiphysis

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12
Q

diaphysis

A

central core part of the bone - between the epiphysis

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13
Q

layers of bone

A

periosteum –> compact (hard and thick) –> medullary bone–> that has the trabecular network of bone with the bone marrow between the networks) this is SPONGY BONE

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14
Q

BONE MASS CONTINUUM

A

peaks at age 30

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15
Q

Achondroplasia

A

Most common form of dwarfism
developmental disorder in bone
due to a POINT mutation in the FGFR3 (fibroblast grwoth factor receptor 3)

effecting all bones that form from endochondrial ossification because the FGFR3 is inhibiting chondrocyte proliferation so we dont see growth in the epiphyseal plates really -stunting the growth of long bones

normal thorax/trunk but do have bowing of legs, lordotic posture, bulging forehead
inherited in autosommal dominant manner

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16
Q

Thanatrophic dwarfism

A

more severe than achondroplasia and also is due to a mutation in FGFR3 - but different one - also see reduce in thorax size which compromises the ability of the lungs to expand and this is lethal and will die from a respiratory failure

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17
Q

diseases of decreased bone

A

Osteogenesis imperfecta –> defective type 1 collagen so aka brittle bone disease - DENTIN IN TEETH ALSO COMPROMISED

Osteoporosis -decrease in bone mass with primary (age and onset of menopause) and secondary causes

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18
Q

types of osteoporosis

A

disuse - localized like having a cast

metabolic bone - generalized - more common and associatiated with post menopausal woman and elderly

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19
Q

maximize bone mass/ density

A

try and do this before age 30 to increase peak bone density
nutrition - sufficient amounts of calcium and vitamin d supplements
exercise

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20
Q

bisphosphonate

A

decreases bone resorption

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21
Q

diagnosis of osteoporosis

A

radiographic technique that screen the bone density

dual energy absorptiometry and quantatiative computed tomography

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22
Q

diseases associated with osteoclast dysfunction

A

osteopetrosis and paget’s disease

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23
Q

Paget Disease

A

prominent CEMENT LINES

24
Q

diseases associated with abnormal mineral homeostasis

A

Rickets/osteomalacia

hyperparathyroidism

25
Q

osteonecrosis and causes

A

aka avascular necrosis
- osteonecrosis is an ischemic event with resultant bone and marrow infarction (loss of blood supply)

caused by sickle cell disease, vascular compression or disruption following a fracture, thromboembolic event, treatment with steriods, and vasculitis

26
Q

two types of osteomyelitis

A

bacteria (pyogenic)

Tuberculous

27
Q

pyogenic osteomyelitis

A

inflammation of the bone marrow due to infectious etiology
starts out as an acute systemic illness
Staphylococcus aureus is the most frequent bacteria causing this

caused by three ways

  1. hematogenous dissemination - infection through blood stream
  2. extension from an infection in an adjacent joint or soft tissue
  3. traumatic implantation after compound fractures or orthopedic procedure
28
Q

pyogenic osteomyelitis

A

inflammation of the bone marrow due to infectious etiology
starts out as an acute systemic illness
Staphylococcus aureus is the most frequent bacteria causing this

caused by three ways

  1. hematogenous dissemination - infection through blood stream
  2. extension from an infection in an adjacent joint or soft tissue
  3. traumatic implantation after compound fractures or orthopedic procedure
29
Q

bacteria involved in osteomyelitis

A

STaph. aureus
E. coli and group B strep in neonates
salmonella (common in sickle cell disease)
mixed bacteria including anaerobes - likely in bone trauma

30
Q

two types of osteomyelitis and main causes

A

bacteria (pyogenic)
Tuberculous

pyogenic Bacteria and mycobacterium tuberculosis are the most common causes

31
Q

pyogenic osteomyelitis clinical findings

A

characteristic radiograph findings such as destructive lytic focus surrounded by a sclerotic rim

biopsy and bone culture usually rewuired to identify pathogen

32
Q

Gardner’s syndrome

A

when there are multiple osteomas present (especially in mandible) this may be the cause

33
Q

Osteoma

A

benign overgrowth of bone most commonly seen in the head and neck region.
usually solitary, however when multiple present (like in mandible may be gardners syndrome) and could cause compression - but not locally invasice or malignant

34
Q

Gardner’s syndrome

A

when there are multiple osteomas present (especially in mandible) this may be the cause

35
Q

Osteoid osteoma and osteoblastomas

A

benign bone-producing neoplasms with similar histological features
2nd and 3rd decade
involve the cortex
osteoid osteoma - smaller (less than 2 cm) and localized bone pain can be treated with aspirin
osteoblastomas - present with diffuse bone pain usually in the vertebral area and bigger than 2cm - aspirin does not relieve the pain

36
Q

Osteoid osteoma and osteoblastomas

A

benign bone-producing neoplasms with similar histological features
2nd and 3rd decade
involve the cortex
osteoid osteoma - smaller (less than 2 cm) and localized to the proximal femur and tibia bone pain can be treated with aspirin
osteoblastomas - present with diffuse bone pain usually in the vertebral area and bigger than 2cm - aspirin does not relieve the pain

37
Q

osteosarcoma

A

bone-producing malignant mesenchymal tumor
most common behind myeloma and lymphoma in primary tumor of the bone
bi-modal age distribution
75% occurring before age 20 and second peak in elderly
show specific anatomical involvement with 60% occuring at the knee region (proximal tibia with distal femur) 15 % in iliac bone
10% in proximal humerus
8% in the jaw (mandible)

most ostecarcomas occurring in the metaphysis

38
Q

primary and seconday osteosarcomas

A

primary - young patients with no known specific underlying cause

secondary - more common in elderyl and could be from Paget disease or previous radiation exposure

39
Q

genetic mutations seen in osteosarcomas

A

Retinoblastoma gene (RB) mutated in 60-70% of tumors
p53 mutations
cylcins, cyclin-dependent kinases, kinase inhibitors

40
Q

primary and seconday osteosarcomas

A

primary - young patients with no known specific underlying cause (most common type)

secondary - more common in elderyl and could be from Paget disease or previous radiation exposure

41
Q

genetic mutations / pathogenesis seen in osteo-sarcomas

A

Retinoblastoma gene (RB) mutated in 60-70% of tumors
p53 mutations
cylcins, cyclin-dependent kinases, kinase inhibitors

42
Q

characteristics of primary osteosarcoma

A

solitary, intramedullary, poorly differentiated, and producing a predominately bony matrix

treatment yields 60-70% survival
first treat with chemo then limb-salvage - excising the bone completely

secondary with metastasis does not respond well to treatment

43
Q

characteristics of primary osteosarcoma

A

solitary, intramedullary, poorly differentiated, and producing a predominately bony matrix - most in the metaphysis

treatment yields 60-70% survival
first treat with chemo then limb-salvage - excising the bone completely

secondary with metastasis does not respond well to treatment

44
Q

most likely metastasis for bone cancer

A

lung - 10/20% of first time diagnosis will present with pulmonary metastases

45
Q

cartilage forming tumors

A

osteochondroma
chondroma
chondrosarcoma

46
Q

osteochondroma

A

benign cartilage capped outgrowth attached by a bony stalk to the underlying bone.
cap is composed of hyaline cartilage resembling a disorganized growth plate undergoing endochondral ossification

47
Q

chondroma and two names depending on region

A

benign tumors of hyaline cartilage
arise within medulla = enchondromas
arise on the bone surface = juxtacorticol chondromas

48
Q

chondrosarcoma location and defintion

A

malignant tumor of cartilage - made of malignant chondrocytes
common in the pelvis, shoulder, and ribs- presenting with painful, progressively enlarging masses

arise within the medullary cavity of the bone to form an expansile glistening mass that erodes the cortex

49
Q

clinical aspects / manifestations/ details about chondrosarcoma

A

eroding the cortex
malignant chondrocytes show atypia in the form of hyperchromatic nuclei and multinucleation
matrix is composed of hyaline cartilage and myxoid cartilage

50
Q

tumor grade determination of chondrosarcomas

A

grade 1,2,3 determined by cellularity, cytologic atypia, and mitotic activity
low grade - resembles normal cartialge
higher grade - contain pleomorphic chondrocytes with frequent mitotic figures

51
Q

correlation between grade and biologic behavior of a chondrosarcoma tumor

A

DIRECT correlation
low grade - 80-90% chance survival and rarely metastasize

vs 43% chance in high grade -

bigger more aggressive (larger than 10 cm) and can metastasis to lungs and distant bone

52
Q

Ewing Sarcoma

A

primary, malignant small round-cell tumor of bone.
PEDIATRIC TUMOR seen between ages 10-15 years old.
Caucasian population >
femur diaphysis or pelvic flat bones

approximately 95% of patients have TRANSLOCATIONS OF t(11;22) (q24;q12) or t(21;22)(q22;q12)

53
Q

PNET

A

primitive neuroectodermal tumor - close relationship to Ewing’s sarcoma but involves soft tissue and more neural differentiation

54
Q

metastatic disease

A

metastatic tumors involving bone is more common than a primary bone tumor.
in adults more than 75 % of skeletal metastasis originate from cancers in the prostate, breast, kidney, and lung

55
Q

Common causes/sources of pediatric bony metastases

A

neuroblastoma, Wilm’s tumor, osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma