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Path Bone
KYCOM Block 12
197
Pathology
Graduate
11/29/2013

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Cards

Term
what are the 6 cells/components of bone turn over
Definition
matrix: type 1 collagen

cytokiines and GF deposited at some point and are liberated when needed vi osteoclast

resistant acid phosphatases

osteoblasts and osteoclasts
Term
osteoblast: function, origin
Definition
build bone
come from mesenchymal cells
Term
osteoclast: function, origin
Definition
reabsorb bone
monocyte cell line in marrow
Term
osteoclast MOA
Definition
ruffled border to cause acidic area where they make contact and resorb bone
Term
what are the 10 signalers in bone turn over
Definition
IL1
IL6
TNF
RANK Ligand
M CSF
estrogen
PTH
osteoprotagerin
calcitonin
bisphosphates
Term
what is the function of IL1 IL6 TNF in bone turn over
Definition
stimulate reabsorption by stimulating osteoblasts which turn on osteoclast
Term
what is the function of RANK L and M CSF in bone turn over
Definition
made on osteoblast and presented to osteoclast to tall them to resorb bone
Term
what is the function of estrogen in bone turn over
Definition
favors bone building
Term
what is the function of PTH in bone turn over
Definition
stimulates reabsorption
Term
what is the function in calcitonin in bone turn over
Definition
not much unless it is from another species
inhibits osteoclast
Term
what is the function of bisphosphates in bone turn over
Definition
inhibit osteoclast
Term
5 bone congenital diseases
Definition
bone dystrophies
bone dysplasia
acondroplasia
osteogenesis imperfecta
osteopetrsis / marble bone disease / albers-schonberg disease
Term
cause of bone dysostoses
Definition
congenital malformations: defect in nuclear proteans and transcription factors (usually homeobox genes and some cytokines)
Term
4 examples of bone dysostoses
Definition
failure of development: rib, phalanx, clavicle
supernumerary bones: extra ribs and digits
syndactyly: fusion of digits
craniorachischisis: failure of skull / spinal column to close
Term
what is a complication of craniorachischisis
Definition
CNS and meningies herniate through opening causing meningomyelocele or meningoencephalocele
Term
cause of bone dysplasias
Definition
mutation in matrix or cell signaling affects all cartiladge and bone
Term
4 examples of bone dysplasias
Definition
achhondroplasia
thanatrophic dwarfism
osteogensis inperfect
osteopetrosis
Term
in genera, what is achondroplasia
Definition
disorder of structure of growth of cartilage causing chondrodysplasia
Term
what is the most common disease of the growth plate
Definition
achondroplasia
Term
what is the inheritence of achondroplasia
Definition
dominant
Term
what causes achondroplasia
Definition
inherited fibroblast GF receptor 3 mutation

receptor has influence on matrix, inhibits cartilage proliferation to keep growth in check

point mutation causes activation that supresses growth in bones formed by endochondral ossification (esp long bones)
Term
13 signs of achondroplasia
Definition
large head

frontal bossing

hypoplastic nose bridge

base of skull underdeveloped

posterior elements of spine underdeveloped

narrow spinal canal

narrow neural foramina

short tubular bones

bowing tibia, femur, humerus

premature deposition of bone seals growth plate

zone of proliferation and hypertrophy in growth plates narrowed and disorganized

clusters of chondrocytes instead of growth plate columns
Term
what are the two types of achondroplasia
Definition
homozogyous achondroplasia
thanatrophic dwarfism
Term
cause of homozygous achondroplasia and inheritence
Definition
two abnormal FGFR3 genes
autosomal domonant
Term
thanatophoric dwarfism: cause, clinical signs
Definition
frameshift or point mutation in FGFR3 that is more severe, often due to offspring of two dwarves

most common form of lethal dwarfism, respiratory insufficiency due to underdeveloped thoracic cavity
Term
in general, what is osteogenesis imperfecta
Definition
disorder of type I collagen synthesis causing too little bone resulting in skeletal fragility
Term
what is the most common genetic disease of the skeleton
Definition
osteogenesis imperfecta
Term
what are the main areas of the body affected in osteogenesis imperfecta
Definition
90% in osteoid protein in bone
joints, EYES, ears, skin, TEETH
Term
cause of osteogenesis imperfecta
Definition
mutation in type I collagen synthesis a1 (COL1A1 CH17) or a2 (COL1A1 CH7) chain triple helix mutation or both
Term
what are the two types of mutation s in osteogenesis imperfecta
Definition
null allele effect (1/2 normal collagen): mutant gene not transcribed or mutant chain degraded

dominant negative: more severe chain abnormalities compormise function usually due to glycine substitution. most severe in C terminus, less severe in smaller AA still allowing helix
Term
what is the most common cause of osteogenesis imperfecta and its inheritence
Definition
type I
autosomal dominant
Term
what is the most severe form of osteogenesis imperfecta and its inheritence
Definition
type II
autosomal recessive
Term
what is the intermediate and least common form of osteogenesis imperfecta and its inheritence
Definition
type IV
autosomal dominant
Term
signs of osteogenesis imperfecta type I
Definition
diagnosed middle age due to osteopenia and fracture history
normal stature in 50%
hearing impairment in 25%
skeletal fragility
blue sclerae
Term
why is sclerae blue in osteogenesis imperfecta
Definition
translucent sclera so can see choroid
Term
signs of osteogenesis imperfecta type II
Definition
excessive fragility especially during birthing, often die in utero or within days of birth
blue sclerae
Term
signs of osteogenesis imperfecta type III
Definition
growth retardation (<4ft)
multiple fractures (~200 as adult)
limb bowing
blue sclera
hearing impairment
Term
signs of osteogenesis imperfecta type IV
Definition
moderate skeletal fragility
short stature
intermediate symptoms
Term
signs of osteogenesis imperfecta upon birth
Definition
big forehead
deafness
white of eye blye
poor teeth
barrel chest
round back
Term
in general, what is osteopetrosis
Definition
decreased bone reabsorption causes radiodense bones (bone keeps getting built)
Term
causees of osteopetrosis
Definition
carbonic anhydrase II deficiency osteoclasts
mutation in CIC-7 osteoclasts
macrophage colony stimulating factor (M-CSF) deficiency
Term
explain how carbonic anhydrase deficiency causes osteopetrosis
Definition
needed by osteoclast for renal tubular cells to excrete H+ and acidify area
mutation prevents acidification of resorption put and solubulization of hydroxtapatite favoring bone building
Term
explain how mutation in CIC-7 osteoclasts cause osteopetrosis
Definition
Cl- channel gene helps proton pumo of ATPase on osteoclast ruffled border
osteoclasts cannot acidify resbsorption pit favoring bone building
Term
how does M-CSF mutation cause osteopetrosis
Definition
osteoblasts cannt make M-CSF for osteoclast differentiation
osteoclasts are reduced
Term
signs of osteopetrosis
Definition
high bone densiry
fragile bones
erlenmeyer fkast long bones
rugger hersey vertebraw (also hyperparathyroidism)
bones of skull thick and sclerotic
CN compression, blindness, deafness
hair on calvarium (skull hematopoesis)
primary spongiosa persists and fills medullary cavity leaving no room for hematopoietic marrow leading to anemia
Term
treatment of osteopertrosis
Definition
marrow transplant - not effective in dosorders where osteoclasts are dysfunctional

high dose vitamin D

INFy stimulates osteoclast superoxide
Term
define osteoperosis
Definition
increased porosity of skeleton that esults from decreased bone mass causes predisposition to fracture
Term
what are the risk factors for osteoperosis
Definition
post menopausal
white
female
(men and african americans start out with higher level of bone density to begin with)
Term
what are 6 causes of osteoperosis
Definition
receptor mutation
decreased physical activity
post-menopause
vitamin D deficiency
aging
prolonged glucocorticoids
Term
what receptor mutations lead to osteoperosis
Definition
vit D, estrogen, IGF-a, COL1A1
Term
why does osteoperosis happen post menopause
Definition
decreased estrogen decreases IL1, 6, TNF which increases RANK and RANKL which increases bone reabsorption, osteoblast activity goes down and osteoclast up
Term
how does age cause osteoperosis
Definition
decreased replication of osteoprogenitor cells
decreased osteoblast activity
reduced physical activity
Term
how do glucocorticoids cause osteoperosis
Definition
stimulate bone reabsorption
reduce bone formation on cortical and trabecular surfaces
Term
how can glucocorticoid osteoperosis be avoided
Definition
keep therapy short term
bisphosphate administration minimizes bone loss
Term
clinically, how can glucocorticoid therapy be distinguished
Definition
osteoperosis develops rapidly
Term
what are the osteoperosis tests, which is better, why
Definition
bone density testing - better, more acurate
heel ultrasound - fast, cheap, not acurate
Term
what is the WHO recomendation on osteoperosis testing
Definition
recommends density test in all women >65 and younger women with risk factors
Term
what does bone density testing measure, why
Definition
hip, vertebrae, and wrist because they are most prone to fracture
Term
what are the components of a bone density testing reading
Definition
T score: compares to young adult mean, more usefuk

Z score: compares to others in same age group
Term
what is a normal bone density test
Definition
T > -1 within one standard deviation of young adult mean
Term
what is a low / osteopenic bone density test
Definition
T = -1 to -2.5 with no predisposition to fracture
>2.5 standard deviations below mean
Term
what is a severe / osteoperosis bone density test
Definition
>2.5 standard deviation below young adult mean and one or more osteoporotoc fractures
Term
signs of osteoperosis
Definition
vertebral compression fracture leading to lumbar lordosis/kyphoscoliosis

wrist, femur neck, head of vertebrae are most common fractures due to aging

complications of pulmonary embolism and pneumonia

Ca P, and alkaline phosphatase levels (not diagnostic)
Term
how is osteoperosis prevented
Definition
exercise
Ca
vit D
bisphosphonates
estrogen replacing agents
Term
cause of sarcoidosis
Definition
multisystemic disease unknown cause likley autoimmune
Term
who is most at risk for sarcoidosis
Definition
adults <40, danish, swedish and US blacks 10x>US whites
Term
define sarcoidosis
Definition
noncaseating granuloma in many organs and tissues
Term
regions of involvement in sarcoidosis and the frequency
Definition
noncaseating epitheloid granulomas
hilar lymphadenopathy 75-90%
lung involvement 90%
spleen involvement 75%
bone involvement 20%
skin lesions 33-50%
eye, eye gland, salivary glands 20-50%
muscle
Term
what are the components of a noncaseating granuloma
Definition
multinucleated giant cells
schaumann bodies: laminated concentration of Ca and protein
asteroid bodies: stellate inclusions inside giant cells
Term
what is the major presenting manifestation in most sarcoidosis cases
Definition
hilar lymphadenopathy
Term
what occurs in spleen in sarcoidosis
Definition
small granulomas coalesce to form small nodules
Term
what occurs in bone in sarcoidosis
Definition
granulomas produce PTH like hormone, TNF, IL6 enhancing vitamin D activation increasing osteoclast activity

enhances bone rabsorption that is most detectable in hands in eet

high Ca levels in serum
Term
what are signs of sarcoidosis in xray
Definition
fine reticular patterns
total destruction of phalanges seen in advanced stages
Term
what occurs in skin in sarcoidosis
Definition
erythematous plaques, scaling, lesions of mucous membrane
Term
what occurs in eye in sarcoidosis
Definition
corneal opacities, glaucoma, total vision loss
Term
what occurs in muscle in sarcoidosis
Definition
weakness, ache, tenderness, fatigue
Term
causes of vitamin d deficiency
Definition
inadequate sun exopsure
decreased intake, absorption, metabolism
poor Ca or P homeostasis

rare in developing countries, elderly most susceptible
Term
what occurs as a result of a vitamin d deficiency
Definition
decrease in Ca and P absorption in gut

hypocalcemia increases PTH activating renal a-hydroxylase which causes Ca absorption in gut, Ca reabsorption in kidney, and P excretion in kidney

Ca serum levels are near normal, hypophosphatemia causes impaired bone mineralization
Term
what are the two types of vitamin d deficiency, what is the difference
Definition
osteomalacia: growth plates closed so bone isnt deranged but osteoid matrix is inadequatly ineralized and susceptible to fracture

ricketts: deficiency before plates close so epiphyseal cartiladge is inadequatly calcified causing dernaged bone growth
Term
what are the areas most susceptible to fracture in osteomalacia
Definition
vertebral bodies, femoral neck
Term
histology signs of osteomalacia, distinguish from osteoperosis and osteopenia
Definition
unmineralized osteoid visible on H&E aranged around mineralized trabeculae (rather than loss of skeletal mass like in osteoperosis and osteopenia)
Term
what would the Ca and P levels be like in osteomalacia
Definition
low vitamin D, low ionized Ca by 40%, low P

later: elevated PTH and normal Ca

increased alkaline phosphatase in 94% due to upregulation from Ca and P levels
Term
what test can be done to confirm osteomalacia
Definition
decalcified bone biopsy: give tetracycline and if there is no space between the two doses you get no gap and they have osteomalacia
Term
what are the histological signs of rickets
Definition
overgrowth of epiphyseal cartiladge cells with projection of irregular cartilage masses into marrow

deposition of osteoid matrix into cartilage remnants

enlargment and expansion of osteochondral junction

overgrowth of capillaries and fibroblasts due to microfractures

inadequate calcification of epiphyseal cartilage causing deranged bone growth
Term
signs of rickets in head
Definition
occipital flat
parietal buckle inward
frontal bossing: excess osteoid/square head
soft spot slow to close
bony necklace
Term
signs of rickets in chest
Definition
rachitic rosary: overgrowth of cartilage or osteoid at costochondral junction
pigeon brest deformity due to pull of respiratory muscles
harrisons groove; from inward pull od diaphragm
Term
what are ambulatory signs of rickets
Definition
mottledn enamel due to dentin effects
deformities mostly in spine, pelvis, long bone
bowing of legs, bulging of wrists, big lumpy joints
Term
what is the cause of hyperparathyroidism
Definition
uncontrolled PTH (maybe parathyroid tumor)
Term
how is hyperparathyroidism diagnosed
Definition
x-ray pattern virtually diagnostic: thinned cortices in radial middle phalangies of the index and middle finger
Term
4 signs of hyperparathyroidism
Definition
generalized osteotis fibrosa cystica
dissecting osteitis
peritrabecular fibrosis
cystic brown tumor
Term
what is dissecting osteitis
Definition
osteoclasts tunnel through trabecular
Term
what is peritrabecular fibrosis
Definition
marrow replaced by fibrovascular tissue
Term
what causes cystic brown tumor, what are the concerns with this
Definition
brown due to hemosiderin deposition

bone loss perdisposes to microfracture and secondary hemorrhage so macrophages come and make mass of reactive tissue
Term
what is the cause of renal osteodystrophy
Definition
chronic renal failure is ALWAYS complicated by wide spread bone disease
kidney can no longer regulate Ca and P metabolism causes hyperphosphatemia and secondary hyperparathyroidism that increases osteoclast activity
Term
explain the pathology of renal osteodystrophy
Definition
kidney cannot get rid of P causing secondary hyperparathyroidism and increasing osteoclast activity

hypocalcemia causes kidney damage and stops vitamin D activation so Ca cannot be absorbed

metabolic acidosis: stimulates Ca hydroxyapetite release from matrix and bone reabsorption
Term
what is aluminum bone disease, and the cause
Definition
may come from dialysis

Al interferes with deposition of Ca hydroxyapatite causing deposition of amyloid in bone (long term hemodialysis leads to B2-microglobulin)
Term
in general, what is paget's disease
Definition
collage of matrix maddness: gain in bone mass but new bone is highly disorganized
Term
risk factors of paget's disease
Definition
mid adult and likleyhood increasing with age

white female

england, france, austria, germany, australia, new zeland, US

rare in japan, china, africa, scandinavia
Term
what are the stages of pagets and the activities in each
Definition
osteolytic: increased osteoclast activity,lytic lesions

mixed osteoclastic-osteoblastic: ends facoring bone formation

osteosclerotic: quiescent, lack of remodeling causes deformity
Term
cause of pagets
Definition
paramyxovirus infection
osteoclasts hyperresponsive to vit D and RANKL
antigens found to measles, RSV
particles like necleocapsids in cytoplasms and nuclei of paget osteoclasts
Term
how does paramyxovirus cause pagets
Definition
induces secretion of IL6 which happens in pagets disease
Term
how does measles cause pagets
Definition
virus transcripts in affected osteoclasts
Term
most common affected bone in pagets
Definition
vertebrae, humerus, femur head, femur, tibia
Term
what are the signs in bone in pagets
Definition
pain, deformity, chalk stick, compression fracture, neoplasm
platybasia: compression of posterior fossa sutures
teeth problems
lentiasi ossea: lion shaped head, large head difficult to hold up
secondary osteoarthritis
Term
what are the non bone signs of pagets
Definition
CN compression
deafness
blindness
hypertrophic heart with high output failure
hypervascularity causes shunt leading to heart failure
Term
pagets increases tumor probability, what are the two types of tumors and their defining characteristics
Definition
benign: giant cell reparative granuloma, extraosseous hematopoietic masses

malignant: OSTEOSARCOMA, malignant fibrous histocytoma, chondrosarcoma
Term
what are some radiologic signs of pagets
Definition
mosaic lamellar bone: haphazard cement lines
lytic lesions: nelarged misshapen bone
large, numerous osteoclast nuclei
marrow replaced by loose CT and reformed in osteosclerotic stage
large bones with thick trabecular
Term
what will the blood levels of Ca and P look like in pagets
Definition
increased alkaline phosphate
Term
what does the bone scane look like in pagets
Definition
hydrpxyproline: areas of bone remodeling
Term
tx pagets
Definition
celcitonin, bisphosphate
Term
causes of osteonecrosis
Definition
fracture
corticosteroids
thrombosis and embolism
vessel injury
vascular compression
venous HTN
Term
associated conditions with osteonecrosis
Definition
pregnancy, trauma, corticosteroids, infection, dysbarism, radiation therapy, CT disorders, gauchers disease
sickle cell, anemia, alcohol abuse, chronic pancreatitis, tumors, epiphyseal disorders
Term
signs of osteonecrosis
Definition
cancellous bone and marrow involvement
chest pain
predisposition to osteoarthritis
Term
what is the cause of 10% of all joint replacements
Definition
osteonecrosis via osteoarthritis
Term
2 types of osteonecrosis
Definition
medullary infarction: most stable
subchondral infarction
Term
subchondral infarction: shape, location, composition
Definition
wedge: due to vessel pattern

over articular cartilage getting nutrients from synovial fluid

dead bone with necrotic adipocytes around it
Term
pathogenesis of subchondrial infarction
Definition
frequently reuptues releasing FA and binding Ca causing Ca soaps

creeping substitution: osteoclasts reabsorb dead bone, osteoblasts cannot keep up replacement so articular cartilage collapses and soloughs off
Term
define osteomyelitis
Definition
inflammation of bone and marrow ususlly due to infection (or secondary infection like TB, but usually primary infection)
Term
causes of osteomyelitis and where they get picked up
Definition
staph aureus: most common, injury

E. coli, pseudomonas: GU tract

H. influenza, GBS: neoates infector

salmonella: sickle cell pt
Term
routes of infection for osteomyelitis
Definition
hematogenous spread- most common
extension from contigous site
direct implantantion (prostetic)
Term
what is the pathology of osteomyelitis in kids
Definition
spreads to subperiosteum causing it to lift
interrupting blood supply causing necrosis and sequestration
rupturing periosteum causing abscess in soft tissue
Term
what is the pathology of osteomyelitis in infants
Definition
epiphyseal infection usually
spreads to joints causing septic/suppurative arthritis
Term
what is the pathology of oseeomyelitis in adults
Definition
necrosis due to inflammatory reaction within 48h

inflammatory cells attract cytokines stimulating osteoclasts to resorb bone increasing fibrous tissue causing sclerotic rim around lesion on x-ray

involucrum: reacrive bone in a sleeve of dead bone
Term
what are clinical and x-ray signs of osteomyelitis
Definition
sclerotic rim around lesion: fibrous tissue

involucrum: reactive bone in sleeve of dead bone

malaise, fever, chills, leukocytosis
infants: just fever
Term
what are the diagnostic tests for osteomyelitis
Definition
blooc culture: only finds 50% of microbes

biopsy: do whtn culture is negative or antibiotics are not working
Term
what is the prognosis of osteomyelitis
Definition
5-25% persist and cause chronic infection: falure ups, pathologic fracture, secondary amyliodisis, endocarditis, sepsis, squamous cell carcinoma in sinus, sarcoma (rare)

CAUSES CANCER!
Term
TB sometimes spreads to bone, where does it go, why is it a big concern
Definition
very destructive
usually in spine (pott disease), knee, hip
Term
define -oma
Definition
benign
Term
define -sarcoma
Definition
malignant tumor from mesenchymal tissue
Term
define osteoblastoma
Definition
benign tumor of osteoblasts
Term
define chondrosarcoma
Definition
cartiladgenous malignant tumor
Term
what type of bone tumors cause pain
Definition
benign pressing on something - uncommon
growing lesions
agresive osteoblastoma
giant cell tumor
malignant tumor: fast growht, destruction
pathologic fracture complication - benign or malignant
signigicant loss of tissue due to tumor
Term
who is at risk for metastasis to bone
Definition
<10 yo
>40 yo
Term
what is the most common location and presentation of metastasis to bone
Definition
multi focal lesions in ribs, vertebrae, pelvis, cranium, proximal long bones

AXIAL
Term
what is the uncommon location and presentation of metastasis to bone
Definition
solitary lesions
thyroid, lung, kidney
Term
what are the cancers that usually metastasize to bone
Definition
prostate
thyroid
lung
kidney
breast
Term
where does cancer from bone metastasize to
Definition
usually lungs
Term
who usually gets bone tumors
Definition
teens and twenties (first 3 decades)
Term
are bone tumors usually benign or malignant
Definition
benign
Term
where do most primary bone tumors originate (benign or malignant)
Definition
distal femur, proximal tibia
Term
what are two characteristics of bone cancer that are unique and important
Definition
de-differentiation to more anaplastic (agressive) type

high grade sarcome comes from damaged bone
Term
what are two examples of tumor de-differentiation
Definition
enchondroma
low grade chondrosarcoma transforming into high grade
Term
what are 3 examples of high grade sarcome coming from damaged bone
Definition
bone infarct site
radiation osteitis
paget's disease
Term
what are the characteristics of a non-growing lesion
Definition
sclerotic rim
95% benign
Term
what are the characteristics of a slow growing lesion
Definition
well demarcated zone of bone destruction without sclerotic rim

provoke focal cortical thinking (solid periosteal reaction)

with continued growth such lesions may show cortical expansion
Term
what are the characteristics of a rapidly growing lesion
Definition
penetrate through cortex causing seperation of periosteum (codman's triangle)
hair on end or sunburst pattern
moth eaten appearance
Term
osteoma: locations, associated conditions, malignant or benign
Definition
mandible usually, skull, long bone

often seen with clonic polyposis (familial adenomatous polyposis) which is cardner syndrome
osteome usually comes before radiological signs of clonic polyposis

benign
Term
osteoid osteoma: malignant or benign, clinical signs, epidemology
Definition
benign

painful, worse at night

males > females (2:1)
Term
osteoid osteoma: pathology
Definition
releases COX causing pain (relieved by aspirin)

growth in diaphysis of long bone (<2cm lesion of cortex)

Xray shows central radionuclency surrounded by sclerotic rim
Term
lesions in intramedullary diaphysis
Definition
eqings sarcoma
enchondroma
Term
lesions in cortex diaphysis
Definition
osteoid osteoma
Term
lesions in metaphysis cortex
Definition
non-ossifying fibroma
Term
lesions in metaphysis intramedulary
Definition
osteosarcoma
chondrosarcoma
osteoblastoma
Term
lesions in metaphyseal exostosis
Definition
osteochondroma
Term
llesions in epiphysis
Definition
chondroblastoma in open growth plate
giant cell tumor: often crosses into metaphysis, only in closed growth plate
Term
what condition is similar to osteoid osteoma
Definition
osteoblastoma
Term
where does osteoblastoma occur
Definition
vertebrae or metaphysis of long bone
Term
signs of osteoblastoma
Definition
dull pain, not relieved by aspirin
no sclerotic rum (growing too fast)
Term
who gets osteochondroma(exostosis), what bones
Definition
adolescents
long bones esp at knee
Term
physiology of osteochondroma
Definition
come from metaphysis near growth plate of tubular bones of endochondral origin

made of BONE and CARTILAGE

usually solitary lesion
Term
clinical signs of osteochondroma
Definition
asymptomatic or painful
produce deformity
Term
is osteochondroma malignant or benign
Definition
benign
but it can undergo malignant transformation
Term
tx osteochondroma
Definition
easy to excise because it is solitary and on top of bone but dont unless painful
Term
what os osteocondromatosis
Definition
multiple hereditary exostosis
osteochondroma with multiple often symmetric lesions
more likey to transform and be malignant
Term
chondroma malignant or benign, location
Definition
benign
hayline cartilage
endochondrial origin bones (hands, feet)
Term
endochondroma: define, location, age range
Definition
condroma of the medullary cavity
20-50yo
most common intraosseous tumor
Term
what is the most common intraosseous tumor
Definition
chondroma: endochondroma
Term
define endochondromatosis, associated conditions
Definition
multiple endochondroma
soft tissue hemangioma of endothelial cell orogin
Term
chondroblastoma location
Definition
epiphysis
epiphyses: iliac crest
Term
pathology chondroblastoma
Definition
disorder apparent in chondroblasts
cellular tomor with little hayline matrix
when calcifies looks like chicken wire
Term
signs if chondroblastoma
Definition
painful due to joint effusions causing limited mobility
x-ray: spotty calcifications in apiphysis
Term
who gets giant cell tumor
Definition
women?men
20-40yo
Term
giant cell tumor: location, malignant or benign
Definition
epiphysis but can spread to metaphysis
usually around knee
benign but can metastazise
Term
composition of giant cell tumor
Definition
osteoclast like giant cells and stroma
Term
signs of giant cell tumor
Definition
x-ray: expanding lytic lesions, demacrated, not sclerotic (cell in bone)

red brown mass with cystic degeneration
Term
what is giant cell tumor often confused with, why
Definition
red brown mass with cystic degeneration looks like brown tumor of hyperparathyroidism. here its due to high vascularization
Term
prognosis of giant cell tumor
Definition
surgical removal leaves with 40-60% reoccurance
4% spread to lungs
Term
what is a fibrous cortical defect
Definition
not neoplasm! developmental defect in 30-50% of kids >2yo
Term
signs of small fibrous cortical defect
Definition
<0.5cm
bilateral
maybe multiple
usually asymptomatic
spontaneously resolve in a few years replaced by normal bone
Term
signs of large fibrous cortical defect
Definition
5-6cm
aka non-ossifying fibroma
metaphyseal lesion with rim of sclerotic bone
usually involves cortex
Term
what is the most common primary malignant tumor of bone
Definition
osteosarcoma
Term
who gets osteosarcoma, where
Definition
teenagers
usually around knee (sometime hip)
Term
where does osteosarcoma come from
Definition
malignant mesenchymal tumor that makes bone matrix
Term
prognosis osteosarcoma
Definition
agressive
can become very large
Term
radiograph signs of osteosarcoma
Definition
codmans triangle: as tumor penetrates it seperates periosteum

sunburst pattern

bone destruction
Term
who gets chondrosarcoma
Definition
40yo+
Term
location of chondrosarcoma
Definition
AXIAL

pelvic bones, spine, shoulder, ribs, long bones
Term
pathology chondrosarcoma
Definition
atypical chondrocytes and chondroblasts with multiple nuclei in lacuna
produce neoplastic cartilage
Term
what are the grades of chondrosarcoma, how are they identified
Definition
more radiolucent higher grade

slow growing: reactive thickening of cortex

fast growing: no time for reaction, cortex destoried
Term
who gets ewing sarcoma
Definition
5-20yo white males
Term
signs of ewing sarcoma on xray
Definition
concentric onion skin layering of periosteum
Term
clinical signs of ewing sarcoma
Definition
painful, swelling
Term
location of ewing sarcoma
Definition
DIAPHYSIS of femur, pelvis, tibia
Term
histological signs ewing sarcoma
Definition
homer wright rosettes: tumors cells in circle around fibrillary space due to neural differention

sheets of undifferentiated blue cells resembling lymphocytes
Term
cause of ewing sarcoma
Definition
translocation of EWS gene to a member ot ETS family transcription factors and EWS acts as an oncogene activating chimeric protein stimulating cell proliferation

t(11;22)
Term
what condition is ewing sarcoma similar to, what is the difference
Definition
primitive neuroectodermal tumor (PNET)

ewing is less differetiated, everything else is the same
Term
prognosis of ewing sarcome
Definition
used to be poor
now 75% 5 year survival with chemo
50% long term cure
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