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139 Cards in this Set
- Front
- Back
What are the different parts of a tubular bone?
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Physis = growth plate
Diaphysis = shaft Metaphysis = flaredportion Epiphysis = end of bone, beyond the physis |
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What are the different histologic types of bone?
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Lamellar
-mature -perpendicular rows of collagen bundles Woven -immature |
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When do you see woven bone?
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Development and in pathological conditions!
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What are the properties of cortical bone? Where is it found?
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Dense, compact
Thickest in he shafts (diaphysis) of long bones |
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What are the properties of cancellous bone? Whereis it round?
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Spongy, trabecular
Compression resistant At the ends of long bones, also in vertabrae |
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What type of bone is more active: cancellous or cortical bone?
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Cancellous
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What is the vascular supply to the bone?
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Penetrating arteries
-Nutrient artery -Metaphyseal, epiphyseal arteries Periostealarteries -Augments the principal supply |
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What part of bone is solid? Water?
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92% solid
8% water |
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What are the components of the bone matrix?
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Type I collagen
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What are the mineral components of bone?
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Calcium hydroxyapatite
99% of body calcium, 85% of the phosphorus |
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What are the cell types inside of bone? What is their function?
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Osteoblast: forms osteoid
Osteocytes: Ca/PO4 homeostasis Osteoclasts: macrophages that resorb bone |
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What is wolff's law?
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It states that the internal structure of bone is altered in response to the magnitude of force placed upon it
More force, stronger bone! |
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What is the embryonic origin of the skeleton?
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Somites (which comes fromthe mesoderm), which divides intot he dermatomes, myotomes, and sclerotomes
Bone forms fromthe sclerotomes |
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What is the process of bone formation in long bones? Flat bones?
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Long bones (except for cranium, part of clavicle): from cartilage precursors through endochondral ossification
Flat bones: membranous ossification |
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What are the four zones of bone growth?
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Reserve
Proliferation Hypertrophy Ossification |
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What is the definition of a dysotoses?
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Localized problems in the migration or formation of bone
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What are the most common causes of dysostoses?
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Defects in nuclear proteins, TFs
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What does someone look like who has dysostoses?
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Missing, extra bones
Fusion of bones |
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What is the definition of a dysplasia of bones?
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Global skeletal abnormalities secondary to mutations of genes involved in regulation of skeletal development or structure
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What gene is mutated in achondroplasia, hypochondroplasia, and thanatatophoric dwarfism?
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FGFR3
Fibroglast growth receptor; functions in signal transduction |
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What gene is mutated in osteogenesis imperfecta?
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COL1A1
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What does someone look like with Achondroplasia?
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Short limbs, normally sized torso
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What is the cause of achondroplasia?
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Point mutation of FGFR3
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What's the inheritance of achondroplasia?
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Autosomal dominant
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What is thanatophoric dwarfism?
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Lethal form of dwarfism
Thorax can't develop-->no respiratory capacity |
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What mutation occurs in thanatophoric dwarfism?
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FGFR3 gain of function mutation
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What mutation takes placein osteogenesis imperfecta?
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Mutations impacting COL1 synthesis
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What are the clinical manifestations of osteogeneis imperfecta?
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1. Skeletal fragility
Blue sclera Small, brown-yellow teeth Hearing impairment |
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What is osteopetrosis?
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Genetic disease of osteoclasts!
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What is the treatment of osteopetrosis?
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Bone marrow treatment
CURATIVE |
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What are the symptoms of osteopetrosis?
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Diffuse, symmetric skeletal sclerosis
Brittle bones that can easily fracture Medullary cavity filed with immature bones Anemia, infections --> NO MARROW! |
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What do the bones look like in osteopetrosis?
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NO MARRO!
Erlenmeyer flask bones |
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What are some of the acquired abnormalities of bone structure?
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Osteoporosis (decreased bone mass)
Paget disease (increased bone mass) Abnormal mineral homeostasis -Rickets/osteomalacia (decreased vitamin D) -Hyperparathyroidism (decreased calcium-->increased osteoclast activity) -Renal osteodystrophy |
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What happens to the bones in osteoporosis?
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Reduced bone mass; "porpous" bones
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What are the risk factors for osteoporosis?
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Hereditary factors
Decreased physical activity Muscle strength Nutrition, Ca intake Drugs Hormones (esp. estrogen!) |
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What does the histology of osteoporosis look like?
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Bottom shows very POROUS bones
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What happens to the vertebrae during osteoporosis?
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Compression fractures!
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What happens to the bones in Paget disease of bone?
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Net gain in bone mass
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What are the phases of paget disease of bone?
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1. Osteolytic (osteoclastic)
2. Mixed osteoclastic/osteoblastic 3. Osteosclerotic |
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What does the histopathology of Paget disease of bone look like?
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Mosaic lamellar bone (prominent cement/bluish lines)
HUGE OSTEOCLASTS! Marrow fibrosis, hypervascularity |
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What is the most likely cause of osteomyelitis?
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Bacteria
Most likely S. aureus |
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What kind of bacteria causes osteomyelitis in neonates?
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H. influenza
Group B strep |
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What kind of bacteria causes Osteomyelitis in people with sickle cell disease?
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Salmonella
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What are some different ways that ostomyelitis can get spread to the bone?
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Hematogenous
Direct extension from a contiguous site -Diabetics with gangrenous digits -Decubitius ulcers Traumatic (direct) implantation |
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What is a complication from osteomyelitis seen in kids?
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The periosteum is loosely attached, so the growth can push it out
This then forms new bone, or an INVOLUCRUM |
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What does the histology of osteomyelitis look like?
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Dead, irregular bonewith pus
Dead osteocytes within the bone |
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What is osteonecrosis?
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Avascular necrosis/asepticnecrosis of the bonehead
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What are the mechanisms that can causes osteonecrossi/
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Trauma
Corticosteroids Thrombosis/embolism Vascular inury Alcoholism |
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What is a common complication folowing osteonecrosis?
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Secondary osteoarthritis
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What is the common pathway for fracture healing in bone?
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1. Hematoma
2. Inflammatory cell infiltration 3. Activation of osteoprogenitor cells 4. Granualtion tissue 5. Bony/hard callus 6. Remodeling and reconstitution |
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What are some things that can cause a fracture not to fuse?
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Poor immobilization
Infeciton Ischemia |
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What are the different kinds of boneformingtumors?
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Osteoid osteoma
Osteoblastoma Osteosarcoma |
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What are the different types of cartilage forming tumors?
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Osteochondroma
Chondroma Chondroblastoma Chondromyxoid fibroma Chondrosarcoma |
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What are some of the miscellaneous tumors of bone?
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Giant cell tumor
Aneurysmal bone cyst Non-ossifying fibroma Fibrous dysplasia Ewing sarcoma |
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What are some of the hematopoietic tumors of bone?
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Myeloma
Lymphoma |
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What kind of a primary bone tumor is the most common?
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Hematopoietic (40%)
Cartilage forming (22%) Bone forming (19%) |
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What type of a person gets an osteoid osteoma?
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Man (2:1 M:F) between 2nd and 3rd decate
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Where in the bone do osteoid osteomas occur?
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Inside the cortex
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What bones do osteoid osteomas usually occur in?
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Femur or tibia (50%)
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What size are osteoid osteomas?
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<2 cm
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What is helps with the pain from osteoid osteomas? Why?
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NSAIDS!
They produce prostaglandins, which cause pain |
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What's the treatmnet for osteoid osteomas?
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Radioablation is curative!
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What kind of a person gets an osteoblastoma?
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Man(2.5:1 M:F) in 2nd-3rd decade
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Where do osteoblastomas usually occur?
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medulla
posterior spine, sacrum (50%) Jaws |
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How large are osteoblastomas?
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>2 cm (< 2 cm is by definition an osteoid osteoma)
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What are the symptoms of osteoblstomas?
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Pain that's NOT relieved by NSAIDs
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What's the treatment of osteoblastomas?
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Curettage/excision
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What's the most common primary malignancy of bone?
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Osteosarcoma
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What are the risk factors for an osteosarcoma?
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Radiation
Paget's diseaseof bone INfarct |
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What is the most typical person to have an osteosarcoma?
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Man (1.5:1 M:F) in second decade
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How does someone with an osteosarcoma present?
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Pain +/-a palpable mass
Much less commonly, they'll present with a pathologic fracture |
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What's the treatment for an osteosarcoma?
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Chemo followed by a biopsy to see how much the tumor is undergoing necrosis
If a lot of necrosis is happening, then there's a good prognosis Also, resection of the tumor and masses |
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Where do osteosarcoma metsmostoftengo?
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The lung
Other bones |
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Where are osteosarcomas foundin the bone?
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90% metaphysis
10% in the diaphysis Most are in the medulla of the bones |
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What is an osteochondroma?
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It's a growth plate that has found itself in the wrong place and all of a sudden starts growing
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What type of a person most commonly gets an osteochondroma?
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Man (3:1 M:F) in 2nd -3rd decade
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What bones are most commonly involved in an osteochondroma?
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Distal femur (30%)
Proximal tibia (15%) Proximal humerus (15%) |
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What does someone with an osteochondroma present like?
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Palpable mass or asymptomatic
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What's the treatment of an osteochondroma?
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Excision is curative!
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What is an enchondroma?
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A piece of the growth plate that has wound up within the medulla of a bone
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What kind of a person gets an endhondroma?
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Male or female (1:1) in 3rd - 4th decade
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What'e the most usual place to find an enchondroma?
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Hands/feet (50%)
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What's the treatment for enchondroma?
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Observation or curettage
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What is it called if you have multiple enchondromas? How about multiple enchondromas + hemangiomas?
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Multiple = Ollier
Multiple + hemangiomas = Maffucci |
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What kind of a person gets a chondroblastoma?
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Male (2:1 M:F) in 2nd decade
THE SKELETON IS IMMMATURE |
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Where do chondroblastomas take place?
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Long bone epiphyses
-Femur, tibia, humerus -Talus, calcaneus |
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What types of cells are inside chondroblastomas?
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Giant cells
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What types of tumors of bone show giant cells? Which happens in what types of populations?
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Chondroblastoma: kids
Giant cell tumor: adults |
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What's the treatment for chondroblastoma?
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Curettage: scooping it out!
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Where do chondrosarcomas metastasize to?
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Lungs
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What are the properties of a giant cell tumor of bone?
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Giant cells!
Benign but locally aggressive |
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Where do you find a giant cell tumor of bones?
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Ends of longbones
-Distal femur -Proximal tibia -Distal radius -Proximal humerus |
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What are the sympoms of a giant cell tumor of bone?
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Pain
Swelling Fracture |
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What's the treatment for a giant cell tumor of bone?
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SURGERY
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What is the problem with giant cell tumors of bone?
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They recur!
1/4 to 1/2 will do this. |
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What is the most typical person to get a giant cell tumor of bone?
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Female (1:1.5 M:F) in 3rd - 4th decade
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What occurs in a fibrous dysplasi/
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Benign
Normal medulla is replaced by abnormal fibro-osseous tissue |
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What ages is a fibrous dysplasia most common in?
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Broad range
Peak from 2nd-3rd decade |
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What bones most commonly show fibrous dysplasi?
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Craniofacial, long bones, ribs
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What is the most common form of fibrous dysplasia?
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Monostotic (70%)
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What is the least common type of fibrous dysplasia?
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McCune-Albright syndrome:
-Precocious puberty -Cafe-au-lait pigmentation |
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What mutation is behind fibrous dysplasia?
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Gain of function in GNAS1
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What is the pathology that happens in Ewing's sarcoma?
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Malignant "small round blue cell" sarcoma with SOFT TISSUE INVOLVEMENT
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Where does Ewing sarcoma happen?
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Diaphysis of long bones
-Pelvis -Ribs |
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What kind of a person gets a Ewing sarcoma?
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Kids!
80% are in people under 20 |
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What is the mutation found in Ewing sarcoma?
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t(11;22)(q24;q12) - 85%
A fusion of two genes |
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What's the prognosis for Ewing sarcoma?
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GREAT!
75% 5 year survival |
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What's more common: metastatic or primary bone tumors?
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METS!
They're usually multifocal |
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In adults, what are common metastatic bone tumors?
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Breast
Prostate Lung Kidney Thyroid |
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What are common metastatic bone tumors in kids?
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Neuroblastoma
Ewing sarcoma Wilms tumor Rhabdomyosarcoma Osteosarcoma |
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What are possible signs of mets to bone?
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Pain
Pathological fracture Hypercalcemia Marrow replacement |
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What is the nomenclature of the different soft tissue tumors?
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What is the most common type of soft tissue tumor?
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Lipomas
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Are most soft tissue tumors benign of malignant?
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Most are benign.
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What can a fibromatosis cause?
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Trigger finger!
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What are the different types of fibromatosis?
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Superficial
-Dupuytren (palmar) -Plantar -Penile Deep -Locally aggressive -3 types |
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What mutations are deep fibromatosis associated with?
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FAP
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Where are leiomyomas found?
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All over the place!
-Skin -Deep soft tissue -GI tract -GU tract 3/4 of women have uterin leiomyomas (FIBROIDS!) |
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What should you think of if someone has multiple cutaneous leiomyomas?
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Underlying RCC
-There's a germline syndrome for this. |
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What is a neurofibroma?
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Benign tumor of a peripheral nerve sheath
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What is the most common type of a neurofibroma?
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Cutaneous, solitary (90%)
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If there are multiple, plexiform neurofibromas, what should you thin?
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NF1
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What is a schwannoma?
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Benign tumor of the peripheral nerve sheaths
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What condition should you think of if there is a bilateral schwannoma of CNVIII?
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NF2
NF2 mutation on chromosome 22 |
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What is the most common person to get a soft tissue sarcoma?
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Man around the age of 65
It's the 4th most common malignancy of kids! |
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Where are the most common places to find a soft tissue sarcoma?
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Extremities
Trunk Retroperitoneum |
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Who has liposarcomas?
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Adults, not kids!
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What are the different types of liposarcomas?
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Well-differentiated (resemble lipomas)
Myxoid/round cell Pleomorphic |
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Who gets leiomyosarcomas?
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Adults; F>M
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Where do leiomyosarcomas arise from?
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Skin, deep soft tissue
GI tract GU tract |
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Who's at risk of a leiomyosarcoma from a parenchymal organ? What causes this?
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Immunosuppressed people
EBV |
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What's the most common type of soft tissue sarcoma in kids/adolescents?
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Rhabdomyosarcoma
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What are the different types of rhabdomyosarcoma?
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Embryonal (most common type)
Alveolar (2nd most common) Pleomorphic |
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What are the most common sites for childhood rhabdomyosarcoma?
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Head/neck
Extremities GU |
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What is a unique variant of an embronal rhabdomyosarcoma?
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Botryoid embryonal rhabdomyosarcoma
Arise from beneath the mucosal epithelial surface -Bladder -Biliary -Vagina -Pharynx -Conjunctiva -Ear -Nose |
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Where do alveolar rhabdomyosarcomas form? When?
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At the extremities
Occurs at an older age than the embryonal |
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What types of rhabdomyosarcomas have the worst prognosis? The best?
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Superior: embryonal, botryoid variant
Intermediate: conventional embryonal Poor: alveolar RMS, pleomorphic |
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What makes a RMS prognosis favorable?
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Infants/kids
Orbit, GU <5 cm Botryoid Localized |
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What makes a RMS prognosis unfavorable?
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Adults
Non-orbital/GU >5cm Alveolar, pleomorphic Invasive Incomplete initialresection |