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139 Cards in this Set

  • Front
  • Back
What are the different parts of a tubular bone?
Physis = growth plate
Diaphysis = shaft
Metaphysis = flaredportion
Epiphysis = end of bone, beyond the physis
Physis = growth plate
Diaphysis = shaft
Metaphysis = flaredportion
Epiphysis = end of bone, beyond the physis
What are the different histologic types of bone?
Lamellar
-mature
-perpendicular rows of collagen bundles

Woven
-immature
Lamellar
-mature
-perpendicular rows of collagen bundles

Woven
-immature
When do you see woven bone?
Development and in pathological conditions!
What are the properties of cortical bone? Where is it found?
Dense, compact

Thickest in he shafts (diaphysis) of long bones
Dense, compact

Thickest in he shafts (diaphysis) of long bones
What are the properties of cancellous bone? Whereis it round?
Spongy, trabecular
Compression resistant

At the ends of long bones, also in vertabrae
Spongy, trabecular
Compression resistant

At the ends of long bones, also in vertabrae
What type of bone is more active: cancellous or cortical bone?
Cancellous
What is the vascular supply to the bone?
Penetrating arteries
-Nutrient artery
-Metaphyseal, epiphyseal arteries

Periostealarteries
-Augments the principal supply
Penetrating arteries
-Nutrient artery
-Metaphyseal, epiphyseal arteries

Periostealarteries
-Augments the principal supply
What part of bone is solid? Water?
92% solid

8% water
What are the components of the bone matrix?
Type I collagen
What are the mineral components of bone?
Calcium hydroxyapatite

99% of body calcium, 85% of the phosphorus
What are the cell types inside of bone? What is their function?
Osteoblast: forms osteoid
Osteocytes: Ca/PO4 homeostasis
Osteoclasts: macrophages that resorb bone
What is wolff's law?
It states that the internal structure of bone is altered in response to the magnitude of force placed upon it

More force, stronger bone!
What is the embryonic origin of the skeleton?
Somites (which comes fromthe mesoderm), which divides intot he dermatomes, myotomes, and sclerotomes

Bone forms fromthe sclerotomes
What is the process of bone formation in long bones? Flat bones?
Long bones (except for cranium, part of clavicle): from cartilage precursors through endochondral ossification

Flat bones: membranous ossification
What are the four zones of bone growth?
Reserve
Proliferation
Hypertrophy
Ossification
Reserve
Proliferation
Hypertrophy
Ossification
What is the definition of a dysotoses?
Localized problems in the migration or formation of bone
What are the most common causes of dysostoses?
Defects in nuclear proteins, TFs
What does someone look like who has dysostoses?
Missing, extra bones

Fusion of bones
What is the definition of a dysplasia of bones?
Global skeletal abnormalities secondary to mutations of genes involved in regulation of skeletal development or structure
What gene is mutated in achondroplasia, hypochondroplasia, and thanatatophoric dwarfism?
FGFR3

Fibroglast growth receptor; functions in signal transduction
What gene is mutated in osteogenesis imperfecta?
COL1A1
What does someone look like with Achondroplasia?
Short limbs, normally sized torso
What is the cause of achondroplasia?
Point mutation of FGFR3
What's the inheritance of achondroplasia?
Autosomal dominant
What is thanatophoric dwarfism?
Lethal form of dwarfism

Thorax can't develop-->no respiratory capacity
What mutation occurs in thanatophoric dwarfism?
FGFR3 gain of function mutation
What mutation takes placein osteogenesis imperfecta?
Mutations impacting COL1 synthesis
What are the clinical manifestations of osteogeneis imperfecta?
1. Skeletal fragility

Blue sclera
Small, brown-yellow teeth
Hearing impairment
What is osteopetrosis?
Genetic disease of osteoclasts!
What is the treatment of osteopetrosis?
Bone marrow treatment

CURATIVE
What are the symptoms of osteopetrosis?
Diffuse, symmetric skeletal sclerosis
Brittle bones that can easily fracture
Medullary cavity filed with immature bones

Anemia, infections --> NO MARROW!
What do the bones look like in osteopetrosis?
NO MARRO!

Erlenmeyer flask bones
What are some of the acquired abnormalities of bone structure?
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
What happens to the bones in osteoporosis?
Reduced bone mass; "porpous" bones
What are the risk factors for osteoporosis?
Hereditary factors
Decreased physical activity
Muscle strength
Nutrition, Ca intake
Drugs
Hormones (esp. estrogen!)
What does the histology of osteoporosis look like?
Bottom shows very POROUS bones
Bottom shows very POROUS bones
What happens to the vertebrae during osteoporosis?
Compression fractures!
What happens to the bones in Paget disease of bone?
Net gain in bone mass
What are the phases of paget disease of bone?
1. Osteolytic (osteoclastic)
2. Mixed osteoclastic/osteoblastic
3. Osteosclerotic
What does the histopathology of Paget disease of bone look like?
Mosaic lamellar bone (prominent cement/bluish lines)
HUGE OSTEOCLASTS!
Marrow fibrosis, hypervascularity
Mosaic lamellar bone (prominent cement/bluish lines)
HUGE OSTEOCLASTS!
Marrow fibrosis, hypervascularity
What is the most likely cause of osteomyelitis?
Bacteria

Most likely S. aureus
What kind of bacteria causes osteomyelitis in neonates?
H. influenza
Group B strep
What kind of bacteria causes Osteomyelitis in people with sickle cell disease?
Salmonella
What are some different ways that ostomyelitis can get spread to the bone?
Hematogenous

Direct extension from a contiguous site
-Diabetics with gangrenous digits
-Decubitius ulcers

Traumatic (direct) implantation
What is a complication from osteomyelitis seen in kids?
The periosteum is loosely attached, so the growth can push it out

This then forms new bone, or an INVOLUCRUM
What does the histology of osteomyelitis look like?
Dead, irregular bonewith pus

Dead osteocytes within the bone
Dead, irregular bonewith pus

Dead osteocytes within the bone
What is osteonecrosis?
Avascular necrosis/asepticnecrosis of the bonehead
What are the mechanisms that can causes osteonecrossi/
Trauma
Corticosteroids
Thrombosis/embolism
Vascular inury
Alcoholism
What is a common complication folowing osteonecrosis?
Secondary osteoarthritis
What is the common pathway for fracture healing in bone?
1. Hematoma
2. Inflammatory cell infiltration
3. Activation of osteoprogenitor cells
4. Granualtion tissue
5. Bony/hard callus
6. Remodeling and reconstitution
What are some things that can cause a fracture not to fuse?
Poor immobilization
Infeciton
Ischemia
What are the different kinds of boneformingtumors?
Osteoid osteoma
Osteoblastoma
Osteosarcoma
What are the different types of cartilage forming tumors?
Osteochondroma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Chondrosarcoma
What are some of the miscellaneous tumors of bone?
Giant cell tumor
Aneurysmal bone cyst
Non-ossifying fibroma
Fibrous dysplasia
Ewing sarcoma
What are some of the hematopoietic tumors of bone?
Myeloma
Lymphoma
What kind of a primary bone tumor is the most common?
Hematopoietic (40%)
Cartilage forming (22%)
Bone forming (19%)
What type of a person gets an osteoid osteoma?
Man (2:1 M:F) between 2nd and 3rd decate
Where in the bone do osteoid osteomas occur?
Inside the cortex
What bones do osteoid osteomas usually occur in?
Femur or tibia (50%)
What size are osteoid osteomas?
<2 cm
What is helps with the pain from osteoid osteomas? Why?
NSAIDS!

They produce prostaglandins, which cause pain
What's the treatmnet for osteoid osteomas?
Radioablation is curative!
What kind of a person gets an osteoblastoma?
Man(2.5:1 M:F) in 2nd-3rd decade
Where do osteoblastomas usually occur?
medulla

posterior spine, sacrum (50%)
Jaws
How large are osteoblastomas?
>2 cm (< 2 cm is by definition an osteoid osteoma)
What are the symptoms of osteoblstomas?
Pain that's NOT relieved by NSAIDs
What's the treatment of osteoblastomas?
Curettage/excision
What's the most common primary malignancy of bone?
Osteosarcoma
What are the risk factors for an osteosarcoma?
Radiation
Paget's diseaseof bone
INfarct
What is the most typical person to have an osteosarcoma?
Man (1.5:1 M:F) in second decade
How does someone with an osteosarcoma present?
Pain +/-a palpable mass

Much less commonly, they'll present with a pathologic fracture
What's the treatment for an osteosarcoma?
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
Where do osteosarcoma metsmostoftengo?
The lung
Other bones
Where are osteosarcomas foundin the bone?
90% metaphysis
10% in the diaphysis

Most are in the medulla of the bones
What is an osteochondroma?
It's a growth plate that has found itself in the wrong place and all of a sudden starts growing
What type of a person most commonly gets an osteochondroma?
Man (3:1 M:F) in 2nd -3rd decade
What bones are most commonly involved in an osteochondroma?
Distal femur (30%)
Proximal tibia (15%)
Proximal humerus (15%)
What does someone with an osteochondroma present like?
Palpable mass or asymptomatic
What's the treatment of an osteochondroma?
Excision is curative!
What is an enchondroma?
A piece of the growth plate that has wound up within the medulla of a bone
What kind of a person gets an endhondroma?
Male or female (1:1) in 3rd - 4th decade
What'e the most usual place to find an enchondroma?
Hands/feet (50%)
What's the treatment for enchondroma?
Observation or curettage
What is it called if you have multiple enchondromas? How about multiple enchondromas + hemangiomas?
Multiple = Ollier

Multiple + hemangiomas = Maffucci
What kind of a person gets a chondroblastoma?
Male (2:1 M:F) in 2nd decade

THE SKELETON IS IMMMATURE
Where do chondroblastomas take place?
Long bone epiphyses
-Femur, tibia, humerus
-Talus, calcaneus
What types of cells are inside chondroblastomas?
Giant cells
What types of tumors of bone show giant cells? Which happens in what types of populations?
Chondroblastoma: kids

Giant cell tumor: adults
What's the treatment for chondroblastoma?
Curettage: scooping it out!
Where do chondrosarcomas metastasize to?
Lungs
What are the properties of a giant cell tumor of bone?
Giant cells!

Benign but locally aggressive
Where do you find a giant cell tumor of bones?
Ends of longbones
-Distal femur
-Proximal tibia
-Distal radius
-Proximal humerus
What are the sympoms of a giant cell tumor of bone?
Pain
Swelling
Fracture
What's the treatment for a giant cell tumor of bone?
SURGERY
What is the problem with giant cell tumors of bone?
They recur!

1/4 to 1/2 will do this.
What is the most typical person to get a giant cell tumor of bone?
Female (1:1.5 M:F) in 3rd - 4th decade
What occurs in a fibrous dysplasi/
Benign

Normal medulla is replaced by abnormal fibro-osseous tissue
What ages is a fibrous dysplasia most common in?
Broad range

Peak from 2nd-3rd decade
What bones most commonly show fibrous dysplasi?
Craniofacial, long bones, ribs
What is the most common form of fibrous dysplasia?
Monostotic (70%)
What is the least common type of fibrous dysplasia?
McCune-Albright syndrome:
-Precocious puberty
-Cafe-au-lait pigmentation
What mutation is behind fibrous dysplasia?
Gain of function in GNAS1
What is the pathology that happens in Ewing's sarcoma?
Malignant "small round blue cell" sarcoma with SOFT TISSUE INVOLVEMENT
Where does Ewing sarcoma happen?
Diaphysis of long bones
-Pelvis
-Ribs
What kind of a person gets a Ewing sarcoma?
Kids!

80% are in people under 20
What is the mutation found in Ewing sarcoma?
t(11;22)(q24;q12) - 85%

A fusion of two genes
What's the prognosis for Ewing sarcoma?
GREAT!

75% 5 year survival
What's more common: metastatic or primary bone tumors?
METS!

They're usually multifocal
In adults, what are common metastatic bone tumors?
Breast
Prostate
Lung
Kidney
Thyroid
What are common metastatic bone tumors in kids?
Neuroblastoma
Ewing sarcoma
Wilms tumor
Rhabdomyosarcoma
Osteosarcoma
What are possible signs of mets to bone?
Pain
Pathological fracture
Hypercalcemia
Marrow replacement
What is the nomenclature of the different soft tissue tumors?
What is the most common type of soft tissue tumor?
Lipomas
Are most soft tissue tumors benign of malignant?
Most are benign.
What can a fibromatosis cause?
Trigger finger!
What are the different types of fibromatosis?
Superficial
-Dupuytren (palmar)
-Plantar
-Penile

Deep
-Locally aggressive
-3 types
What mutations are deep fibromatosis associated with?
FAP
Where are leiomyomas found?
All over the place!
-Skin
-Deep soft tissue
-GI tract
-GU tract

3/4 of women have uterin leiomyomas (FIBROIDS!)
What should you think of if someone has multiple cutaneous leiomyomas?
Underlying RCC
-There's a germline syndrome for this.
What is a neurofibroma?
Benign tumor of a peripheral nerve sheath
What is the most common type of a neurofibroma?
Cutaneous, solitary (90%)
If there are multiple, plexiform neurofibromas, what should you thin?
NF1
What is a schwannoma?
Benign tumor of the peripheral nerve sheaths
What condition should you think of if there is a bilateral schwannoma of CNVIII?
NF2

NF2 mutation on chromosome 22
What is the most common person to get a soft tissue sarcoma?
Man around the age of 65

It's the 4th most common malignancy of kids!
Where are the most common places to find a soft tissue sarcoma?
Extremities
Trunk
Retroperitoneum
Who has liposarcomas?
Adults, not kids!
What are the different types of liposarcomas?
Well-differentiated (resemble lipomas)
Myxoid/round cell
Pleomorphic
Who gets leiomyosarcomas?
Adults; F>M
Where do leiomyosarcomas arise from?
Skin, deep soft tissue

GI tract

GU tract
Who's at risk of a leiomyosarcoma from a parenchymal organ? What causes this?
Immunosuppressed people

EBV
What's the most common type of soft tissue sarcoma in kids/adolescents?
Rhabdomyosarcoma
What are the different types of rhabdomyosarcoma?
Embryonal (most common type)
Alveolar (2nd most common)
Pleomorphic
What are the most common sites for childhood rhabdomyosarcoma?
Head/neck
Extremities
GU
What is a unique variant of an embronal rhabdomyosarcoma?
Botryoid embryonal rhabdomyosarcoma

Arise from beneath the mucosal epithelial surface
-Bladder
-Biliary
-Vagina
-Pharynx
-Conjunctiva
-Ear
-Nose
Where do alveolar rhabdomyosarcomas form? When?
At the extremities

Occurs at an older age than the embryonal
What types of rhabdomyosarcomas have the worst prognosis? The best?
Superior: embryonal, botryoid variant

Intermediate: conventional embryonal

Poor: alveolar RMS, pleomorphic
What makes a RMS prognosis favorable?
Infants/kids
Orbit, GU
<5 cm
Botryoid
Localized
What makes a RMS prognosis unfavorable?
Adults
Non-orbital/GU
>5cm
Alveolar, pleomorphic
Invasive
Incomplete initialresection