Bones, Joints, and Soft Tissue Tumors Flashcards Preview

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Flashcards in Bones, Joints, and Soft Tissue Tumors Deck (100):
1

aWhat is the precursor cell of osteoblasts?

Osteoprogenitor cells

2

What is the precursor cell of osteoclasts?

Fusion of  Preosteoclasts 

(that's how they become giant, multinucleate cells)

3

What is the function of osteoprotegerin?

  • Blocks RANK ligand
  • Can't bind RANK receptor on preosteoclast
  • Prevents bone resorption by inhibiting differentiation

 

NOTE:

  • proteger = to protect in spanish
  • Osteo- = bone
  • Osteoprotegerin = bone protector

4

Where is woven bone normally seen?

  • Fetal skeleton
  • Pathologic state in adults
    • healing fractures
    • Infection
    • Tumor

5

What is the major protein present in bone?

Collagen type I

6

Osteocalcin is produced and used as a marker of activity of which cell?

Osteoblasts

7

What genes control skeletal morphogenesis?

Homeobox genes

8

When in development does endochondral ossification begin?

8th week of gestation

9

How does bone enlargement occur?

Appositional growth

  • deposition of new bone on a pre-existing surface

10

What are dysostoses?  How do they occur?

  • Dysosteogenesis = defective bone formation
  • Localized problems in migration of mesenchymal cells and formation of condensations

11

What is synpolydactyly?  What causes this?

  • Def
    • extra digit between 3rd and 4th fingers
    • some syndactyly
  • Cause
    • mutation in homeobox gene
    • defective transcription factor

12

Achondroplasia

  • Mutation
  • Symptoms

  • Mutation
    • in FGF receptor (constant stimulation)
    • inhibits chondrocyte proliferation
    • Premature deposition of horizontal struts of bone seals growth plates and inhibits growth
  • Symptoms
    • shortened proximal extremities
    • Enlarged head
    • Normal trunk length

13

Thanatophoric dwarfism

  • Mutation
  • Symptoms

  • Mutation
    • FGF receptor gene
  • Symptoms
    • Dwarfism
    • Bell-shaped abdomen
    • Small chest cavity
      • leads to respiratory insufficiency and death

14

Osteogenesis Imperfecta

  • Mutation
  • Symptoms

  • aka Brittle bone disease
  • Mutation
    • defects in synthesis of Type I collagen
  • Symptoms (general)
    • too little bone
    • Osteoporosis with
      • cortical thinning
      • attenuation of trabeculae

15

What symptoms are associated with Osteogenesis imperfecta type I?

  • Increased risk of fractures
  • Blue sclerae
  • hearing loss
  • Small, mis-shapen, blue-yellow teeth

16

What symptoms are associated with Osteogenesis imperfecta type II?

  • Multiple fractures
  • Fatal in utero

17

What symptoms are associated with Osteogenesis imperfecta type III?

​Progressive, deforming disease

  • Growth retardation
  • Multiple fractures
  • Hearing loss
  • Tooth irregularities

18

What symptoms are associated with Osteogenesis imperfecta type IV?

  • Postnatal fractures
  • Normal Sclerae
  • Moderate skeletal fragility
  • Short stature

19

Achondrogenesis II

  • Mutation
  • Symptoms

  • Mutation
    • Type II collagen
  • Symptoms
    • Short trunk
    • Short extremities
    • Enlarged cranium
    • Flattened face

20

Schmid Metaphyseal Chondrodysplasia

  • Mutation
  • Symptoms

  • Mutation
    • type 10 collagen defect
  • Symptoms
    • short stature
    • Bowing of lower extremities
    • Coxa Vera (deformity of hip joint)

    • Abnormal metaphysis

21

Patient presents with history of fractures, anemia, increased infections, and hepatosplenomegaly.  Radiography shows bones with symmetric sclerosis and an erlenmeyer flask deformity.  What is the disease?  What is the cause of disease? What causes the symptoms?

  • Disease
    • Osteopetrosis
  • Cause of disease
    • Reduced osteoclast activity so reduced bone resorption
    • Type II Carbonic anhydrase deficiency
      • Failure to excrete H+ to solubilize hydroxyapatite
  • Cause of symptoms
    • Bones lack a medullary canal
      • decreased hematopoiesis
    • Extramedullary hematopoiesis
      • hepatosplenomegaly

22

Osteopetrosis

  • Characteristics
  • Symptoms
  • Cause

  • Characteristics
    • symmetric sclerosis
    • erlenmeyer flask deformity
      • ​ends are bulbous and misshapen
    • lack medullary canal
    • Neural foramina compress exiting nerves
  • Symptoms
    • Fractures
    • anemia
    • increased infections
    • hepatosplenomegaly
  • Cause
    • Reduced osteoclast activity so reduced bone resorption
    • Type II Carbonic anhydrase deficiency
      • Failure to excrete H+ to solubilize hydroxyapatite

23

Post-menopausal woman presents with height loss and history of fractures.  Her bones are found to have a reduced mass and increased porosity.  Radiograph shows a collapsed vertebra.  What is the disease?  What is the cause?  What is the possible complication?

  • Disease
    • Post-menopausal osteoporosis
  • Cause
    • Estrogen deficiency increases secretion of proinflammatory cytokines
    • Increased activity of osteoclasts
  • Complication of fracture
    • Pulmonary emboli

24

What is the difference in bone structure in post - menopausal and senile osteoporosis?

  • Post-menopausal
    • Trabeculae are thinned
    • Mainly affects bone that has increased surface area
      • vertebral bodies
  • Senile
    • Cortex is thinned
    • Haversian systems are widened

25

What is the difference in cause of post - menopausal and senile osteoporosis?

  • Post-menopausal
    • Estrogen deficiency
      • increased inflammatory cytokines
      • (+) osteocyte activity
  • Senile
    • decreased replication and activity of osteoblasts
    • reduced physical activity

26

Patient presents with localized bone pain, joint pain in the knees, and history of fractures.  Labs show elevated serum alkaline phosphatase and increased urine hydroxyproline.  Histology shows a mosaic pattern of bone with increased bone mass.  What is the disease?  What is the cause? 

  • Disease
    • Paget disease
  • Cause
    • high concentration of IL-6 and M-CSF
      • induces osteoclast activity

27

Paget disease

  • Cause 
  • Signs
  • Symptoms

  • Cause
    • High concentrations of IL-6 and M-CSF
      • induces osteoclast activity
  • Signs
    • Increased bone mass
      • mosaic pattern
    • Elevated serum alkaline phosphatase
    • Increased urine hydroxyproline
  • Symptoms
    • Pain localized to affected bone
      • microfractures
      • compression of nerves
    • Osteoarthritis in weight-bearing joints

28

What are the stages of Paget disease?  What is the net effect?

  1. Osteolytic
    • active bone resorption
  2. Osteoclastic-osteoblastic
    • ends in bone formation
  3. Quiescent osteosclerotic phase

Net effect: increased bone mass that is disordered and unsound

29

What is the cause of Primary Hyperparathyroidism?

Adenoma

30

What is the cause of Secondary Hyperparathyroidism?

Prolonged hypocalcemia

(results in increased PTH secretion)

31

Osteitis Fibrosa Cystica

  • Cause
  • Signs 
  • Symptoms

  • Cause
    • Hyperparathyroidism
    • uncontrolled bone resorption
  • Signs 
    • Osteopenia (decreased density)
    • Replacement of marrow spaces with fibrovascular tissue
      • Microfractures
      • Hemorrhage
      • Cell influx
    • Brown tumor
      • result of hemorrhage
  • Symptoms
    • Pain

32

What is the pathogenesis of Renal osteodystrophy?

  • Chronic renal failure
    • Phosphate retention
      • Hyperphosphatemia
      • Secondary hyperparathyroidism
  • Causes bone resorption 

33

What is the difference between an closed and a compound fracture?

  • Closed
    • overlying tissue intact
  • Compound
    • break through skin

34

What is a comminuted fracture?

Bone is splintered

35

What is a displaced fracture?

Ends of bone are not aligned

36

What is the mechanism of fracture repair?

  • Fibrin clot seals fracture site
    • creates scaffolding for cells and angiogenesis
  • Organization of hematoma
  • Soft tissue callus or Procallus formed
    • uncalcified tissue
  • Chondroblasts make cartilage along fracture line
    • undergoes endochondral ossification
  • Bony callus formation
  • Mineralization and resorption of callus sections not physically stressed

37

What is the most common cause of osteonecrosis?

Corticosteroid use

38

What is the difference between medullary and subchondral infarcts in osteonecrosis?

  • Medullary
    • Involve cancellous bone and marrow
    • Subclinical
  • Subchondral
    • Wedge-shaped
    • Results in chronic pain

39

How is osteonecrosis repaired?

Creeping substitution

  • Not effective
  • Results in collapse of necrotic bone

40

What is the most common cause of osteomyelitis?

Staph. aureus

41

Osteomyelitis

  • Cause
  • Pathology
  • Signs and symptoms

  • Cause
    • S. aureus infection (most common)
    • M. tuberculosis
    • T. pallidum (syphilis)
  • Path
    • sequestrum = dead bone
    • Rupture of periosteum 
      • abscess in soft tissue
      • eventually forms draining sinus
    • New bone depositied as a sleeve around sequestrum
      • Involucrum
  • Signs and Symptoms
    • Pain 
    • Fever
    • X ray: lytic lesion surrounded by zone of sclerosis

42

Pott disease

  • Cause
  • Symptoms
  • Most common site of involvement

  • Cause
    • M. tuberculosis
  • Symptoms
    • Osteomyelitis
  • Most common site of involvement
    • Spine

43

What is an osteoma?

  • Benign
  • Bone-forming
  • Slow growing
  • Project from subperiosteal or endosteal surfaces of cortex

44

What are the differences between an Osteoid Osteoma and an Osteoblastoma?

  • Osteoid Osteoma
    • Size:
      • less than 2 cm
    • Origin:
      • cortex of femur or tibia
    • Symptoms:
      • Painful due to secretion of PGE2
      • Relieved by aspirin
  • Osteoblastoma
    • Size
      • larger
      • large amounts of reactive bone encircle the lesion
    • Origin
      • Spine
    • Symptoms
      • Dull pain
      • Does not respond to aspirin

45

Patient presentswith a painful, progressively enlargening mass near the knee.  Radiographs show extra bone formation with a lace-like architecture that forms the Codman triangle (shown below).  What is the disease?  What is commonly found in the soft tissue surrounding affected bone?

  • Disease
    • Osteosarcoma
    • Malignant
  • Soft tissue
    • forms masses

46

What is the most common subtype of osteosarcoma?

  • Arises in metaphysis of long bones
  • Primary (not associated with underlying path)
  • Solitary
  • Intramedullary
  • Poorly differentiated
  • Produces predominantly bony matrix

47

What is the most common site of metastasis in osteosarcoma?

Lungs

48

What is an osteochondroma?

  • Benign
  • Slow growing
  • Sometimes painful
  • Cartilage-capped outgrowth attached by bony stalk
  • Develop in bones of endochondral origin

49

What is the most common form of Chondroma?  Where does it form?

  • Most common:
    • Enchondroma
  • Location
    • Medullary cavity of bone of hands and feet
  • Other form:
    • Subperiosteal
      • on surface of bone

50

What is Ollier disease?

  • Multiple enchondromas
    • (in medullary cavity of hands and feet)

51

What is Maffucci syndrome?

  • Enchondroma associated with soft tissue hemangiomas

52

What is a chondroblastoma?

  • Benign
  • Structure
    • Well demarkated
    • Sheets of compact chondroblasts
    • Osteoclast-like giant cells
    • Small amounts of hyaline matrix
  • Location
    • epiphyses
    • especially near the knee

53

Chrondromyxoid fibroma

  • benign
  • painful
  • Structure
    • poorly formed hyaline cartilage and myxoid tissue

54

Chondrosarcoma

  • Cell characteristics
  • Location
  • Symptoms

  • Malignant
    • metastasis to lungs and bone
  • Infiltrates marrow and soft tissue
  • Cell
    • Matrix-producing
    • Pleomorphic
    • Large nuclei
  • Locations
    • Intramedullary = central
    • Juxtacortical = peripheral
    • Central portions of skeleton
      • pelvis
      • shoulders
      • ribs
  • Symptoms
    • painful
    • Progressively enlargening mass

55

Conventional Chondrosarcoma morphology

  • Nodules of hyaline and mixoid cartilage

56

Dedifferentiated chondrosarcoma morphology

  • Looks like poorly differentiated sarcoma

57

Clear cell chondrosarcoma

  • Morphology
  • Location

  • Morphology
    • Sheets of large chondrocytes
    • Abundant cytoplasm
    • Numerous giant cells
  • Location
    • Epiphyses of long bones

58

Mesenchymal chondrosarcoma morphology

  • Islands of well-differentiated hyaline cartilage surrounded by sheets of round cells

59

Fibrous cortical defects

  • Appearance
  • Cause
  • Cells
  • Location

  • Appearance
    • elongated, sharply demarcated radiolucencies surrounded by a zone of sclerosis
  • Cause
    • Developmental defect
    • NOT neoplasm
  • Cells
    • fibroblasts
      • storiform (pinwheel) pattern
    • Histiocytes
      • multinucleated giant cells
      • foamy macrophages
  • Location
    • Metaphysis of femur and tibia

60

What are the complications of polyostotic fibrous dysplasia?

  • Significant deformities and fractures
    • craniofacial involvement

61

McCune-Albright Syndrome

  • Signs
  • Pathology

  • Signs
    • Bone lesions
    • Cutaneous macules
      • Cafe-au-lait
      • "coast of Maine" borders
      • Respect the midline
      • Follow developmental lines
    • Precocious sexual development
      • Endocrinopathis
  • Path
    • intramedullary
    • Curvilinear trabeculae
      • mimic Chinese characters
    • Hemorrhage and foamy macrophages common

62

Fibrosarcoma

  • Histology
  • Signs / Symptoms

  • Histology
    • Fibroblasts arranged in herringbone pattern
  • Signs / Symptoms
    • Painful enlargening masses
    • Pathologic fractures
    • Destroy bone
    • Invade soft tissue

63

Malignant Fibrous Histiocytoma

  • Histology
  • Signs / Symptoms

  • Histology
    • Spindled fibroblasts
    • Large multinucleated giant cells
  • Signs / Symptoms
    • Painful enlargening masses
    • Pathologic fractures
    • Destroy bone
    • Invade soft tissue

64

Ewing Sarcoma and Primitive Neuroectodermal Tumor (PNET)

  • Presentation
  • Histology
  • Location

  • Presentation
    • painful enlargening mass
    • Warm and swollen
  • Histology
    • Sheets of "small round blue cells"
    • large nuclei
    • little cytoplasm
    • Rich in glycogen
    • Homer Wright rosettes
      • indicative of neural differentiation
  • Location
    • Diaphyses of long tumular bones

**Second most common bone tumor in children, after osteosarcoma**

65

Giant Cell Tumor

  • Histology
  • Pathology
  • Location
  • Metastasis location

aka Osteoclastoma

  • Histology
    • Multinucleated osteoclast-like giant cells
    • Uniform mononuclear cells
  • Path
    • Necrosis
    • Hemorrhage
    • Hemosiderin deposition
    • Reactive bone formation
  • Location:
    • epiphyses and metaphyses around knee
  • Metastasis
    • lung

66

Where are the most common locations that cause metastasis to bone?

  1. Prostate
  2. Breast
  3. Lung 
  4. Kidney

67

What are the most likely bones to develop metastases?

  • Axial skeleton
    • skull
    • sternum
    • ribs
    • vertebrae
    • pelvis
  • Proximal femur
  • Proximal humerus

68

Osteoarthritis

  • Signs / Symptoms
  • Path

  • Signs / Symptoms
    • joint has appearance of polished ivory
    • Radiograph: loss of joint space
    • Deep pain that worsens with use
    • Morning stiffness
    • Oneor a few joints are involved
  • Path
    • weakened collagen network
    • Increased proinflammatory cytokines
    • chondrocyte apoptosis

69

Juvenile Idiopathic Arthritis (JIA)

  • Onset
  • Joints affected
  • Symptoms
  • Path
  • Complications

  • Onset
    • Infection
    • DRB1 alleles
  • Joints affected
    • large joints
  • Symptoms
    • arthritis
    • fever and rash (systemic - onset)
  • Path
    • Like RA
      • TNF mediated
  • Complications
    • impaired bone and joint growth

70

Characteristics of the following forms of JIA

  • Pauciarticular
  • Polyarticular
  • Systemic

  • Pauciarticular
    • <5 joints involved
    • peak age: 2-3
  • Polyarticular
    • >5 joints involved
    • Peak age: 2-5 and 10-14
  • Systemic
    • any number of joints involved
    • fever
    • rash
    • Peak age: under 17

71

Ankylosing Spondylitis

  • Path
  • Location
  • Symptoms

  • Path
    • Chronic Imflammation
    • Proliferative synovitis similar to RA
      • w/o rheumatoid factor
      • Ossification of sacroiliac joints and spine
        • advanced disease
  • Location
    • Sacroiliac
    • Spine 
    • Large peripheral joints
  • Symptoms
    • Low back pain and stiffnes
    • Relieved by exercise

72

Reactive Arthritis

  • Cause
  • Path
  • Symptoms

  • Cause
    • Noninfectious
    • Occurs w/i 1 mo. of primary infection located elsewhere in the body
    • GI or GU infections
  • Path
    • Autoimmune
  • Symptoms
    • asymmetric synovitis of ankles, knees, feet

73

Psoriatic Arthritis

  • Path
  • Symptoms

  • Path
    • Immunologic
      • Th1 and Th17
    • Proinflammatory cytokines similar to RA
      • TNF, IL-1, 6, and 8
  • Symptoms
    • asymmetric

74

Infectious (Septic) Arthritis

  • Causes
  • Signs / Symptoms

  • Monoarthritis
  • Causes
    • Trauma
    • Infection:
      • Bacteria: S. aureus
      • Virus: HBV
      • Fungi
  • Signs / Symptoms
    • Acute onset of pain
    • Swelling
    • Fever

75

What is the pathology of Gout?

  • Path
    • Hyperuricemia
      • Enzyme deficiency in purine metabolism
      • Overconsumption of purine - rich foods
    • Causes crystal formation in synovial tissue and cartilage

76

Acute Gouty Arthritis

  • Characteristics
  • Signs / Symptoms

  • Characteristics
    • neutrophil infiltrate
  • Signs / Symptoms
    • joint is painful, swollen, and red

77

Chronic Tophaceous Arthritis

  • Histology

  • Histology
    • Synovium becomes fibrotic
    • Inflammatory cell infiltrate
    • Tophi form
      • large aggregates of urate crystals surrounded by inflammatory cells
      • Pathognomonic hallmark of gout

78

Pseudogout

  • Path
    • Calcium pyrophosphate crystal deposition
    • Develop first in articular tissue then released into synovial fluid

79

Ganglion

  • Characteristics
  • Location

  • Reactive tumor-like lesion
  • Characteristics
    • small cyst near joint capsule or tendon sheath
    • aspiration = clear gelatinous fluid
  • Location
    • common in the wrist

80

Synovial cyst

  • Characteristics

  • Benign
  • Reactive tumor-like lesions
  • Herniation of synovium thru joint capsule

81

Tenosynovial Giant Cell Tumor

  • Characteristics
  • Location
  • Types
    • PVNS
    • GCT

  • Characteristics
    • Benign
  • Location
    • Synovial lining of joints, tendon sheaths, bursae
  • Types
    • Diffuse type / Pigmented Villonodular Synovitis (PVNS)
      • involves synovium
      • Presents with arthritis (usually in knee)
    • Localized type / Giant Cell Tumor (GCT)
      • Involved tendon sheath
      • Painless mass 
      • Common along wrists and fingers

82

Conventional Lipoma

  • Characteristics

  • Mature adipose tissue
  • Most common

83

Spindle Cell Lipoma

  • Characteristics

  • Spindle cells and hyaline cartilage

84

Angiolipoma

  • Characteristics

  • Mature adipose tissue
  • Vessels containing fibrin thrombi

85

Myxoid liposarcoma

  • Characteristics

  • Abundant ground substance
  • Mature fat cells and primitive cells

86

Well-differentiated Liposarcoma

  • Characteristics

  • Fat cells of various sizes
  • Prominent fibrous septa

87

Nodular Fasciitis

  • Characteristics
  • Location
  • Signs / Symptoms

  • Reactive Pseudosarcomatous Proliferation
  • Characteristics
    • Benign
    • Immature fibroblasts with myxoid stroma
  • Location
    • Deep dermis
    • Subcutaneous tissue
    • Muscle
  • Signs / Symptoms
    • Painful, rapidly enlargening mass

88

Myositis Ossificans

  • Characteristics
  • Target population
  • Signs / Symptoms

  • Characteristics
    • Benign
    • Fibroblasts
    • Metaplastic bone
    • Lesion ossifies
  • Target population
    • Young athletes (following trauma)
  • Signs / Symptoms
    • Swollen, painful area

89

Superficial Fibromatosis

  • Characteristics 
  • Locations

  • Characteristics
    • Proliferating fibroblasts
    • Surrounded by dense collagen
  • Locations: 3 P's
    • Palmar
    • Plantar
    • Penile

90

Deep Seated Fibromatosis

  • Characteristics

  • Characteristics
    • Well differentiated fibroblasts
    • Arranged in fascicles
    • Infiltrate adjacent tissue

91

Fibrosarcoma

  • Histology
  • Characteristics

  • Histology
    • Spindle cells in herringbone pattern
  • Characteristics
    • Aggressive
    • areas of necrosis and hemorrhage

92

Benign Fibrous Histiocytoma

  • Histology
  • Signs / Symptoms
  • Location

  • Histology
    • CD34+ spindle cells
    • Storiform (cartwheel or swirling) pattern
  • Signs / Symptoms
    • Painless
    • Slow growing
  • Location
    • Dermis or subcutaneous tissue

93

Malignant Fibrous Histiocytoma

  • Histology
  • Location

  • Histology
    •  Fibroblast cells
      • Malignant
      • Pleomorphic
    • Storiform pattern
  • Location
    • Musclulature of proximal extremities
    • Retroperitoneum

94

Rhabdomyosarcoma

  • Histology
  • Location

  • Histology
    • Rhabdomyoblast
      • Eosinophilic cytoplasm
      • Round or elongated (tadpole or strap cells)
    • Positive for muscle markers
      • Desmin
      • Myogenin
  • Location
    • Head 
    • Neck
    • GU tract

95

Embryonal Rhabdomyosarcoma

  • Histology
  • Age of Onset

  • Most common form
  • Histology
    • Sheets of malignant round and spindle cells
    • Myxoid stroma
  • Age of onset
    • children under 10

96

Alveolar Rhabdomyosarcoma

  • Histo
  • Location
  • Age of onset

  • Histo
    • Fibrous septae divide cells into clusters
    • Resemble alveoli
  • Location
    • Deep musculature of extremities
  • Age of onset
    • early to mid adolescence

97

Pleomorphic (Anaplastic) Rhabdomyosarcoma

  • Histology

  • Histology
    • Large, multinucleated eosinophilic cells

98

Leiomyoma

  • Histology
  • Location
  • Signs / Symptoms

  • Aka: Fibroids
  • Histology
    • Spindle cells
    • Elongated nuclei
  • Location
    • Uterus
  • Signs / Symptoms 
    • Heavy, prolonged menses
    • Subfertility
    • Adverse pregnancy outcomes

99

Leiomyosarcoma

  • Histology
  • Location

  • Histo
    • Spindle cells
    • Elongated nuclei
    • Arranged in interweaving fascicles
  • Location
    • Skin and deep soft tissue of extremities and retroperitoneum

100

Synovial Sarcoma

  • Histology
  • Location

  • Histology
    • Biphasic nature of cell (Hallmark)
      • Epithelial-like
        • forms gland-like structures
      • Spindle cells
        • form fascicles
  • Location
    • Deep soft tissue around large joints of extremities
      • Knees
      • Thighs