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Pathology > Bones, Joints, and Soft Tissue Tumors > Flashcards

Bones, Joints, and Soft Tissue Tumors Flashcards

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1
Q

aWhat is the precursor cell of osteoblasts?

A

Osteoprogenitor cells

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2
Q

What is the precursor cell of osteoclasts?

A

Fusion of Preosteoclasts

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

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3
Q

What is the function of osteoprotegerin?

A
  • 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
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4
Q

Where is woven bone normally seen?

A
  • Fetal skeleton
  • Pathologic state in adults
    • healing fractures
    • Infection
    • Tumor
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5
Q

What is the major protein present in bone?

A

Collagen type I

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6
Q

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

A

Osteoblasts

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7
Q

What genes control skeletal morphogenesis?

A

Homeobox genes

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8
Q

When in development does endochondral ossification begin?

A

8th week of gestation

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9
Q

How does bone enlargement occur?

A

Appositional growth

  • deposition of new bone on a pre-existing surface
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10
Q

What are dysostoses? How do they occur?

A
  • Dysosteogenesis = defective bone formation
  • Localized problems in migration of mesenchymal cells and formation of condensations
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11
Q

What is synpolydactyly? What causes this?

A
  • Def
    • extra digit between 3rd and 4th fingers
    • some syndactyly
  • Cause
    • mutation in homeobox gene
    • defective transcription factor
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12
Q

Achondroplasia

  • Mutation
  • Symptoms
A
  • 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
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13
Q

Thanatophoric dwarfism

  • Mutation
  • Symptoms
A
  • Mutation
    • FGF receptor gene
  • Symptoms
    • Dwarfism
    • Bell-shaped abdomen
    • Small chest cavity
      • leads to respiratory insufficiency and death
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14
Q

Osteogenesis Imperfecta

  • Mutation
  • Symptoms
A
  • aka Brittle bone disease
  • Mutation
    • defects in synthesis of Type I collagen
  • Symptoms (general)
    • too little bone
    • Osteoporosis with
      • cortical thinning
      • attenuation of trabeculae
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15
Q

What symptoms are associated with Osteogenesis imperfecta type I?

A
  • Increased risk of fractures
  • Blue sclerae
  • hearing loss
  • Small, mis-shapen, blue-yellow teeth
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16
Q

What symptoms are associated with Osteogenesis imperfecta type II?

A
  • Multiple fractures
  • Fatal in utero
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17
Q

What symptoms are associated with Osteogenesis imperfecta type III?

A

​Progressive, deforming disease

  • Growth retardation
  • Multiple fractures
  • Hearing loss
  • Tooth irregularities
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18
Q

What symptoms are associated with Osteogenesis imperfecta type IV?

A
  • Postnatal fractures
  • Normal Sclerae
  • Moderate skeletal fragility
  • Short stature
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19
Q

Achondrogenesis II

  • Mutation
  • Symptoms
A
  • Mutation
    • Type II collagen
  • Symptoms
    • Short trunk
    • Short extremities
    • Enlarged cranium
    • Flattened face
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20
Q

Schmid Metaphyseal Chondrodysplasia

  • Mutation
  • Symptoms
A
  • Mutation
    • type 10 collagen defect
  • Symptoms
    • short stature
    • Bowing of lower extremities
    • **Coxa Vera **(deformity of hip joint)
    • Abnormal metaphysis
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21
Q

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?

A
  • 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
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22
Q

Osteopetrosis

  • Characteristics
  • Symptoms
  • Cause
A
  • 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
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23
Q

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?

A
  • Disease
    • Post-menopausal osteoporosis
  • Cause
    • Estrogen deficiency increases secretion of proinflammatory cytokines
    • Increased activity of osteoclasts
  • Complication of fracture
    • Pulmonary emboli
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24
Q

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

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

Pathology (27 decks)

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