SM_230a: Neoplasia Flashcards

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1
Q

Describe classification of major bone tumors

A

Classification of bone tumors

  • Bone marrow: malignant (myeloma, malignant lymphoma)
  • Bone: benign (osteoma, osteoid osteoma, osteoblastoma), malignant (osteosarcoma)
  • Cartilage: benign (osteochondroma, chondroma, chondroblastoma, chondromyxoid fibroma), malignant (chondrosarcoma)
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2
Q

Describe histology of each bone component

A

Histology of each bone component

  • Bone: pink dense connective tissue composed of osteocytes
  • Bone marrow: adipocytes and adipose
  • Cartilage: connective tissue w/ each chondrocyte in a lacuna
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3
Q

Describe bone tumors in general

A

Bone tumors

  • Most commonly occur due to metastasis from other sites: usually osteolytic (punched-out lesion), prostate carcinoma is osteoblastic
  • Most common primary bone tumors are multiple myeloma and osteosarcoma
  • Primary bone tumors often more common in males (some exceptions like giant cell tumor)
  • Histology comes last because bone can exhibit limited changes
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4
Q

Benign bone tumors are ____, ____, ____, ____, ____, ____, and ____

A

Benign bone tumors are osteoma, osteoid osteoma, osteoblastoma, osteochondroma, chondroma, giant cell tumor of bone, and fibrous dysplasia

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5
Q

Describe osteoma

A

Osteoma

  • Benign solitary
  • Middle age
  • Most commonly arise on surface of facial bones
  • Slow-growing, little significance unless blocks the sinus cavity
  • Multiple osteomas associated with Gardner’s syndrome
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6
Q

Describe osteoid osteoma

A

Osteoid osteoma

  • Small benign tumor of osteoblasts (< 2 cm)
  • Young adults < 25, more common in males
  • Nocturnal bone pain relieved by aspirin
  • Cortex of long bones (femur)
  • X-ray: zone of sclerotic lamellar bone surrounding a lucent focus (nidus): woven bone in vascular stroma
  • Surgery or radiofrequency ablation
  • Prognosis excellent (must remove nidus)
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7
Q

Osteoid osteoma is a _____ tumor of _____

A

Osteoid osteoma is a small benign tumor of osteoblasts (< 2cm)

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8
Q

Osteoid osteoma presents as _____ pain relieved by _____

A

Osteoid osteoma presents as nocturnal bone pain relieved by aspirin

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9
Q

Osteoid osteoma manifests as ____ on X-ray

A

Osteoid osteoma manifests as zone of sclerotic lamellar bone surrounding a lucent focus (nidus) on X-ray

(woven bone in lamellar stroma)

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10
Q

Osteoid osteoma is treated with ____ or ____

A

Osteoid osteoma is treated with surgeery or radiofrequency ablation

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11
Q

Describe the nidus in osteoid osteoma

A

Nidus in osteoid osteoma

  • Radiolucent central focus of woven bone with a vascular stroma, surrounding cortical bone is thickened
  • Anastomosing woven bone + osteoblastic rimming + vascular stroma
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12
Q

Contrast osteoblastoma and osteoid osteoma

A

Osteoblastoma and osteoid osteoma

  • Tumors of osteoblasts
  • Osteoid osteoma: < 2cm, long bone (femur), responds to NSAIDs
  • Osteoblastoma: > 2 cm, vertebrae, does not respond to NSAIDs
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13
Q

Describe osteochondroma

A

Osteochondroma

  • Most common benign bone tumor
  • Male < 25, after fracture or radiation
  • Bone + cartilaginous cap arise from metaphysis (growth plate)
  • Distal femur, proximal tibia
  • Prognosis: 1-2% recur, rarely can transform to chondrosarcoma
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14
Q

____ is the most common benign bone tumor

A

Osteochondroma is the most common benign bone tumor

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15
Q

Osteochondroma looks like an ___ on X-ray

A

Osteochondroma looks like an ice cream cone on X-ray

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16
Q

Osteochondroma has a _____ cap on a bony stalk continuous with the _____ of the bone

A

Osteochondroma has a cartilaginous cap on a bony stalk continuous with the medulla of the bone

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17
Q

Describe chondroma

A

Chondroma

  • Benign intramedullary cartilaginous neoplasm
  • 2nd-4th decades, males and females equally likely
  • Medulla of small bones of hands and feet
  • X-ray: well-circumscibed, lucent with cartilage matrix calcification (“arc and ring” due to enchondral bone formation at periphery of tumor nodule)
  • Observation with serial radiographs, curettage (5% recurrence rate)
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18
Q

Chondroma occurs in the medulla of ____

A

Chondroma occurs in the medulla of small bones of hands and feet

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19
Q

Chondroma manifests as ______ on X-ray

A

Chondroma manifests as well-circumscribed lucent with cartilage matrix calcification (“arc and ring” due to enchondral bone formation at periphery of tumor nodule)

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20
Q

Chondroma is managed by ____ and ____

A

Chondroma is managed by observation with serial radiographs and curettage

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21
Q

Describe giant cell tumor of the bone

A

Giant cell tumor of the bone

  • 20-40 years (skeletally mature), more common in females
  • Epiphysis of long bone: most common in distal femur/proximal tibia (knee region)
  • Radiograph: soap-bubble lytic lesion on epiphysis
  • En bloc excision preferred
  • Locally aggressive benign tumor
  • May recur
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22
Q

Giant cell tumor of bone occurs more commonly in _____ than _____

A

Giant cell tumor of bone occurs more commonly in females than males

23
Q

Giant cell tumor of the bone occurs most often in the ____

A

Giant cell tumor of the bone occurs most often in the knee region

(epiphysis of long bone: most common in distal femur/proximal tibia)

24
Q

Giant cell tumor of the boan manifests as ____ on X-ray

A

Giant cell tumor of the boan manifests as soap-bubble lytic lesion in epiphysis

25
Q

Giant cell tumor of the bone is treated with ______

A

Giant cell tumor of the bone is treated with en bloc excision

(locally aggressive benign tumor, may recur)

26
Q

Describe fibrous dysplasia

A

Fibrous dysplasia

  • Benign, linked to localized developmental arrest
  • All normal component of bone present but they do not differentiate into mature structures
  • Mostly in first three decades of life
  • 3 patterns
    • Single bone / monostotic (70%): femur most common
    • Multiple bone (polyostotic): 27% w/o endocrine dysfunction, 3% w/ cafe-au-lait spots + endocrinopathy (McCune-Albright syndrome)
27
Q

Fibrous dysplasia is linked to ____

A

Fibrous dysplasia is linked to developmental arrest

(all normal components present but do not differentiate into mature structures)

28
Q

Describe the 3 patterns of fibrous dysplasia

A

Fibrous dysplasia patterns

  • Signle bone (monostotic): 70%, femur most common
  • Multiple bone (polyostotic): 27% w/o endocrine dysfunction, 3% w/ cafe-au-lait spots + endocrinopathy (McCune-Albright syndrome)
29
Q

Summarize benign bone lesions

A

Benign bone lesions

  • Osteoma: benign solitary, surface of facial bone, slow-growing, little clinical significance
  • Osteoid osteoma: < 2cm long, long bone (femur), responds to NSAIDs
  • Osteoblastoma: > 2 cm, vertebrae, does not respond to NSAIDs
  • Osteochondroma: most common benign lesion, male < 25, bone + cartilage cap
  • Chondroma: M=F, medulla of small bones of hands and feet
  • Giant cell tumor of bone: F>M, epiphysis of long bones, soap-bubble
  • Fibrous dysplasia: benign, linked to localized developmental arrest
30
Q

Malignant bone tumors are ____, ____, and ____

A

Malignant bone tumors are osteosarcoma, chondrosarcoma, and Ewing sarcoma

31
Q

Describe osteosarcoma

A

Osteosarcoma

  • Malignant proliferation of osteoblasts
  • Bimodal age: peak at 10-20 years and > 65 years
  • Males: females is 3:2
  • RF: familial retinoblastoma, Li-Fraumeni syndrome (germ line p53 mutation), Paget disease of the bone, bone infarct, radiation
  • Metaphysis of long bones: usually distal femur / proximal tibia (knee) and proximal humerus
  • Prognosis depends on preoperative chemotherapy
32
Q

Osteosarcoma most commonly occurs in the ____

A

Osteosarcoma most commonly occurs in the distal femur / proximal tibia (knee)

(occurs in metaphysis of long bones so can also occur in proximal humerus)

33
Q

Osteosarcoma manifests as _____ and _____ on X-ray

A

Osteosarcoma manifests as Codman triangle and sunburst reaction on X-ray

  • Codman triangle: new subperiosteal bone that is created raises the periosteum away from bone
  • Sunburst reaction due to periositis
34
Q

Describe management of osteosarcoma

A

Osteosarcoma management

  • >90% necrosis: continue chemo, local therapy if margins positive
  • <90% necrosis: change chemo, local therapy if margins positive
35
Q

Describe chondrosarcoma

A

Chondrosarcoma

  • Peak in 5th-7th decades, M:F is 2:1
  • Central portion of skeleton (pelvis, shoulder, rib)
  • Most commonly central (within medullary cavity)
  • Prognosis depends on grade and surgical adequacy
  • Metastases are rare and occur late

(multiple nuclei in lacunae if low grade, very cellular w/ little matrix if high grade)

36
Q

Chondrosarcoma occurs in the ______ portion of the skeleton

A

Chondrosarcoma occurs in the central portion of the skeleton (pelvis, shoulder, rib)

37
Q

Chondrosarcoma manifests as _____, _____, and _____ on X-ray

A

Chondrosarcoma manifests as a lobulated lytic lesions with cartilage matrix calcification, cortical destruction, and no/minimal periosteal reaction

38
Q

Describe Ewing sarcoma

A

Ewing sarcoma (primitive neuroectodermal tumor)

  • Malignant neuroectodermal tumor of bone
  • Characterized by t(11;22) creating fusion protein EWS-FLI1
  • Most at < 20 years, M>F 2:1 (boys < 15)
  • Diaphysis of long bones: most common in femur, then pelvis and ribs
  • Treated with chemo (like osteosarcoma)
    Prognosis: better if non-metastatic than if metastatic
39
Q

Ewing sarcoma most often occurs in ____ that are ____ years old

A

Ewing sarcoma most often occurs in boys that are < 15 years old

40
Q

Ewing sarcoma is characterized by ____, creating fusion protein ____

A

Ewing sarcoma is characterized by t(11;22), creating fusion protein EWS-FLI1

41
Q

Ewing sarcoma most commonly occurs in _____

A

Ewing sarcoma most commonly occurs in femur

(diaphysis of long bones so also occurs in pelvis and ribs)

42
Q

Ewing sarcoma manifests as _____, _____, _____, and _____ on X-ray

A

Ewing sarcoma manifests as poorly defined lytic lesion, cortical destruction, “onion skin”, and some soft tissue extension on X-ray

43
Q

“Onion skin” on X-ray is indicative of the presence of _____

A

“Onion skin” on X-ray is indicative of the presence of Ewing sarcoma

44
Q

Summarize malignant bone lesions

A

Malignant bone lesions

  • Osteosarcoma: bimodal age (10-20 and > 65 years), prognosis depends on response to preoperative chemo
  • Chondrosarcoma: peak in 5-7th decades, central portion of skeleton (pelvis, shoulder, rib), prognosis depends on grade and surgical adequacy
  • Ewing sarcoma: malignant neuroectodermal tumor of bone, characterized by t(11;22) and fusion protein (EWS-FLI1), boys < 15, treated with chemo
45
Q

Hematopoietic bone tumors are ____

A

Hematopoietic bone tumors are multiple myeloma

46
Q

Describe multiple myeloma

A

Multiple myeloma

  • Clonal proliferation of plasma cells (mostly IgG): kappa or lambda
  • Most common primary malignant tumor of bone
  • M>F, >40 years w/ peak in 65-70 years
  • CRAB: C (hypercalcemia), R (renal insufficiency), A (anemia), B (lytic bone lesions)
  • X-ray: multiple punched-out lytic lesions
  • Location: vertebra, ribs, skull, pelvic bones, femur
  • Treatment: chemo, individual lesion radiotherapy
  • Prognosis is poor
47
Q

Multiple myeloma is more common in ____ than ____

A

Multiple myeloma is more common in males than females

48
Q

CRAB in multiple myeloma stands for ____, ____, ____, and ____

A

CRAB in multiple myeloma stands for hypercalcemia, renal insufficiency, anemia, and lytic bone lesions

49
Q

Multiple myeloma occurs in the ____, ____, ____, ____, and ____

A

Multiple myeloma occurs in the vertebra, ribs, skull, pelvic bones, and femur

50
Q
A
51
Q

Multiple myeloma manifests as ____ on X-ray

A

Multiple myeloma manifests as multiple punched-out lytic lesions on X-ray

52
Q

Prognosis for multiple myeloma is _____

A

Prognosis for multiple myeloma is poor

53
Q

Summarize the locations of bone tumors

A

Locations of bone tumors