Bone Tumors Flashcards

1
Q

Key radiologic features

A

Bones involved, location, lytic vs sclerotic, tumor margin/zone of transition, matrix formation, periosteal reaction, soft tissue involvement

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2
Q

Cartilage/chondroid producing tumors

A

Endochondroma, osteochondroma, chondrosarcoma, chondroblastoma, chondromyxoid fibroma

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3
Q

Bone producing tumors

A

Osteoid osteoma/osteoblastoma, osteosarcoma

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4
Q

No matrix/unknown cell type

A

Giant cell tumor of bone, non-ossifying fibroma, Ewing sarcoma, aneurysmal bone cyst, fibrous dysplasia

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5
Q

Enchondroma definition

A

Benign tumor of hyaline cartilage

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6
Q

Enchondroma age and sex

A

20-50 years old, males and females equally affected

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7
Q

Enchondroma common locations

A

Metaphysis of long bones, tubular bones of hands and feet

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8
Q

Enchondroma gross appearance

A

Lobules of cartilage in medullary cavity

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9
Q

Enchondroma histologic features

A

Circumscribed lobules of cartilage with low cellularity

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10
Q

Enchondroma syndromes

A

Ollier’s disease and Maffucci syndrome

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11
Q

Osteochondroma definition

A

Benign mixed tumor

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12
Q

Osteochondroma age and sex

A

10-30 years, males and females equally affected

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13
Q

Osteochondroma locations

A

Long bone metaphysis

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14
Q

Osteochondroma gross appearance

A

Mushroom shaped growth at metaphysis

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15
Q

Osteochondroma histologic features

A

Cartilage cap with underlying bone and marrow in stalk, cap points away from joint

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16
Q

Osteochondroma syndromes

A

Multiple hereditary exostosis (MHE)

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17
Q

Chondrosarcoma definition

A

Malignant sarcoma that forms cartilage

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18
Q

Chondrosarcoma age and sex

A

50-70 years, unless associated with syndrome, males and females equally affected

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19
Q

Chondrosarcoma locations

A

Axial skeleton more often than appendicular skeleton

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20
Q

Chondrosarcoma gross appearance

A

Can look like enchondroma but often shows destruction of cortex and soft tissue invasion

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21
Q

Chondrosarcoma histologic features

A

Lobules of more cellular cartilage that entrap host trabecular bone and destroy cortex

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22
Q

Chondrosarcoma syndromes

A

Ollier’s disease, Maffucci syndrome, multiple hereditary exostosis (MHE), the most common sarcoma in Paget’s disease

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23
Q

Osteoid osteoma/osteoblastoma definition

A

Circumscribed benign bone tumors

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24
Q

Osteoid osteoma/osteoblastoma age and sex

A

10-20 years, males more often affected than females

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25
Q

Osteoid osteoma/osteoblastoma locations

A

Osteoid osteoma - metaphysis of long bone

Osteoblastoma - spine

26
Q

Osteoid osteoma/osteoblastoma gross appearance

A

Central lucent area surrounded by abundant new bone

27
Q

Osteoid osteoma/osteoblastoma histologic features

A

Central lytic bone forming area with reactive surrounding sclerosis

28
Q

Osteoid osteoma/osteoblastoma syndromes

A

None

29
Q

Osteosarcoma definition

A

Malignant bone forming sarcoma

30
Q

Osteosarcoma age and sex

A

Bimodal age, but most are younger than 20 years old, males are affected more than females

31
Q

Osteosarcoma locations

A

Metaphysis of long bones, especially of knee

32
Q

Osteosarcoma gross appearance

A

Destructive tumor with bone with or without cartilaginous area

33
Q

Osteosarcoma histologic features

A

Malignant osteoblasts form immature tumor bone

34
Q

Osteosarcoma syndromes

A

Hereditary retinoblastoma, Li-Fraumeni, others

35
Q

Giant cell tumor of bone definition

A

Benign in almost all cases but has aggressive local destructive growth, non-aggressive “metastases”

36
Q

Giant cell tumor of bone age and sex

A

Adults, 20-40 years old (epiphysis closed), males and females equally affected

37
Q

Giant cell tumor of bone locations

A

Epiphysis (can invade into metaphysis and diaphysis) of long bones, distal radius, pelvis

38
Q

Giant cell tumor gross appearance

A

Large lesion located in the epiphysis of bone, sometimes surrounded by smaller, similar looking lesions

39
Q

Giant cell tumor of bone histologic features

A

Numerous osteoclasts evenly distributed in a background of plump spindle cells, osteoclasts (multinucleated giant cells) are reactive, osteoblast progenitor cells are neoplastic (express high levels of RANKL)

40
Q

Giant cell tumor of bone syndromes

A

None

41
Q

Ewing sarcoma definition

A

Malignant, high grade sarcoma

42
Q

Ewing sarcoma age and sex

A

Most under the age of 20, males affected more often than females

43
Q

Ewing sarcoma locations

A

Diaphysis of long bones and pelvic bones

44
Q

Ewing sarcoma gross appearance

A

“Onion skin” appearance of bone

45
Q

Ewing sarcoma histologic features

A

Small round blue cell tumor (undifferentiated blastic appearing cells), often shows membranous reactivity for CD99 (MYC) in immunohistochemistry

46
Q

Ewing sarcoma syndromes

A

None

47
Q

Fibrous dysplasia definition

A

Benign bone lesion that some consider a localized developmental arrest

48
Q

Fibrous dysplasia age and sex

A

Adolescence (before epiphysis closes), males and females equally affected

49
Q

Fibrous dysplasia locations

A

Knee, ribs, skull, gnathic, humerus, pelvic bones (pelvis in polyostotic only), can be any bone

50
Q

Fibrous dysplasia gross appearance

A

The normal components of bone are present, but do not differentiate into mature structures

51
Q

Fibrous dysplasia histologic features

A

Circumscribed intramedullary nodule of “Chinese character” or alphabet soup of woven bone spicules set in a fibrous matrix, cartilage present in 20% of cases

52
Q

Fibrous dysplasia syndromes

A

Polyostotic (one side of the body), Mazabraud syndrome, McCune-Albright syndrome

53
Q

Effects of metastases to bone

A

Pathologic fractures, factors released locally by tumors (such as RANKL) lead to lytic lesions or other factors that lead to increased reactive density (sclerotic lesions), bone pain, osteoporosis (tumor-induced), hypercalcemia

54
Q

Osteosarcoma mutations

A

CDKN2A, Rb1, p53, MDM2 CDK4

55
Q

Ewing sarcoma mutations

A

FLI-1-EWS or ERG-EWS, t(11;22), t(21;22)

56
Q

Chondrosarcoma mutations

A

EXT1 or EXT2 (with MHE), IDH1 or IDH2 (with Ollier’s/Maffucci), CDKN2A

57
Q

Osteochondroma mutations

A

EXT1 or EXT2

58
Q

Osteoid osteoma/osteoblastoma mutations

A

Unknown

59
Q

Enchondroma mutations

A

IDH1 or IDH2

60
Q

Giant cell tumor of bone mutations

A

H3F3A

61
Q

Fibrous dysplasia mutations

A

GNAS1