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fibrous tumors Flashcards

1
Q

FCD age

A

4-8 yo

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2
Q

NOF age

A

8-20

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3
Q

FCD/NOF estimated %

A

50% boys and 20% girls under 2 yo

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4
Q

FCD/NOF location

A

LTB 90%
-tibia/femur

**UE uncommon

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5
Q

if FCD/NOF at site other than long bone may be called….

A

benign fibrous histiocytoma or fibroxanthomas

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6
Q

bone location FCD/NOF

A

metaphysis but can migrate to diaphysis w/ growth

-medial and posterior wall more common

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7
Q

rad appearance FCD/NOF

A

“bone blister”

cortical eccentric geographic bubbly lucent lesion w/ sclerotic borders

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8
Q

if >____% of the diameter of the bone involved w/ FCD/NOF then it may pathologically fx

A

50

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9
Q

bone scan FCD/NOF

A

may show mild uptake

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10
Q

MR FCD/NOF

A

low T1; variable T2

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11
Q

Jaffe-Campanacci Syndrome

A

consists of multiple NOF ( >3) and extra skeletal abnormalities

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12
Q

extra skeletal abnormalities assoc w/ Jaffe-Campanacci Syndrome

A

cafe-au-lait spots, mental retardation, hypogonadism or cryptorchidism, ocular abnormalities, and CV malformations

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13
Q

lesion in the jaw assoc w/ Jaffe-Campanacci Syndrome

A

giant cell reparative granuloma

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14
Q

prognosis Jaffe-Campanacci Syndrome

A

spontaneous resolution; bowing of bones or pathological fx; can look aggressive

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15
Q

periosteal desmoid history

A

trauma or chronic activity

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16
Q

periosteal desmoid often designated…

A

avulsive cortical irregularity or tug lesion

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17
Q

periosteal desmoid AKA

A

adductor splints

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18
Q

periosteal desmoid muscles most affected

A

adductor magnus or medial head of gastroc

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19
Q

location periosteal desmoid

A

posteromedial cortex of distal femur (adjacent to condyle)

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20
Q

rad appearance periosteal desmoid

A

saucer like defect of cortex w/ adjacent sclerosis and periostitis

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21
Q

MR periosteal desmoid

A

marrow edema

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22
Q

desmoplastic fibroma age

A

2-3rd decade

75% <30 yo

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23
Q

desmoplastic fibroma clinical symptoms

A

pain and swelling

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24
Q

desmoplastic fibroma location

A

mandible, LTB (femur, humerus, tibia, radius, innominate

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25
Q

desmoplastic fibroma bone location

A

M
D possible
E unusual

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26
Q

desmoplastic fibroma rad appearance

A

lytic w/ trabeculated soap bubble or honeycomb appearance

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27
Q

desmoplastic fibroma may resemble…

A

NOF, CMF, GCT, ABC, FD

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28
Q

ddx desmoplastic fibroma

A

malignant/aggressive tumors

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29
Q

prognosis desmoplastic fibroma

A

wide resection is curative

recurrence can occur

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30
Q

fibrosarcoma may occur…

A

de novo or 2nd to paget’s, AVN, chronic OM, irradiation, or dedifferentiation of other neoplasms (chondrosarc)

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31
Q

fibrosarcoma age

A

30-60yo

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32
Q

fibrosarcoma symptoms

A

pain, swelling, decrease ROM

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33
Q

fibrosarcoma location

A

LTB

33-80% around knee

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34
Q

fibrosarcoma bone location

A

M or MD

extension into E not common

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35
Q

fibrosarcoma histologically similar to…

A

MFH

FS has herringbone pattern and MFH may be spiral like

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36
Q

fibrosarcoma absence of…

A

calcification or ossification

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37
Q

fibrosarcoma rad appearance

A

lytic foci w/ destruction
centric or eccentric
cortical destruction and ST mass

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38
Q

fibrosarcoma management

A 
surgical resection/amputation
can reoccur (more than once)
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39
Q

fibrosarcoma of bone carry poorer prognosis than of….

A

ST

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40
Q

lichtenstein’s rule

A

unidentifiable aggressive tumor about the knee in adult must have FS as prime consideration

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41
Q

DDX fibrosarcoma

A 
MFH
telangiectatic OS
lymphoma/plasmactyoma
desmoplastic fibroma
mets
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42
Q

fibrous histiocytoma AKA

A

fibroxanthoma

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43
Q

fibrous histiocytoma location

A

sacrum/ilium

E/D of tubular bones

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44
Q

GCT F or M?

A

F

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45
Q

GCT % benign and malignant

A

80/20

46
Q

GCT clinical symptoms

A

aggravated by activity

47
Q

GCT location

A

LTB

femur, tibia, radius humerus

48
Q

% GCT knee

A

50-65%

49
Q

% GCT at distal femur/radius and proximal tibia

A

50%

50
Q

GCT bone location

A

M and ext to E to subarticular bone

51
Q

GCT MC area in the spine

A

sacrum 8%

52
Q

MC benign tumor of the sacrum

A

GCT

53
Q

GCT in sacrum

A

appears aggressive w/ ST mass

54
Q

bone scan GCT

A

doughnut configuration

extended uptake pattern from tumor and adjacent hyperemia

55
Q

GCT MRI

A

Low T1 and high T2

fluid levels may represent 2nd ABC

56
Q

CT GCT

A

may show fluid levels

extent of tumor

57
Q

recurrence of GCT

A

generally seen w/in 2 yrs

40-60%

58
Q

% GCT that met and to where

A

2-5% to lung

59
Q

most malignant transformation of GCT occurs after…

A

radiation treatment

60
Q

bones affected in multi centric GCT

A

hands

61
Q

Goltz’s Syndrome

A

focal dermal hypoplasia, ocular defects, and skeletal abnormalities

62
Q

Goltz’s Syndrome assoc w/…(2)

A

solitary/multifocal GCT and osteopathia striata…

63
Q

giant cell reparative granuloma represents ____% of all benign tumors of the _____; gender predominance in this location

A

<10; jaw; female

64
Q

giant cell reparative granuloma location

A

craniofacial - maxilla, mandible, facial bones, sinuses

hands, wrist, ankles

65
Q

giant cell reparative granuloma may have predilection for…

A

tibial tuberosity

66
Q

rad appearance giant cell reparative granuloma craniofacial bones

A

round lucent lesion
don’t calcify**
trabeculated/expansile
may contain ossification

67
Q

giant cell reparative granuloma age

A

6-53 yo

68
Q

DDX of giant cell reparative granuloma in hands

A

enchondroma, ABC, GCT

69
Q

giant cell reparative granuloma similar to…

A

brown tumor and NOF

70
Q

MFH is MC…

A

malignant ST tumor

71
Q

MFH can occur de novo or w/ other abnormalities….

A

bone infarct, intraosseous lipoma, Paget’s, after radiation

-Hardcastle syndrome

72
Q

hard castle syndrome

A

rare familial skeletal dysplasia characterized by path fxs, diaphyseal sclerosis, and marrow infarction w/ necrosis)

73
Q

age MFH

A

40-70 yo

74
Q

MFH location

A

similar to OS
TB 75%
LE

75
Q

MFH rare in…

A

patella and hands/ft

76
Q

bone location MFH

A

M w/ frequent extension into E/D

77
Q

% of path fx in MFH

A

30-50%

78
Q

MFH percentage in LB

A

75%

79
Q

cortical destruction and ST ext in % of MFH

A

80-100

80
Q

rad appearance MFH bone

A 
lytic destruction, cortical erosion, limited periostitis, ST mass
bubbly lytic (expansion unusual)
81
Q

CT MFH

A

hemorrhage/necrosis common

82
Q

MR MFH

A

T1 similar to muscle; T2 hetero high to muscle

may have fluid fluid levels

83
Q

ST survival MFH

A

50-70%

84
Q

ST MFH mets to…

A

lung 90%

85
Q

bone mets % MFH

A

45-50% lung

86
Q

DDX ST MFH

A

liposarc, leiomyoma, rhabdomyosarcoma

87
Q

prognosis MFH

A

guarded
80% reoccur
mets to LN and distant sites

88
Q

cemento-ossifying fibroma AKA

A

cementoma

89
Q

age cementoma

A

3-4th decade

90
Q

M or F? cementoma

A

F

91
Q

most frequently occurs in what region of the mandible? cementoma

A

premolar

teeth often displaced

92
Q

rad appearance cementoma

A

well circumscribed, expansile mass

don’t usually cause cortical breach

93
Q

cementomas can become large in…

A

maxilla or paranasal sinuses

94
Q

MR cementoma

A

low T1 and T2

95
Q

management/recurrence cementoma

A

surgical excision/bone graft

recurrence not common 28%

96
Q

FD frequently involved sites

A

skull, pelvis, spine, shoulder girdle

97
Q

polyostotic FD usually recognized before…

A

10 yo

98
Q

FD skull and facial bones % of mono and poly

A

10-25% mono

50% poly

99
Q

FD in orbital/periorbital bones causes…

A

hypertelorism (wide set eyes), displacement of globe, exophthalmos, diplopia, visual impairment

100
Q

FD tubular bone location

A

D and intramedullary

101
Q

FD femoral neck ….

A

shephard’s crook deformity

102
Q

LB rad appearance of FD

A

ground glass
rind of sclerosis
endosteal scalloping
septations, expansion, and bowing deformities

103
Q

MC benign tumor of the ribs…

A

FD

104
Q

FD makes up ____% of primary benign chest wall lesions

A

30

105
Q

FD MRI

A

low T1 and variable T2

106
Q

malignant degeneration FD %

A

RARE 0.4-1%

107
Q

malignant degeneration FD

A

OS, FS, MFH, CS

108
Q

Mazabraud’s syndrome

A

benign myxomas assoc w/ FD (polyostotic, F)

109
Q

McCune-Albright syndrome

A

poly FD w/ precocious puberty and abnormal cutaneous pigmentation

110
Q

assoc of McCune-Albright syndrome…

A

hypophosphatemic rickets and osteomalacia

Tumors (3 decks)

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