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fibrous tumors Flashcards

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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
desmoplastic fibroma bone location
M D possible E unusual
26
desmoplastic fibroma rad appearance
lytic w/ trabeculated soap bubble or honeycomb appearance
27
desmoplastic fibroma may resemble...
NOF, CMF, GCT, ABC, FD
28
ddx desmoplastic fibroma
malignant/aggressive tumors
29
prognosis desmoplastic fibroma
wide resection is curative | recurrence can occur
30
fibrosarcoma may occur...
de novo or 2nd to paget's, AVN, chronic OM, irradiation, or dedifferentiation of other neoplasms (chondrosarc)
31
fibrosarcoma age
30-60yo
32
fibrosarcoma symptoms
pain, swelling, decrease ROM
33
fibrosarcoma location
LTB | 33-80% around knee
34
fibrosarcoma bone location
M or MD | extension into E not common
35
fibrosarcoma histologically similar to...
MFH FS has herringbone pattern and MFH may be spiral like
36
fibrosarcoma absence of...
calcification or ossification
37
fibrosarcoma rad appearance
lytic foci w/ destruction centric or eccentric cortical destruction and ST mass
38
fibrosarcoma management
``` surgical resection/amputation can reoccur (more than once) ```
39
fibrosarcoma of bone carry poorer prognosis than of....
ST
40
lichtenstein's rule
unidentifiable aggressive tumor about the knee in adult must have FS as prime consideration
41
DDX fibrosarcoma
``` MFH telangiectatic OS lymphoma/plasmactyoma desmoplastic fibroma mets ```
42
fibrous histiocytoma AKA
fibroxanthoma
43
fibrous histiocytoma location
sacrum/ilium | E/D of tubular bones
44
GCT F or M?
F
45
GCT % benign and malignant
80/20
46
GCT clinical symptoms
aggravated by activity
47
GCT location
LTB | femur, tibia, radius humerus
48
% GCT knee
50-65%
49
% GCT at distal femur/radius and proximal tibia
50%
50
GCT bone location
M and ext to E to subarticular bone
51
GCT MC area in the spine
sacrum 8%
52
MC benign tumor of the sacrum
GCT
53
GCT in sacrum
appears aggressive w/ ST mass
54
bone scan GCT
doughnut configuration | extended uptake pattern from tumor and adjacent hyperemia
55
GCT MRI
Low T1 and high T2 | fluid levels may represent 2nd ABC
56
CT GCT
may show fluid levels | extent of tumor
57
recurrence of GCT
generally seen w/in 2 yrs | 40-60%
58
% GCT that met and to where
2-5% to lung
59
most malignant transformation of GCT occurs after...
radiation treatment
60
bones affected in multi centric GCT
hands
61
Goltz's Syndrome
focal dermal hypoplasia, ocular defects, and skeletal abnormalities
62
Goltz's Syndrome assoc w/...(2)
solitary/multifocal GCT and osteopathia striata...
63
giant cell reparative granuloma represents ____% of all benign tumors of the _____; gender predominance in this location
<10; jaw; female
64
giant cell reparative granuloma location
craniofacial - maxilla, mandible, facial bones, sinuses | hands, wrist, ankles
65
giant cell reparative granuloma may have predilection for...
tibial tuberosity
66
rad appearance giant cell reparative granuloma craniofacial bones
round lucent lesion don't calcify** trabeculated/expansile may contain ossification
67
giant cell reparative granuloma age
6-53 yo
68
DDX of giant cell reparative granuloma in hands
enchondroma, ABC, GCT
69
giant cell reparative granuloma similar to...
brown tumor and NOF
70
MFH is MC...
malignant ST tumor
71
MFH can occur de novo or w/ other abnormalities....
bone infarct, intraosseous lipoma, Paget's, after radiation | -Hardcastle syndrome
72
hard castle syndrome
rare familial skeletal dysplasia characterized by path fxs, diaphyseal sclerosis, and marrow infarction w/ necrosis)
73
age MFH
40-70 yo
74
MFH location
similar to OS TB 75% LE
75
MFH rare in...
patella and hands/ft
76
bone location MFH
M w/ frequent extension into E/D
77
% of path fx in MFH
30-50%
78
MFH percentage in LB
75%
79
cortical destruction and ST ext in % of MFH
80-100
80
rad appearance MFH bone
``` lytic destruction, cortical erosion, limited periostitis, ST mass bubbly lytic (expansion unusual) ```
81
CT MFH
hemorrhage/necrosis common
82
MR MFH
T1 similar to muscle; T2 hetero high to muscle | may have fluid fluid levels
83
ST survival MFH
50-70%
84
ST MFH mets to...
lung 90%
85
bone mets % MFH
45-50% lung
86
DDX ST MFH
liposarc, leiomyoma, rhabdomyosarcoma
87
prognosis MFH
guarded 80% reoccur mets to LN and distant sites
88
cemento-ossifying fibroma AKA
cementoma
89
age cementoma
3-4th decade
90
M or F? cementoma
F
91
most frequently occurs in what region of the mandible? cementoma
premolar | teeth often displaced
92
rad appearance cementoma
well circumscribed, expansile mass | don't usually cause cortical breach
93
cementomas can become large in...
maxilla or paranasal sinuses
94
MR cementoma
low T1 and T2
95
management/recurrence cementoma
surgical excision/bone graft | recurrence not common 28%
96
FD frequently involved sites
skull, pelvis, spine, shoulder girdle
97
polyostotic FD usually recognized before...
10 yo
98
FD skull and facial bones % of mono and poly
10-25% mono | 50% poly
99
FD in orbital/periorbital bones causes...
hypertelorism (wide set eyes), displacement of globe, exophthalmos, diplopia, visual impairment
100
FD tubular bone location
D and intramedullary
101
FD femoral neck ....
shephard's crook deformity
102
LB rad appearance of FD
ground glass rind of sclerosis endosteal scalloping septations, expansion, and bowing deformities
103
MC benign tumor of the ribs...
FD
104
FD makes up ____% of primary benign chest wall lesions
30
105
FD MRI
low T1 and variable T2
106
malignant degeneration FD %
RARE 0.4-1%
107
malignant degeneration FD
OS, FS, MFH, CS
108
Mazabraud's syndrome
benign myxomas assoc w/ FD (polyostotic, F)
109
McCune-Albright syndrome
poly FD w/ precocious puberty and abnormal cutaneous pigmentation
110
assoc of McCune-Albright syndrome...
hypophosphatemic rickets and osteomalacia

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