B6.077 Bone Tumors (Part 2) Flashcards

1
Q

epidemiology of unicameral (simple) bone cyst

A

common lesion found in kids

occasionally in adults

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2
Q

location of unicameral (simple) bone cyst

A

centrally located in the bone (medullary canal)

usually metaphyseal

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3
Q

presentation of unicameral (simple) bone cyst

A

usually presents with fracture

no known risk factors

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4
Q

treatment of unicameral (simple) bone cyst

A

observation
steroid injection
BM injection
curettage / grafting
**30% recurrence with any treatment, likely due to underlying vascular abnormality
goal is to keep kids achieving their developmental milestones

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5
Q

appearance of unicameral (simple) bone cyst on xray

A

sharp distinction
lytic areas of fluid / blood
ground glass area where lytic lesions healed

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6
Q

unicameral (simple) bone cyst on histo

A

spindle cells
open spaces
no necrosis / mitotic activity

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7
Q

epidemiology of osteoid osteoma

A

teens and early 20s

M:F = 2:1

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8
Q

location of osteoid osteoma

A
< 2 cm
appendicular skeleton (femur or tibia 50%)
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9
Q

symptoms of osteoid osteoma

A

painful
excess PGE2 produced by proliferating osteoblasts (causes swelling within bone, leading to pain)
pain relieved by aspirin ( can be used long term)

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10
Q

treatment of osteoid osteoma

A

radiofrequency ablation

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11
Q

appearance of osteoid osteoma on xray

A

active bone around lesion

presence of nidus within lesion

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12
Q

osteoid osteoma on histo

A

nidus surrounded by reactive bone
spindles in background
osteoblastic rimming

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13
Q

presentation of osteoid osteoma in the spine

A

painful scoliosis
lesion on concave side of spinal curve
-PGE causes contraction of muscles

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14
Q

appearance of osteochondroma on xray

A

sharp transition
medullary canal of bone contiguous with medullary canal of lesion
cortex of bone contiguous with cortex of lesion

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15
Q

appearance of osteochondroma on CT

A

bone present behind cartilage cap on lesion

reactive bone

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16
Q

appearance of osteochondroma on histo

A

looks like a physes, but less organized

cartilage cap on reactive bone

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17
Q

what is an osteochondroma

A

exostosis

benign cartilage capped outgrowth that is attached to the skeleton by a bony stalk

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18
Q

hereditary osteochondromas genetics

A

multiple hereditary exostosis (multiple osteochondromas)

EXT1 and EXT2 mutations

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19
Q

solitary osteochondromas genetics

A

85% EXT1 mutation

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20
Q

what is the EXT gene

A

in growth plate chondrocytes

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21
Q

prognosis / treatment of osteochondroma

A

1% risk of transformation to chondrosarcoma per lesion
multiple lesions increases risk
don’t usually remove unless symptomatic

22
Q

periosteal chondroma location

A

sits on the surface of bone
arises in only one bone (don’t need to look for other lesions)
arises from cambium layer of periosteum

23
Q

treatment of periosteal chondroma

A

usually resected due to risk of enlargement

24
Q

periosteal chondroma on imaging

A

intact cortex with mineralized lesion on top

25
Q

periosteal chondroma on histo

A

more cellular than a lesion within bone

has lacunae with cartilage and matrix and fibrous tissue

26
Q

presentation of enchondroma

A

frequently presents in hand after fracture

radiographically and histologically more aggressive in the hand

27
Q

treatment of enchondroma

A

usually surgical intervention in the hand
intervention not necessary in other locations unless concern about more aggressive lesion
-follow for increasing lytic component (growth without mineralization)

28
Q

appearance of enchondroma on histo

A

less matrix and more cellularity than normal cartilage

histologically inactive

29
Q

appearance of enchondroma on xray

A

popcorn areas within bone

30
Q

typical location of enchondromas

A

usually solitary
metaphyseal region
develop from rests of growth plate cartilage

31
Q

syndromes associated with enchondromas

A

Ollier’s disease- multiple enchondromas
Maffucci syndrome - enchondromatosis + soft tissue hemangiomas
-increased risk of malignancy in all body parts, unrelated to lesions

32
Q

prognosis of enchondromas

A

small risk (<1% per lesion) of malignancy transformation

33
Q

location of chondroblastoma

A

epiphyseal or apophyseal (next to growth plates)

34
Q

epidemiology of chondroblastoma

A

occurs in adolescents

35
Q

presentation of chondroblastoma

A

presents with acute onset of pain and swelling of the adjacent joint
similar to an infected joint

36
Q

evaluation of chondroblastoma

A

should be urgent, to not miss infected joint and to provide pain relief
MRI and labs can help rule out infection

37
Q

treatment of chondroblastoma

A

biopsy
curettage
grafting

38
Q

differentiation of low grade liposarcoma from lipoma

A

slightly more cellular
presence of lipoblasts
MDM2 increased in liposarcoma (test done after excision)

39
Q

treatment of lipomas

A

not all need to be removed

if consistent with fat on MRI, can be watched if less than 5 cm in greatest dimension and are not enlarging

40
Q

assessment of lipoma

A

assess for ring chromosome or MDM2 amplification (proto-oncogene amplified in liposarcomas)

41
Q

second most common soft tissue sarcoma

A

liposarcoma

42
Q

appearance of fibromatosis on xray

A

indentation in bone due to pressure effect of soft tissue mass

43
Q

histo appearance of fibromatosis

A

sheets of spindle cells and collagen

44
Q

characteristics of fibromatosis

A

benign but locally aggressive
no ability to metastasize
usually in response to trauma (injury response that doesn’t turn off)
may respond to estrogen (BCP can make it bigger)
hardest lesion that you’ll palpate, usually fixed

45
Q

epidemiology of fibromatosis

A

slightly higher incidence in women

46
Q

treatment of extra-abdominal desmoid

A

confirm diagnosis (vs low grade fibrosarcoma)
if symptomatic or enlarging, resect with wide margins
avoid amputation
amputation may be required if limb is at risk

47
Q

non surgical treatment of extra abdominal desmoid

A

variable course, may regress
responds to RT
possible use of SERMs or chemo for unresectable but symptomatic lesions

48
Q

what is an undifferentiated pleomorphic sarcoma

A

most common type of soft tissue sarcoma

unknown tissue type

49
Q

epidemiology of UPS

A

occurs in middle aged to older adults

50
Q

screening of UPS

A

CT of chest, as with all sarcomas

51
Q

treatment of UPS

A

radiation and surgical resection (wide margin)

chemo if at risk of spread or has metastasis

52
Q

features of UPS on histo

A

large cells with mitotic activity
spindle cells
clumping