Chapter 143 - Benign Bone Tumors and Reactive Lesions Flashcards

1
Q

Osteoid osteoma - presentation

A

young patient
Male 2:1
night pain relieve by NSAIDs

Common locations: Femur, tibia, posterior vertebral elements, humerus
- proximal femur is most common
- hip is most common intra-articular location

Metaphysis or diaphysis - diaphysis more common

if related to a scoli - concavity of the curve and 2/2 paraspinal spasm 2/2 lesion pain - take the lesion out, scoli resolves

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2
Q

Osteoid Osteoma - radiology

A

round, well circumscribed intra-cortical lesion with a radiolucent nidus
- with a periosteal reaction

intense focal uptake on technetium 99 scans

MRI with extensive surrounding edema

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3
Q

Osteoid Osteoma - path

A
  • trabeculae lined with plump osteoblasts
  • on low power images - sharply demarcated nidus surrounded by dense cortical bone
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4
Q

Osteoid osteoma - treatment

A
  • radiofrequency ablation

EXCEPT IN SPINE
- resection vs burring

  • in proximal femur - will need bone grafting and internal fixation as well
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5
Q

Osteoblastoma - presentation

A

rare, aggressive, benign osteoblastic tumor
between age 10-30
male2:female1

genetics: FOS/FOSB rearrangements

slowly progressive, dull aching pain, less severe than osteoid osteoma

NO night pain, NSAIDS dont help

COmmon locations:
- posterior elements of spine (number 1)
metaphysis/diaphysis of tibia/femur

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6
Q

Osteoblatoma - radiographic features

A

radiolucent lesion 2-10 cm

2/3 are cortical based, 1/3 are medullary

expansile, invades soft tissue, with rim of reactive bone

ddx:
osteosarc, abc, osteomyelitis, osteoid osteoma

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7
Q

Osteoblastoma - path

A

interwoven trabeculae with fibrovascular connective tissue

giant cells present

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8
Q

differentiating osteoid osteoma from osteoblastoma

A
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9
Q

osteoblastoma - treatment

A

curretage and bone grafting

en bloc resection for spinal lesions
- leave the nerve roots

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10
Q

parosteal osteoma - presentation

A

females>males
30s-40s yo

often a history of local trauma

long history of gradual swelling or dull pain

classically found int he craniofacial bones
less frequently is tibia/femur, pelvis, vertebrae

multiple lesions: gardner syndrome - colonic polyps, fibromatosis, cutaneous lesions, sub-cu lesions - autosomal dominant

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11
Q

paroseal osteoma - radiology

A

uniform, radiodense lesion, broad base
1-8cm
NO cortical or medullary invasion

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12
Q

parosteal osteoma - path

A

mature, hypocellular lamellar bone

no atypia

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13
Q

enchondroma presenation

A

benign bone tumor composed of mature hyaline cartilage within the medullary canal

age 20-50

incidentaloma

IDH1, IDH2 somatic mutations

enchondroma is the most common bone tumor in the hand
other common locations: proximal humerus, distal femur, proximal tibia

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14
Q

enchondroma - radiology

A

well defined, lucent, cental medulalr lesion with stippled calcifications

1-10 cm

MINIMAL cortical erosion (<50% width of the cortex)
except in the hands where cortices may be thinned and expanded

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15
Q

enchondroma - pathology

A

blue-gray lobulated cartilage variable amount of calcification

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16
Q

enchondroma - treatment

A

none necessary

curretage and grafting if painful

pathologic fx, especially in small bone should be allowed to heal before curretage and grafting

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17
Q

enchondroma - related conditions

A

Ollier’s disease
- multiple enchondromas
- tend to be on one side of the body
- sporadic inheritance
- failure of normal endochondral ossification
- IDH1/IDH2 mutations
- increased risk of chondrosarcoma transformation of an enchondroma - 25-30%

Maffucci Syndrome
- multiple enchondromas + soft tissue angiomas
- angiomas present on XR as phleboliths
- IDH1/IDH2 mutations
- increased risk of malignant transformation of an enchondroma to a low grade chondrosarcoma
- very high risk of developing retroperitoneal soft tissue sarcomas (fatal visceral malignancy)

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18
Q

osteochondroma - demographics

A

most common benign bone tumor

most identified in the first two decades of life

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19
Q

oteochondroma - genetics

A

trapped growth plate cartilage that herniates thru the cortex

defect in the perichondral node of ranvier

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20
Q

osteochondroma - presentation

A

most are solitary and asymptomatic
range from <3cm to 15cm

continue to grow until skeletal maturity

occur most commonly around the knee
- distal femur
- proximal tibia
also: proximal humerus, pelvis (high rate of malignant transformation), and posterior elements of spine

risk of malignant degeneration of solitary osteochondroma 1%

sessile lesions more commonly malignant transformation

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21
Q

osteochondroma - treatment

A

try to delay until skeletal maturity to decrease risk of recurrence

surrounding perichondrium over cartilage cap needs to be removed in order to prevent local recurrence

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22
Q

Multiple hereditary exostosis

A

EXT1/EXT2 gene defect -> heparan sulfate deficiency, autosomal dominant

risk of malignant transformation id higher than solitary osteochondroma (5-10%)

most common location of secondary chondrosarcoma is a sessile osteochondroma on the pelvis

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23
Q

Chondroblastoma - presentation

A

rare, benign bone tumor differentiated by giant cell by chondroid matrix

male2:female1
<25yo

location of lesions:
surround the knee - distal femur epiphysis most common, proximal tibial epiphysis next, proximal humerus, proximal femur, calcaneus

can develop benign pulm mets

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24
Q

Chondroblastoma - imaging

A

small round tumors - epiphyseal or apophyseal
+/- extension to the metaphysis
1-4cm
sclerotic rim
cortical expansion often present, invasion of surrounding soft tissue is not
stippled calcifications in 40%

MRI will show extensive edema around the lesion

25
Q

chondroblastoma - path

A
  • background of mononuclear cells
    few/scattered giant cells
    focal chondroid matrix

S100+!!!

chicken wire calcifications

26
Q

chondroblastoma - treatment

A

curretage and grafting
phenol, liquid nitrogen, or argon adjuvant

27
Q

chondromyxoid fibroma - presentation

A

2nd/3rd decades of life
slight male predilection

painful lesions

long bones of the lower extremity/pelvis

28
Q

chondromyxoid fibroma - imaging

A

lucent lesion
ECCENTRIC
Metaphyseal long bone lesion
thinning and expansion of cortical bone
sharp, scalloped rim - shark bite
rare to have intralesional calcifications

29
Q

chondromyxoid fibroma - path

A

look for THREE regions: chondroid, myxoid, fibromatous

  • lobulated with peripheral hypercellularity
  • within lobules, spindle shaped cells

areas of bizarre nuclei are common

30
Q

Non-ossifying fibroma - presentation

A
  • children 5-15
  • up to 30% of kids with open physes wil have an NOF

usually incidental finding

can be assoicated with
neurofibromatosis
Jaffee-Campanacci syndrome (cafe au lait spots, mental retardation, heart, eyes, gonad abnormalities)

common locations:
- long bones of lower extremities (80%)

+/- pathologic fracture

31
Q

non-ossifying fibroma - radiology

A
  • eccentric
  • lytic
  • cortically based with sclerotic rim
  • metaphyseal but migrate toward the diaphysis with age
32
Q

Non-ossifying fibroma - path

A
  • prominent hemosiderin component
  • foamy macrophages (xanthomatous reaction)
  • prominent storiform pattern of fibrohystiocytic cells
  • variable giant cell #
33
Q

treatment - non-ossifying fibroma

A

pathologic fractures are allowed to heal then curretage and grafting

most will resolve spontaneously

curettage and grafting if very symptomatic

34
Q

Fibrous Dysplasia - presentation

A

hamartomatous fibro-osseous proliferation within bone

females>males, generally age <30

inability to produce mature lamellar bone

common locations:
facial involvement, maxilla, proximal femur, rib, tibia

35
Q

fibrous dysplasia genetics

A

GNASalpha mutation -> perpetually active adenylate cyclase-cyclic adenosine monophosphate activation

36
Q

mazabraud syndrome

A

polyostotic fibrous dysplasia
multiple intramuscular myxoma

females>males, lower etremities

37
Q

McCune Albright syndrome

A

polyostotic fibrous dysplasia
precocious puberty
pigmented skin lesions (irregular borders)

unilateral bone lesions, unilateral skin lesions

38
Q

fibrous dysplasia - radiology

A

central lytic lesion within the medullary canal, usually diaphysis/metaphysis

expansile with cortical thinning

ground glass appearance

39
Q

fibrous dysplasia - path

A

gross tissue: yellow-white, gritty tissue

micro: poorly mineralized immature fibrous tissue surrounding islands of irregular poorly mineralized trabeculae of woven bone

40
Q

fibrous dysplasia - treatment

A

curettage and CORTICAL grafting
- dont use cancellous graft, it will just get replaced by dysplastic bone

*** Bisphosphonates helpful here!

41
Q

osteofibrous dysplasia

A

males>females
kids <10
associated with trisomy 7/8/12/22

anterior tibial shaft lesion - well defined only anterior tibial cortex

avoid surgery - brace if necessary
spontaneously regress at skeletal maturity

42
Q

desmoplastic fibroma

A

honeycomb/trabeculated appearance
tumor is hypocellular and similar to scar tissue

tx is curettage and grafting
wide resection in expendable bones

43
Q

Langerhans cell histiocytosis - presentation

A

clonal disease
CD1a positive immature dendritic cells
BRAF oncogene positive

kids <20
male 2:1

common location: skull, rib, scapula, vertebrae (thoracic>lumbsr>cervical), long bones, pelvis

44
Q

langerhans cell histiocytosis - rads

A

punched out lesion with thick periosteal reaction
vertebral plana

45
Q

Langerhans cell histiocytosis - path

A

histiocytes with coffee bean nuclei
eosinophilic cytoplasm
CD1a staining
giant cells often present

scanning electron microscopy - birbeck granules - tennis racket shaped granules

46
Q

langerhans cell histiocytosis - treatment

A

solitary lesions: methylprednisolone injection
curettage and grafting
vertebral plana: bracing alone

47
Q

unicameral bone cyst - presentation

A

serous fluid filled bone cyst
patients <20
pathologic fracture after minor trauma

most common locations:
proximal humerus
proximal femur
ileum, calc less common

48
Q

Unicameral bone cyst imaging

A

purely lytic lesion in medullary canal

cortical thinning with no soft tissue extension

bone expansion does not exceed width of physis

fallen leaf sign

49
Q

unicameral bone cyst - path

A

clear, serous fluid in cavity

cyst lining is thin, fibrous membrane

cementum spherules (calcified eosinophilic fibrinous material)

50
Q

unicameral bone cyst - treatment

A

injection of methylprednisolone

51
Q

aneurysmal bone cyst - presentation

A

75% younger than 20
USP6 mutations

painful lesion, with associated swelling

most common locations: distal femur, proximal tibia, pelvis, spine (posterior elements)

52
Q

aneurysmal bone cyst - radiology

A

eccentric lytic lesion
metaphyseal lesion
aggressive destruction and expnsion of the cortex with soft tissue infiltration
periosteal rim
can expand wider than the epiphyseal plate

t2 mri with fluid fluid levels

53
Q

ABC - path

A

blood filled cyst
solid areas are common
no cellular atypia, but mitoses are common

54
Q

abc - treatment

A

curettage and grafting

highest local recurrence in young patients with open physeal plates

embolization or sclerotherapy for pelvic/spinal lesions that cant be resected

55
Q

Giant cell tumor of bone - presentation

A

benign, agressive bone lesion
age 30-50
females>males
c-myc, n-myc, c-fos oncogene mutations

pain x 2-3 months with swelling
decreased ROM around a joint

locations:
distal femur
proximal tibia
distal radius, proximal humerus, proximal femur, sacrum, pelvis

56
Q

giant cell tumor of bone - radiology

A

eccentric, lytic lesion of the epiphysis, metaphysis of long bones

can extend to the subchondral surface without a sclerotic rim

ANTERIOR vertebral body when spine is involved

commonly have an ABC component secondarily

57
Q

giant cell tumor of bone - path

A

gross specimen: soft, red-brown, hemorrhagic, necrotic

micro: tons of multinucleated gint cells in a background of stromal cells (the stromal cells are actually the neoplastic cell)

58
Q

GCT of bone - treatment

A

thorough curettage and high speed burr
methylmethacrylate back fill
embolization should be used for pelvic or spinal lesions along with surgery

denosumab is FDA approved for unresectable GCT - causes GCT to ossify

benign pulmonary mets in 2%