Bone Tumors Overview

For Medical Students

Abdelfattah Fawzi Khdeir M.D.

Ortho & Trauma Resident
Hebron - 2014

A classification of bone tumors. Modified after Revised WHO Classification Schajowicz (1994)

HISTORY
Asymptomatic until the abnormality is discovered on x-ray.
Must be capable of spontaneous resolution
Situated where there is room for inconspicuous expansion
Age :
During childhood --- (fourth or sixth decades)
Myeloma is seldom seen before the sixth decade
In patients > 70 years of age, metastatic bone lesions are more
common than all primary tumors together.

HISTORY
Pain :
Rapid expansion with stretching of surrounding
tissues
Central haemorrhage
Degeneration in the tumour
Pathological fracture
Very painful if it is encapsulated in dense bone
(e.g. an osteoid osteoma).

HISTORY
Neurological symptoms (paraesthesiae or
numbness)
Pathological fracture may be the first (and only)
clinical signal.
In elderly people any break in the mid-shaft
should be regarded as pathological until proved
otherwise.

EXAMINATION
A lump, where does it arise? Is it discrete or ill-defined? Is it soft or
hard, or pulsatile? And is it tender?
Swelling is sometimes diffuse, and the overlying skin warm and
inflamed; it can be difficult to distinguish a tumor from infection or a
haematoma.
If the tumor is near a joint there may be an effusion and/or limitation of
movement.
Spinal lesions , whether benign or malignant, often cause muscle
spasm and back stiffness, or a painful scoliosis

X-RAYS

X-RAYS
Cortical thickening, a discrete lump, a cyst or ill-defined destruction.
Where is the lesion: in the metaphysis or the diaphysis?
Is it solitary or are there multiple lesions?
Are the margins well-defined or ill-defined?
Remember that cystic lesions are not necessarily hollow cavities: any
radiolucent material (e.g. a fibroma or a chondroma) may look like a
cyst.

X-RAYS
If the boundary of the cyst is sharply defined it is probably benign; if
it is hazy and diffuse it suggests an invasive tumour.
Stippled calcification inside a cystic area is characteristic of cartilage
tumors.
Look carefully at the bone surfaces: periosteal newbone formation and
extension of the tumour into the soft tissues are suggestive of
malignant change.
Look also at the soft tissues: Are the muscle planes distorted by
swelling? Is there calcification?

RADIONUCLIDE SCANNING
Scanning with 99mTc-methyl diphosphonate (99mTcMDP) shows non-specific reactive changes in bone
This can be helpful in revealing the site of a small
tumour (e.g. an osteoid osteoma) that does not show
up clearly on x-ray.
Is also useful for detecting skip lesions or silent
secondary deposits.

COMPUTED TOMOGRAPHY
It shows more accurately both intraosseous and
extraosseous extension
Reveal suspected lesions in inaccessible sites,
like the spine or pelvis

MRI
Its greatest value is in the assessment of tumor
spread: (a) within the bone, (b) into a nearby joint
and (c) into the soft tissues.
Blood vessels and the relationship of the tumor to
the perivascular space are well defined.
MRI is also useful in assessing soft-tissue tumours
and cartilaginous lesions.

 Tumors differential diagnosis

(a) This huge swelling was simply a clotted haematoma.
(b) Bone infection with pathological fracture.
(c) Florid callus in an un-united fracture.
(d) Large erosion in the calcaneum by a gouty tophus.
(e) Bone infarcts

STAGING OF BONE TUMORS

2 systems - one for malignant lesions and one for benign lesions
Malignant lesions are defined using Roman numerals (e.g. I, II, III)
e.g. osteosarcoma most commonly presents as stage IIB

Benign lesions are defined using Arabic numbers (1,2,3)

1 = Latent lesion
e.g. non-ossifying fibroma
enchondroma

2 = Active lesion
e.g. ABC, UBC
chondromyxoid fibroma
chondroblastoma

3 = Aggressive lesion
e.g. giant cell tumor of bone

STAGING OF BONE TUMOURS

STAGING OF BONE TUMOURS

Tumor Compartments
Intracompartmental
bone tumors are confined within the cortex of the bone

Extracompartmental
bone tumors extend beyond the bone cortex

Tumor Grade
Histologically, tumors are graded based on the percentage of cellular atypia
Low grade tumors
low metastatic potential
e.g. parosteal osteosarcoma

High grade tumors

greater metastastatic potential
e.g. intramedullary osteosarcoma, Ewing's sarcoma, dedifferentiated chondrosarcoma

MSTS (Enneking) Staging System

Musculoskeletal Tumor Society system

 A 13-year-old girl presents with an isolated distal femur

osteosarcoma that extends into the soft tissue. Work-up is negative
for metastasis, but biopsy reveals a high grade lesion. What is the
stage of this tumor by the Musculoskeletal Tumor Society system?

1. I A
2. II A
3. I B
4. II B
5. III

 (a) Plain x-ray shows a destructive lesion of the proximal tibia, almost certainly an osteosarcoma
(b,c) Coronal and sagittal MR images show the tumour extending medially, laterally and
posteriorly into the soft tissue.
(d) Transectional MRI shows that the abnormal tissue extends posteriorly right up to the vascular
compartment (arrow).
This tumour would be assessed as Stage IIB.

BENIGN BONE LESIONS

NON-OSSIFYING FIBROMA
(FIBROUS
CORTICAL DEFECT)
The commonest benign lesion of bone.
A developmental defect in which a nest of fibrous tissue
appears within the bone and persists for some years
before ossifying.
It is asymptomatic and is almost always encountered in
children as an incidental finding on x-ray.
The commonest sites are the metaphyses of long bones

NON-OSSIFYING FIBROMA
(FIBROUS
CORTICAL DEFECT)
Common in children 5-15 years old
(Estimated that 30% of children with open physis
have a nonossifying fibroma)
80% in lower extremity
Common locations include the knee (distal femur and
proximal tibia) and distal tibia

NON-OSSIFYING FIBROMA
(FIBROUS
CORTICAL DEFECT)
The x-ray appearance is metaphyseal eccentric "bubbly"
lytic lesion surrounded by sclerotic rim
views in different planes may show that a lesion that
appears to be central is actually adjacent to or within the
cortex, hence the alternative name fibrous cortical defect.
Although it looks cystic on x-ray, it is a solid lesion
consisting of unremarkable fibrous tissue with a few
scattered giant cells.

NON-OSSIFYING FIBROMA
(FIBROUS
CORTICAL DEFECT)

NON-OSSIFYING FIBROMA
(FIBROUS
CORTICAL DEFECT)
As the bone grows the defect becomes less obvious and it
eventually heals spontaneously.
There is no risk of malignant change.
Treatment is usually unnecessary.
If the defect is very large or has led to repeated fractures, it can be
treated by curettage and bone grafting.
Recurrence is rare.

FIBROUS DYSPLASIA
Areas of trabecular bone are replaced by
cellular fibrous tissue containing flecks of
osteoid and woven bone.
It may affect :
One bone (monostotic)
One limb(monomelic)
Many bones (polyostotic).

FIBROUS DYSPLASIA
Females > males
Found in any and all ages
Onset for 75% of patients at <30 years of age

Any bone can be involved

The proximal femur is most common site,followed
by rib, maxilla, and tibia

McCune Albright syndrome

Condition defined by :
The presence of skin abnormalities (cafe au lait spots)
Endocrine abnormalities :
(Precocious puberty)
Renal phosphate wasing due to FGF-23 (oncogenic
osteomalacia)
Polyostotic fibrous dysplasia

(Cafe au lait spots)

Larger and more irregular borders than neurofibromatosis

Polyostotic Fibrous Dysplasia

Punched-out" lesion with well defined margin of sclerotic bone

Highly lytic lesions or a ground glass appearance

Shepherds Crook Deformity

Treatment
Nonoperative
observation
indications
asymptomatic patients

Diphosphonate therapy
indications
symptomatic polyostotic fibrous dysplasia

effective in decreaseing pain and reducing bone turnover

Treatment
Operative
internal fixation and bone grafting
indications

symptomatic lesions
impending/actual fractures through lesions in areas of high stress (femoral neck)
severe deformity
neurologic compromise in the spine

technique
never use autogenous cancellous bone, as the transplanted bone will quickly turn into
fibrous dysplastic woven bone
use cortical or cancellous allografts
intramedullary device more effective than a plate in the lower extremity

Osteotomies for deformities

 Malignant transformation to fibrosarcoma

occurs in 0.5 per cent of patients with
monostotic lesions and up to 5 per cent of
patients with Albrights syndrome.

OSTEOID OSTEOMA
This tiny bone tumour (< 1.5 cm in diameter) causes
symptoms out of all proportion to its size.
Patients are usually under 30 years of age and males
predominate.
Any bone except the skull may be affected, but over half
the cases occur in the femur or tibia.
Typically the pain is relieved by salicylates.

OSTEOID OSTEOMA
50% in diaphysis or metaphysis of long bones of lower
extremity (tibia, femur)
proximal femur > tibia diaphysis > posterior elements of the spine >
fingers and carpus > feet

The most common location is the proximal femur

The most common intra-articular location is the hip joint
The most common locations in the hand are the scaphoid and
proximal phalanx

OSTEOID OSTEOMA
Pathophysiology
thought to be from nerve fibers associated with blood vessels within the nidus
pain is secondary to prostaglandin secretion and COX1/2 expression
COX1/2 expression in tumor makes it sensitive to NSAID therapy

Associated conditions
orthopaedic manifestations
painful scoliosis
growth disturbance
flexion contractures

Prognosis
pain from lesions usually resolves after an average of 3 years
the lesion spontaneously resolves in 5-7 years
in the spine, early resection (within 18 months) leads to resolution of scoliosis in
young children (<11years)

OSTEOID OSTEOMA

OSTEOID OSTEOMA
Symptoms
pain that

increases with time

worse at night and with drinking ETOH
relieved by NSAIDS
may be adjacent to joint and mimic arthritis

hand lesions may present with painless swelling

OSTEOID OSTEOMA
Physical exam
Joint effusions
Contractures
Limp
Muscle atrophy
May present as painful nonstructural scoliosis
As a result of paravertebral spasm
The osteoid osteoma is located on the concave side at
the apex of the curve

OSTEOID OSTEOMA
Radiographs
Intensly reactive bone around radiolucent nidus
Nidus is < 1.5 cm (otherwise osteoblastoma)
Nidus may be difficult to see on plain xray
Because intense periosteal reaction may obscure the nidus

OSTEOID OSTEOMA
The important x-ray feature is a small radiolucent area, the so-called nidus.
Lesions in the diaphysis are surrounded by dense sclerosis and cortical
thickening; this may be so marked that the nidus can be seen only in fine
cut CT scans.
Lesions in the metaphysis show less cortical thickening.
99mTc-MDP scintigraphy reveals intense, localized activity.
It is sometimes difficult to distinguish from a small Brodies abscess without
biopsy.

Osteoid osteoma The x-ray appearance depends on the site of the lesion.
(a) With cortical tumours there is marked reactive bone thickening leaving a small lucent nidus,.
(b) Lesions in cancellous bone produce far less periosteal reaction and are easily mistaken for a Brodies abscess.

OSTEOID OSTEOMA
CT :
Study of choice
To identify nidus surrounded by a sclerotic rim

OSTEOID OSTEOMA
Bone scan :
always hot with intense focal uptake

Treatment
Nonoperative
clinical observation and NSAID administration
indications
NSAIDS are 1st line and will lead to a dramatic decrease in
symptoms
~50% can be treated with NSAIDS alone
Also indicated for painful spine lesions without scoliosis
Fingertip lesions (distal phalanx) may not respond to NSAIDS

OSTEOID OSTEOMA

OSTEOID OSTEOMA
Percutaneous radiofrequency ablation
Relative indications
Failure of medical management
Periarticular lesions, which increase the risk of cartilage injury and premature degenerative disease.
Spinal lesions (controversial) - depends on the location of the lesion and proximity to neural elements

Contraindications
lesions close to spinal cord or nerve roots

Technique
done under CT guidance
probe at 80-90 deg C for 6 minutes to produce a 1cm zone of necrosis

Outcomes
90% of patients are successfully treated with 1-2 sessions of RFA
10-15% recurrence rate

OSTEOID OSTEOMA
Surgical resection indications
Location of lesion is not amenable to CT guided
percutaneous radiofrequency ablation
Spine lesion associated with painful scoliosis
If the excision is likely to weaken the host bone (especially
in the vulnerable medial cortex of the femoral neck),
prophylactic internal fixation may be needed.

Osteoblastoma
(GIANT OSTEOID OSTEOMA)

Aggressive benign osteoblastic tumor of bone

"big brother" of osteoid osteoma (nidus > 2cm)
Relatively rare
less common than osteoid osteoma
males > females (2:1)
majority of patients 10-30 years of age
location
most common in posterior elements of spine

Associated conditions
oncogenic osteomalacia
secondary ABC
10%-40% associated with secondary ABC

Osteoblastoma
Symptoms
Pain
slowly progressive dull aching pain
not relieved by NSAIDS

May see neurologic symptoms with spine involvement

Physical exam
Swelling
Muscle atrophy
Limp

Osteoblastoma
Radiographs
findings

lytic or mixed lytic-blastic lesion with radiolucent nidus > 2cm

reactive sclerotic bone
66% cortically based, 33% medullary based
often expansile with extension into soft tissues with rim of reactive bone
25% appear very aggressive and often mistaken for malignant lesion

CT
indications
necessary to fully evaluate lesion

Bone scan
hot with intense focal uptake

Osteoblastoma

Osteoblastoma

Osteoblastoma
Nonoperative
observation
indications
rarely, if ever, indicated as the lesion will continue to grow

Operative
curettage or marginal excision with bone grafting
indications
standard of care

recurrence 10-20%

Chondroma (a) The hand is a common site.

(b) Another chondroma before and after curettage and
bone grafting.

Enchondromas
A benign chondrogenic tumor composed of hyaline
cartilage
Located in the medullary cavity
Caused by an abnormality of chondroblast function in the
physis
Incidence
2nd most common benign cartilage lesion (osteochondroma is
most common)
Male:female ratio is 1:1
most common in 20-50 year olds

Enchondromas
location
usually found in the medullary cavity of
the diaphysis or metaphysis
the most common location is the hand (60%)
the most common bone tumor in the hand is the
enchondroma
other locations include the distal femur (20%), proximal
humerus (10%) and tibia
rare in the spine and pelvis

Enchondromas
Pathophysiology :
enchondromas represent incomplete
endochondral ossification
chondroblasts and fragments of epiphyseal
cartilage escape from the physis, displace into the
metaphysis and proliferate there

Enchondromas
Associated conditions
Solitary enchondroma
Ollier's disease (Multipe Enchondromatosis)
Sporadic inheritance with no genetic predisposition
Skeletal dysplasia with failure of normal endochondral
ossification
Enchondromas throughout
the metaphyses and diaphyses of long bones
involved bones are dysplastic, with shortening and bowing

Risk of malignant transformation <30%

Ollier's disease (Multipe Enchondromatosis)

Enchondromas
Maffucci's syndrome
Sporadic inheritance with no genetic predisposition
Multiple enchondromas and soft-tissue angiomas
Radiographically, enchondromas in Maffucci's syndrome markedly
expand the bone and angiomas are seen as small, round calcified
phleboliths
Risk of malignant transformation up to 100%
Also has increased risk of visceral malignancies (astrocytoma, GI
malignancy)

Maffucci's syndrome

Maffucci's syndrome

Enchondromas
Symptoms
Asymptomatic, discovered incidentally on
radiographs
usually true for enchondromas in long bones and foot

Pathologic fracture
often seen with enchondromas in the hand

Enchondromas

Enchondromas
Pain
Pain is uncommon
When a patient presents with an enchondroma and pain
in the adjacent joint, the cause of pain is often unrelated
to the tumor
Unlike enchondroma, most chondrosarcomas have nonmechanical pain (rest pain and nocturnal pain)

Enchondromas
Physical exam
Shortening and angular deformities
enchondromas may disrupt the growth plate

Multiple bluish angiomas in Maffucci's syndrome

Enchondromas

Enchondromas

Enchondromas
Radiographs
skeletal survey if polyostotic disease is suspected

Findings
Well defined, lucent, central medullary lesions that calcify
over time
1 to 10cm in size
metaphyseal location when they first appear
appear more diaphyseal as the long bone grows

Pop-corn" stippled calcification and rings

Minimal endosteal erosion (<50% width of cortex)

Pop-corn" stippled calcification and rings

- Cortical expansion and thinning may be present (Especially in the hand)

- May have purely lytic appearance (Especially in hand)

Ollier's disease
enchondromas markedly expand the bone
bones are dysplastic, with shortening and bowing

Maffucci's syndrome
Enchondromas markedly expand the bone
Angiomas are visible as calcified phleboliths

Enchondromas
Unlike enchondromas, chondrosarcomas display
Cortical thickening and destruction
Endosteal erosions and scalloping >50% of the width of the
cortex
Are larger (>5cm)

Enchondromas
Nonoperative
Observation
indications
Treatment for vast majority of asymptomatic enchondromas

Follow up
Serial radiographs for interval growth (every 3-6 months for 1-2
years, then annually)
Long term follow-up for patients with multiple enchondroma
syndromes

Enchondromas
Operative
intralesional curettage and bone grafting
indications
lesion that shows any change on serial xrays
radiographs suspicious for low-grade chondrosarcoma
large lesions at risk for recurrent fracture

outcomes
local recurrence is unusual

immobilization, followed by currettage and bone grafting

indications
pathologic fracture in small tubular bones (hand lesions)

technique
immobilize until fracture union, followed by currettage and grafting

Enchondromas
Malignant transformation
Risk of transformation of enchondroma to low-grade
chondrosarcoma
Solitary enchondroma
risk of transformation is 1%

Ollier's disease (multiple enchondromatosis)

risk of transformation is 25-30%

Maffucci's syndrome
risk of transformation is 23-100%
also has high risk of fatal visceral malignancy

Chondroblastoma
A benign chondrogenic lesion (differs from giant
cell tumor by its chondroid matrix)
2:1 male:female
most patients under 25 years of age

Location
Epiphyseal lesion in young patients (usually around 12 years
of age)
Common locations include distal femur, proximal tibia,
proximal humerus, proximal femur, and apophysis or triradiate
cartilage of the pelvis
Typically epiphyseal but may occasionally cross the physis

Chondroblastoma
Pathophysiology
Thought to arise from cartilaginous epiphyseal plate

Prognosis
1-2% of benign chondroblasts metastasize to the
lungs (similar to giant cell tumor in this respect)
Recurrence is 10%-15% after surgical resection

Chondroblastoma
Well-circumscribed epiphyseal lytic lesion with thin rim of
sclerotic bone that is sharply demarcated from normal
medullary cavity
Lesions often cross physis into metaphysis
Stippled calcifications within the lesion may or may not be
present (25%-45%)
Cortical expansion may be present

Chondroblastoma

Chondroblastoma (a) X-ray shows a cyst-like lesion occupying the epiphysis,

and sometimes extending across the physis into the adjacent bone

Chondroblastoma
Operative
extended intralesional curettage and bone grafting
indications
standard of treatment in symptomatic individuals

technique
may do local adjuvant treatment with phenol or cryotherapy

Resection of rare benign pulmonary metastasis

indications
if pulmonary metastasis

OSTEOCHONDROMA (CARTILAGE-CAPPED EXOSTOSIS)

A benign chondrogenic lesion derived from aberrant cartilage from
the perichondral ring that may take the form of
Solitary ostoechondroma
Multiple Hereditary Exostosis (MHE)
The most common benign bone tumor
Common in adolescents and young adults (tested ages: 9, 10, 12, 20, 24)
Location
Occur on the surface of the bone and often at sites of tendon insertion
Common locations include

knee (proximal tibia, distal femur)

proximal femur
proximal humerus
subungal exostosis (occurs most often at hallux)

OSTEOCHONDROMA (CARTILAGE-CAPPED
EXOSTOSIS)

OSTEOCHONDROMA
Pathophysiology
Solitary osteochondromas can arise because of
Salter-Harris fracture
Surgery
Radiation therapy (commonest benign radiation-induced bone
tumor)

Pathoanatomy
Hamartomatous proliferation of bone and cartilage
Possibly arise from growth plate cartilage that grows through the
cortex by endochondral ossification under the periosteum
Stalk of the lesion is cortical and cancellous bone formed from
ossified cartilage

OSTEOCHONDROMA
Associated conditions
secondary chondrosarcoma
A malignant condition that results from malignant transformation of a
solitary osteochondroma or MHE
- Occurs in older patients (tested ages: 50)
Most common location of secondary chondrosarcoma is the pelvis (usually occur as
low-grade chondrosarcomas)

Prognosis
Risk of malignant transformation is
<1% with solitary osteochondroma
~5-10% with MHE develop secondary chondrosarcoma

Multiple Hereditary Exostosis (MHE)

Disorder characterized by multiple osteochondromas
Other name Multiple Diaphysial Aclasis
Pathophysiology
Mutations affect the prehypertrophic chondrocytes of the physis
Autosomal dominant

Prognosis
5%-10% malignant transformation to chondrosarcoma in
patients with MHE
Proximal lesions more likely to undergo malignant
transformation than distal lesions

Osteochondroma
Symptoms
Most lesions are asymptomatic
Usually present with painless mass
May have mechanical symptoms or symptoms of
neurovascular compression
They continue to grow until skeletal maturity

Physical exam
Palpable mass
may have mechanical symptoms secondary to mass

Multiple hereditary exostosis (MHE)

Symptoms
Limb deformities
Most common sites of deformity include the knee, forearm, and ankle
Femoral shortening and limb-length discrepancy
Coxa valga
Knee valgus (because of shortened fibula) and patellar dislocation
Ankle valgus (because of shortened fibula)
Upper extremity deformities are well tolerated and lead to little loss of function
Ulnar shortening
Radial bowing and radial head dislocation
May be treated with exostosis excision, ulnar lengthening and radial closing wedge
osteotomy

Joint pain
may have symptoms of premature OA

Multiple hereditary exostosis (MHE)

Physical exam
Most common deformities include
Ulnar shortening and radial bowing
Radial head dislocation
Ulnar deviation of the hand

Secondary chondrosarcoma
acute onset of pain in adults with MHE should raise
suspicion for malignancy

Osteochondroma
Radiograph
Sessile (broad base) or pedunculated (narrow stalk) lesions found on
the surface of bones
higher risk of malignant degeneration in sessile lesions
pedunculated lesions point away from the joint

Continuity with native tissue

cortex of the lesion continuous with cortex of the native bone
medullary cavity of lesion continuous with medullary cavity of native bone

Cartilage cap is usually radiolucent and involutes at skeletal maturity

Nodules of metaplastic cartilage can occur within the bursa over cartilage caps

CT or MRI
used to better characterize lesions

Osteochondroma
Nonoperative
Observation alone
indications
asymptomatic or minimally symptomatic cases

Operative
Marginal resection at base of stalk, including cartilage cap
indications
symptomatic lesions
lesion may cause inflammation to surrounding tissue
lesion may be cosmetically displeasing
try to delay surgery until skeletal maturity

Multiple hereditary exostosis (MHE)

Nonoperative
observation
indications
most patients do not require intervention prior to reaching skeletal maturity

Operative
surgical excision of the osteochondroma
indications
dislocated radial heads
loss of forearm rotation
outcomes
simple excision of the osteochondroma optimizes chance of improved motion

Secondary chondrosarcoma
operative
wide surgical resection
treat same as typical chondrosarcoma

Complications
Pseudoaneurysm of the popliteal artery in the popliteal fossa
other vascular complications

vascular compression
true aneurysm
arterial thrombosis
venous thrombosis

Nerve compression
sciatic nerve
common peroneal nerve
atrophy of anterior and lateral compartment muscles of the leg

radial nerve

Complications
Tendon compression
lesions around the shoulder can give rise to
rotator cuff impingement
subscapularis tear
bicipital tendinitis

Chondrosarcoma
in adults, cartilage cap >2cm is associated with increased chance of
malignancy
mean age of diagnosis, 31yrs
seldom in 1st decade or after 5th decade of life

Bursa formation
Recurrence
2-5% of cases after resection

Osteochondroma (a) A young girl presented with this lump on her leg. It felt bony hard. (b) X-ray examination
showed the typical features of a large cartilage-capped exostosis; of course the cartilage cap does not show on x-ray unless
it is calcified. The bony part may be sessile, pedunculated or cauliflower-like.

Osteochondroma treatment (a) This 20-year-old man had known about the lump on his left scapula for many
years. He stopped growing at the age of 18 but the tumour continued to enlarge. (b) Despite the benign histology in the
biopsy, the tumour together with most of the scapula was removed; sections taken from the depths of the lesion showed
atypical cells suggestive of malignant change.

Unicameral Bone Cyst

Also referred to as a simple bone cyst
A non-neoplastic, serous fluid-filled bone lesion thought to result
from temporary failure of medullary bone formation near the physis
usually found in patients <20 years of age

location
usually found in the proximal humerus of young patients
can be found in other locations including proximal femur, distal tibia, ilium,
calcaneus, and occasionally metacarpals, phalanges, or distal radius
arises in the metaphysis adjacent to physis and progresses toward the diaphysis
with bone growth

Unicameral Bone Cyst

Prognosis
As a patient approaches skeletal maturity, a UBC will
often decrease in size and may heal after growth is
complete
Fracture healing usually does not lead to cyst resolution
Requires close follow up while in active phase due to
recurrence and risk of fracture or growth arrest

Unicameral Bone Cyst

Classification is important as it impacts
treatment
Active
if cyst is adjacent to the physis

Latent
if normal bone separates cyst from physis

Latent UBC

Unicameral Bone Cyst

Radiographs
Central, lytic, well-demarcated metaphyseal lesion
(2-3% cross physis)
Cystic expansion with symmetric thinning of
cortices
Trabeculated appearance after multiple fractures

Fallen leaf" sign (pathologic fracture with fallen cortical fragment in base of empty cyst is
pathognomonic )

Unicameral Bone Cyst

Nonoperative
Immobilization alone
indications
proximal humerus lesions with pathologic fracture (15% of lesions fill in with native bone
after acute fracture)

Aspiration/methylprednisolone acetate injection

indications
active cysts (communicates with physis) in the proximal humerus

technique
usually requires several injections, especially in very young children
bone marrow injections have recently been reported to be effective

Simple bone cysts

(a) A typical solitary (or unicameral) cyst on the shaft side of the physis and expanding
the cortex
(b) Injection with methylprednisolone, and (c) healing. (d) Fracture through a cyst.

Unicameral Bone Cyst

Operative
Curettage and bone grafting +/- internal fixation based on tumor
location
Indications
symptomatic latent cysts that have not responded to steroid injections
latent cysts in the proximal femur that are a structural concern and at risk for fracture
and osteonecrosis
proximal femoral lesions with a pathologic fracture have a high rate of refracture and
malunion when treated nonoperatively therefore, internal fixation is recommended

Contraindications
avoid in active lesions as communication with physis may lead to growth arrest

Aneurysmal Bone Cyst

A benign and non-neoplastic reactive bone lesion filled with
multiple blood-filled cavities
Can be locally destructive to normal bone and may extend to soft
tissue
75% of patients are < 20 yrs.

Location

25% in spine
20% in long bones (distal femur, proximal tibia)
Usually in metaphysis
Posterior elements of pelvis
May be found in similar location as telangiectatic osteosarcomas

Aneurysmal Bone Cyst

Radiographs
expansile, eccentric and lytic lesion with bony
septae ("bubbly appearance")
usually in metaphyseal

MRI or CT scan
will show multiple fluid lines

Aneurysmal Bone Cyst

Aneurysmal Bone Cyst

Nonoperative
Nonoperative fracture management
Indications
ABC with acute fracture
indicated until fracture has healed. Once healed, treat as an ABC without fracture unless the fracture has
led to spontaneous healing of the ABC

Operative
Aggressive curettage and bone grafting
Indications
symptomatic ABC without acute fracture

Technique
some use adjuvant treatment (phenol)

Outcomes
local recurrence in up to 25% and more common in children with open physes

Giant Cell Tumor

A benign aggressive tumor typically found in the epiphysis of long
bones
more common in females (unlike most bone tumors which show male
predominance)
ages 30-50 years

Location
50% occur around knee (distal femur or proximal tibia)
10% in sacrum and vertebrae (sacrum is most common site in axial skeleton)
while GCT can rarely occur in the spine, it usually occurs in the vertebral body

Distal radius is third most common location

Phalanges of the hand is also a very common location
May arise in the apophysis (like chondroblastoma)

Giant-cell tumours The tumour always abuts

against the joint margin

Giant Cell Tumor

Malignancy
Primary malignant giant cell tumor
metastatic to lung in 2-5%
hand lesions have greater chance of metastasis

Secondary malignant giant cell tumor

occurs following radiation or multiple resections of giant
cell tumor

Eccentric lytic epiphyseal/metaphyseal lesion that often extends

into the distal epiphysis and borders subchondral bone

Giant Cell Tumor

Nonoperative
radiation alone
indications
only indicated for inoperable or multiply recurrent lesions

outcomes
leads to 15% malignant transformation

Medical management
indications
medical therapy can be used to augment or replace surgical management depending on the
specific clinical scenario
Bisphosphonates
osteclast inhibitors which may decrease the size of the defect in giant cell tumors
Denosumab
monoclonal antibody against RANK-ligand
recent clinical trials suggest denosumab can decrease the size of the bone defect in giant
cell tumor

Giant Cell Tumor

Operative
Extensive curettage and reconstruction (with adjuvant
treatment)
Indications
lesions amenable to currettage
hand lesion treatment is controversial
if no cortical breakthrough treat with curettage and cementing
if significant cortical breakthrough consider intercalary
resection (with free fibular graft) vs. amputation

Giant Cell Tumor

Technique
challenge of treatment is to remove lesion while preserving joint and
providing support to subchondral joint
extensive exterioration (removal of a large cortical window over the
lesion) is required
can fill lesion with bone cement or autograft/allograft bone

outcomes
10-30% recurrence with curettage alone verses 3% with adjuvant
treatment (phenol, hydrogen peroxide, argon beam, etc)

Amputation
indications
hand lesions with cortical breakthrough who are not amendable
to intercalary resection

PRIMARY MALIGNANT BONE

TUMORS

Chondrosarcoma
Malignant chondrogenic lesions can occur in two forms
Primary chondrosarcoma
which includes
Low-grade, high-grade, de-differenitated chondrosarcoma
Clear cell chondrosarcoma
Mesenchymal chondrosarcoma

Secondary chondrosarcoma
arises from benign cartilage lesions including

Osteochondroma (<1% risk of malignant transfomation)

Multiple hereditary exostosis (1-10% risk of malignant transformation)
Enchondromas (1% risk of malignant transformation)
Ollier's disease (25-40% risk of malignant transformation)
Maffucci's (100% risk of malignant transformation)

Chondrosarcoma At the age of 20 years, this young man complained of pain in the right
groin; x-ray showed an osteochondroma of the right inferior pubic ramus.
(a) A biopsy showed benign cartilage but a year later the tumour had
doubled its size (b), a clear sign that it was malignant

Chondrosarcoma
Age & location
Found in older patients (40-75 yrs)
There is a slight male predominance
Most common locations include the pelvis, proximal
femur, scapula
Tumor location is important for diagnosis as the
same histology may be diagnosed as benign in the
hand but malignant if located in the long bones

Chondrosarcoma
Grade
85% of chondrosarcomas are grade 1 or 2
15% of chondrosarcomas are grade 3 or dedifferentiated chondrosarcoma
de-differentiated chondrosarcomas are high grade lesions which develop from low
grade chondroid lesions

Prognosis
axial and proximal extremity lesions have a more aggressive course
histologic grade correlates with survival

Grade I: 90% survival

Grade II: 60-70% survival
Grade III: 30-50% survival
De-differentiated chondrosarcoma: 10% survival

Chondrosarcoma
Chondrosarcoma sub-types
Clear cell chondrosarcoma
malignant immature cartilaginous tumor accounting
for <2% of all chondrosarcomas
most common in 3rd and 4th decades of life
presents as an epiphyseal lesion and can be
mistaken for low-grade chondroblastoma
locally destructive with potential to metastasize

Chondrosarcoma
Mesenchymal chondrosarcoma
May occur at several discontinuous sites at
presentation and can occur in the soft tissues
Treatment includes neo-adjuvant chemotherapy
followed by wide surgical resection

Chondrosarcoma
Radiographs
Lytic or blastic lesion with reactive thickening of
the cortex
Low-grade chondrosarcomas show
Similar appearance to enchondromas with additional
cortical thickening/expansion and endosteal erosion

High-grade chondrosarcomas show

cortical destruction and a soft tissue mass

Low-grade chondrosarcomas

Chondrosarcoma

Intra-lesional "popcorn mineralization may

be seen

de-differentiated chondrosarcomas radiographically show a lower

grade chondroid lesion with superimposed highly destructive
area consistent with the high grade transformed dedifferentiated
chondrosarcoma

Chondrosarcoma
Operative
intra-lesional curettage
indications
Grade 1 lesions
treatment of grade 1 lesions located in the pelvis or axial
skeleton is controversial
many authors recommend wide excision of all
chondrosarcomas (even grade 1) if located in the pelvis

Chondrosarcoma
Wide surgical excision
indications
grade 2 or 3 lesions
some say grade 1 lesions in pelvis

historically, there is no significant role for radiation or chemotherapy

in typical intramedullary chondrosarcoma

Wide surgical excision combined with multi-agent

chemotherapy
indications
mesenchymal chondrosarcoma
the role of chemotherapy in de-differentiated chondrosarcoma is very
controversial

Intramedullary Osteosarcoma
Intramedullary osteosarcoma is the most common primary sarcoma
of bone
The most common malignancy of bone is metastatic disease
The most common primary malignancy of bone is myeloma
Usually occurs in children and young adults
bimodal distribution of occurrence
majority occur in the second decade of life
second peak in occurrence is in elderly patients with Paget's disease

Most common site is the distal femur and proximal tibia

Other common sites include proximal humerus, proximal femur, and pelvis

Intramedullary Osteosarcoma
Malignancy
Most commonly diagnosed as Stage IIB (high grade, extracompartmental, no metastases)
10-20% of patients present with pulmonary metastases (obtain CT of
chest)
Lung is most common site of metastasis
Bone is second most common site

Genetics
Patients who carry the Retinoblastoma tumor suppressor gene (Rb)
are predisposed to osteosarcoma

Intramedullary Osteosarcoma
Prognosis
76% long-term survival with modern treatment
Poor prognostic factors include
Advanced stage of disease (most predictive of survival)
Response to chemotherapy (as judged by percent tumor necrosis of resected
specimen)
Tumor site and size
Expression of P-glycoprotein
High serum alkaline phosphatase
High lactic dehydrogenase
Vascular involvement
Surgical margins
Type of chemotherapy regimen

Characteristic blastic and destructive lesion

Sun-burst or hair on end pattern of matrix mineralization

(Codman's Triangle)

MRI must include entire involved bone to determine soft tissue involvement neurovascular involvement - presence skip metastases

Osteosarcoma pathology (a) After resection this lesion was cut in half; pale tumour
tissue is seen occupying the distal third of the femur and extending through the cortex.

Intramedullary Osteosarcoma
A 13-year-old girl presents with knee pain for 2 months especially
at night. She denies fevers and weight loss. Her physical exam
reveals a painful thigh mass. A radiograph is shown in Figure A.
What is the next most appropriate step in managment?
1.
2.
3.
4.

Repeat radiographs in 3 months with observation

External beam radiation and chemotherapy
Surgical biopsy and culture directed intravenous antibiotics
Neoadjuvant chemotherapy followed by wide excision and
adjuvant chemotherapy
5. MRI of the entire bone, whole body bone scan and CT chest

Intramedullary Osteosarcoma

Intramedullary Osteosarcoma
Operative
Multi-agent chemotherapy and limb salvage resection
Indications
high grade osteosarcoma

Chemotherapy
preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 612 months after surgical resection
98% necrosis after neo-adjuvant chemotherapy is good prognostic sign
expression of multi-drug resistance (MDR) gene portends poor prognosis
tumor cells can pump chemotherapy out of cell with MDR expression
present in 25% of primary lesions and 50% of metastatic lesions

Intramedullary Osteosarcoma
Surgical technique
Trend towards limb salvage whenever possible
Overall survival in osteosarcoma is equal after limb
salvage vs. amputation to deal with local extent of
disease

Wide surgical resection

indications
indicated in low grade osteosarcoma such as parosteal
osteosarcoma

Osteosarcoma operative treatment Postoperative x-rays showing an

endoprosthetic replacement following wide resection of the lesion (Stanmore
Implants Worldwide).

Parosteal Osteosarcoma
A low grade osteosarcoma
More common in females, age 30-40

Location
occurs on surface of metaphysis of long bones
most common sites include posterior distal femur, proximal tibia, and proximal humerus
80% cases occur in the femur

marrow invasion occurs in 25% of cases

Prognosis
95% long term survival when local control has been achieved
dedifferentiation of parosteal osteosarcoma is a poor prognostic factor
invasion into the medullary cavity does not affect long-term survival

Heavily ossified, lobulated mass arising from cortex

appears stuck onto cortex

Parosteal osteosarcoma (a,b) X-rays show an ill-defined

extraosseous tumour note the linear gap between cortex and tumour.

Parosteal Osteosarcoma
Operative
Wide local surgical excision
Indications
standard of care in most patients

Technique
many consider geometric osteotomy of involved bone to decrease
long term morbidity and retain native joint

Chemotherapy
chemotherapy not indicated unless there is a high grade component

Outcomes
often curative

Ewing's Sarcoma
A distinctive small round cell sarcoma
typically found in patients from 5-25 years of age
second most common bone tumor in children
uncommon in African Americans and Chinese

locations
~50% are found in the diaphysis of long bones
the most common locations include pelvis, distal femur, proximal
tibia, femoral diaphysis, and proximal humerus

Genetics
t(11:22) translocation found in all cases

Ewing's Sarcoma
Prognosis
Survival
60-70% long term survival with isolated extremity disease at presentation and
appropriate treatment/tumor response to chemotherapy
40% long term survival with pelvis lesions
15% long term survival if patient presents with metastatic disease

Poor prognostic factors

spine and pelvic tumors

tumors greater than 100cm3
< 90% necrosis with chemotherapy
elevated lactic dehydrogenase levels
p53 mutation in addition to t(11:22) translocation

Ewing's Sarcoma
Presentation
pain often accompanied by fever
often mimics an infection

Physical exam
swelling and local tenderness

Large destructive lesion in the diaphysis or

metaphysis with a moth-eaten appearance

Periosteal reaction may give "onion

skin" or "sunburst" appearance

Ewings tumour Examples of Ewings tumour in

(a) the humerus, (b) the mid-shaft of the fibula and (c) the lower end of the fibula.

Ewing's Sarcoma
Labs
ESR is elevated
WBC is elevated
anemia is common
lactic dehydrogenase

Bone marrow biopsy

required as part of workup for Ewing's to rule out
metastasis to the marrow

Ewing's Sarcoma
Operative
chemotherapy and limb salvage resection
indications
standard of care in most patients

chemotherapy
preoperative chemotherapy given for 8-12 weeks followed by surgical resection and
maintenance chemotherapy for 6-12 months

irradiation
current trend is towards surgical resection and away from irradiation due to long
term morbidity associated with radiation
situations where radiation may be used
non-resectable tumors (eg. large spinal tumors)
patients who present with widely metastatic disease

Ewing's Sarcoma
Small-round-cell tumor differential (by age)
< 5 yrs: neuroblastoma or leukemia
5-10 yrs: eosinophilic granuloma
5-30 yrs: Ewing's sarcoma
>30 yrs: lymphoma
> 50 yrs: myeloma

Multiple Myeloma
A neoplastic proliferation of plasma cells that presents with
skeletal lesions
neoplastic plasma cells produces immunoglobulins
heavy chains: IgG (52%), IgA (21%), IgM (12%)
light chains: kappa or lambda
aka Bence Jones proteins

Disease forms
disease takes multiple forms that vary in treatment and prognosis and
includes
multiple myeloma
solitary plasmacytoma
osteosclerotic myeloma

Multiple Myeloma

Bone scans are cold in 30% so obtain a skeletal

survey

Myeloma The characteristic x-ray features are bone rarefaction, vertebral

compression fractures, expanding lesions (typically in the ribs and pelvis) and
punched-out areas in the skull and the long bones.

Multiple Myeloma
Serum labs
anemia
elevated creatinine
hypercalcemia
present in 30% of patients due to excessive resorption of bone

ESR often elevated

SPEP (serum protein electrophoresis)
M spike present (50% IgG, 25% IgA)

Urine
proteinuria
UPEP (urine protein electrophoresis)
may show Bence Jones proteins (secreted immunoglobulin kappa and lambda light
chains)

METASTATIC BONE DISEASE

Metastatic Cancer of Bone

Metastatic cancer is the most common reason
for a destructive bone lesion in adults
carcinomas that commonly spread to bone include

breast
lung
thyroid
Renal
Adrenal
Prostate

Metastatic Cancer of Bone

Bone is the third most common site for metastatic
disease (behind lung and liver)
Common sites of metastatic lesions include
Axial skeleton (vertebral bodies, pelvis, ribs)
thoracic spine is most common site of bony metastasis
Proximal limb girdle
proximal femur is most common site of fracture secondary
to metastatic bone lesions

Metastatic Cancer of Bone

Prognosis
Median survival in patients with metastatic bone
disease
Thyroid: 48 months
Prostate: 40 months
Breast: 24 months
Kidney: variable depending on medical condition but may be
as short as 6 months
Lung: 6 months

Metastatic Cancer of Bone

Vascular spread :
Batson's vertebral plexus
valveless venous plexus of the spine that provides a route
of metastasis from organs to axial structure
including vertebral bodies, pelvis, skull, and proximal limb
girdles

Arterial tree metastasis

mechanism by which lung and renal cancer spread to the
distal extremities

Metastatic Cancer of Bone

Purly lytic or mixed lytic/blastic lesions
lung, thyroid, and renal are primarily lytic
60% of breast CA is blastic
90% of prostate CA is blastic

Cortical metastasis are common in lung

cancer

Lesions distal to elbow and knee are usually

from lung or renal primary

Metastatic Cancer of Bone

Goal of treatment in metastatic disease is pain control and
maintainence of patient independence
Bisphosphonate therapy
Stabilization of complete fracture, postoperative radiation
Prophylactic stabilization of impending fracture, postoperative
radiation
Preoperative embolization Renal / Thyroid

Synovial Chondromatosis
A proliferative disease of the synovium
associated with cartilage metaplasia
results in multiple intra-articular loose bodies
ranges from synovial tissue to firm nodules of
cartilage
usually affects young adults 30-50 years of age
knee is most common location

Synovial Chondromatosis
Apple Core Appearance

Pigmented Villonodular Synovitis

PVNS is an idiopathic monoarticular reactive synovial disease
characterized by exuberant proliferation of synovial villi and nodules
most commonly in adults age 30-50 but can occur at any age

location
may be localized (intra-articular or classic form)
knee is the most common site of involvement (80%)
other involved sites include hip, shoulder, and ankle

can be diffuse (extra-articular extension)

when extra-articular known as giant cell tumor of tendon sheath
occurring along tendon sheaths of hands and feet

Pathoanatomy and etiology

half of patients report prior history of trauma to afflicted region
thought to be a reactive process

Pigmented Villonodular Synovitis

History
50% of patients will have a prior history of trauma to
the area

Symptoms
Pain and swelling
Mechanical pain and limited motion
Recurrent atraumatic hemarthrosis is hallmark of
disorder

Pigmented Villonodular Synovitis

Arthrocentesis
grossly bloody effusion
Arthroscopy (gross appearance)
brownish or reddish inflamed synovium is typical of PVNS

Pigmented Villonodular Synovitis

Operative
total synovectomy
intra-articular disease
techniques range from arthroscopic partial synovectomy to fully open
total synovectomy
dependent on extent and location of disease
frequent recurrence is common
mostly due to incomplete synovectomy
extra-articular
marginal excision is adequate for giant cell tumor of tendon sheath
recurrence (which is common) is treated with repeat excision

External beam irradiation

when combined with total synovectomy reduces rate of recurrence to 10-20%