1. JULY 2007 TUMOURS 1
BONE TUMOURS &
TUMOUR-LIKE CONDITIONS
Dr. Munshya
17/06/2021
2. TUMOURS 2
Learning Outcomes
By the end of this lecture you should be able to:
1. Classify bone tumours
2. Describe clinical, radiological and histological
features of benign & malignant tumours &
tumour-like conditions
3. Enumerate the common tumours in Zambia
4. Outline the principles of management of most
common tumours
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Introduction
Primary bone tumours are
generally rare
Most common bone tumour is a
secondary (metastatic) deposit
The most common malignant
primary bone tumour in Zambia is
Osteosarcoma
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HISTORY
Presenting complaint
Pain and / or swelling
Character / duration of
symptoms
(distinguish benign / malignant
clinically)
Past and family history
Loss of weight
Other
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AGE
Probability of OS in 12-25 yrs
Mets in > 50 yrs
Chondroblastoma vs GCT
10-20 yrs vs 17-30 yrs
vs
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LOCAL
EXAMINATION
Location ( epi , meta , diaphysis )
( solitary or multiple )
Tumor size
Consistency ( bone or soft tissue )
Fixed or mobile
Solitary or multiple
N/V status
Lymph nodes ?
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GRADING
G0= Histologically benign ( well differentiated and
low cell to matrix ratio )
G1= Low grade malignant (few mitoses, moderate
differentiation and local spread only )
G2= High grade malignant (frequent mitoses, poorly
differentiated, high risk for metastases )
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Features of
aggressive tumours
Cellular atypia
Frequent mitoses
Extensive necrosis
Significant vascularity
Small amounts of immature
matrix
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Enneking’s 5 Questions
1. Age?
2. Site?
3. What is the tumour doing to the bone?
4. What is the bone doing to the tumour?
5. Any complications?
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Osteoid Osteoma
Incidence
• Accounts for 10% of benign bone tumours
• Male : Female 2:1
• Peak age 5 - 25 years (85% in this range)
• Rare over 40 years
Location:
Any bone, rarely multifocal
tibia & femur in 50%
spine - posterior elements
Only occurs in bones formed by endochondral ossification
May affect any part of a bone but is usually intracortical
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Osteoid Osteoma
Clinical Presentation
Pain is the commonest presentation
Pain often worse at night and relieved by aspirin (more likely
NSAIDs)
10% occur in the spine and may -> scoliosis
Other sites may -> joint effusion, synovitis
Runs a self limiting course but usually -> surgery for pain relief
Pain usually decreases as the lesion matures lasting 18 - 30 months
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Osteoid Osteoma
X-Rays
Lytic nidus
surrounded by
sclerotic bone
(which may mask the
nidus)
Centre of nidus may
be calcified
CT or tomograms ->
diagnosis
Hot spot on bone
scan
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Osteoid Osteoma
Differential Diagnosis
• Brodie's abscess
• Osteoblastoma
• Stress fracture
Treatment
NSAIDs
relieves symptoms
may take 3-4 years for symptoms to resolve
Surgical:
Nidus excision -> no recurrence (need only intact rim of reactive bone around
the nidus to ensure complete excision)
Intraoperative localisation with:
• Bone scan
• Tetracycline (4mg tetracycline per kg qid 1-2 days pre operatively -> specimen excised
under UV light)
• CT
• X-Ray excised tissue -> contains nidus
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Osteochondroma
(Exostosis)
Cartilage capped bony projection /
exostosis
Commonest benign tumour of bone
Developmental abnormality of the
metaphyseal area of any bone formed
in cartilage (endochondral ossification)
Probably arise from aberrant cartilage
from the perichondral ring of the
growth plate
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Osteochondroma
(Exostosis)
Incidence
Accounts for 45% of benign bone tumours
12% of all bone tumours
most become evident under 20 years
May be solitary or multiple (diaphyseal aclasis)
Any bone developing by endochondral
ossification may be involved
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Osteochondroma
(Exostosis)
Present with lump or interference of
tendon function
50% are distal femur, upper tibia or
proximal humerus
May be sessile or pedunculated
Active growth during skeletal growth -
>become latent
Move towards the diaphysis with
growth and usually angle away from the
growth plate
During growth period bone scan -
>activity at the tip
Increased activity on bone scan
after maturity suggests malignant
change
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Osteochondroma
(Exostosis)
Radiology
x-ray hallmark is blending of
tumour into underlying metaphysis
flat, sessile lesion or a peduculated
(stalk like) process
pedunculated osteochondromas
tend to point away from the physis
look for a well defined metaphyseal
excrescence of bone with a mottled
density
Cartilaginous cap displays irregular
areas of calcification
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Osteochondroma
(Exostosis)
Pathology
Normal bone covered by a
cap of normal cartilage
Cartilage cap resembles
layers of the normal
growth plate
The cartilage is more
disorganized than normal
Binucleate chondrocytes in
lacunae
Covered with a thin layer
of periosteum
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Osteochondroma
(Exostosis)
Treatment
Nil required unless symptomatic (persistent
irritation (from bursitis or tendon) or neurovascular
compromise)
Extra capsular marginal excision
Including the cartilaginous cap & overlying
perichondrium
Deep bony base has minimal activity & may be
removed piecemeal
Recurrence = < 5%
Decreased risk of recurrence if excised after
maturity
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Giant Cell Tumour
(GCT)
Benign, usually solitary and locally aggressive
10% of benign bone lesions
Can undergo malignant transformation (5-10%)
Not seen until after the growth plate closes
Rarely metastasises (<1% to lungs)
Age 20 - 40 years
More common in females
Most commonly seen in the distal femur, proximal tibia and the distal radius
Nearly always located at the very end of a long bone (Subchondral)
Pathological fracture occurs in 10 - 15%
Neighbouring joint often irritated (effusion)
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Giant Cell Tumour
(GCT)
X-Rays
Usually well defined
lesion in the epiphysis
extending up to the
joint surface
(subchondral) without
marginal sclerosis,
cortex thinned and
sometimes ballooned
soap bubble appearance
Junction with normal
bone poorly defined
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Giant Cell Tumour
(GCT)
Pathology
Soft, friable tumour
Cut surface tan in colour, with
areas of necrosis and
haemorrhage
Numerous multinucleated giant
cells. The stromal cells are
homogenous mononuclear
round/ovoid with large nuclei
The nuclei of the stromal cells
are identical to the nuclei of the
giant cells, a feature which
distinguishes giant cell tumour
from other conditions containing
giant cells
Up to 50% have soft tissue
extension but does not indicate
malignancy
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Giant Cell Tumour
(GCT)
Treatment
Intralesional excision by "extended" curettage
Curettage alone has a high local recurrence rate (50%) and the
curettage is "extended" into the bone by a few millimetres by
either using a burr, liquid nitrogen or phenol
The resulting cavity can be filled with bone graft or cement
En-bloc resection is possible if the bone is expendable e.g. proximal
fibula, proximal radius
Amputation reserved for massive local recurrence, malignant change
or infection
Radiotherapy reserved rare cases of unresectable tumours because
of increased risk of secondary malignancy
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Osteosarcoma
Primary malignant tumour arising from bone and producing bone
Incidence
Male : Female 2:1
Bimodal age distribution: -
Peak 1: 10 - 20 years (age of rapid growth)
Peak 2 : 50 - 70 years (80% less than 30 and those more than 40 years usually
secondary to Pagets)
Most common primary bone tumour in adolescents,
75% occur in the distal femur or around the knee
25% occur in the humerus
90% are metaphyseal in long bones
10% present with macroscopic metastatic disease
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Osteosarcoma
Clinical Presentation
Pain which is constant and worse at
night
Pathological fracture is rare
May have a tender lump which may lack
a definite edge and may be attached
to muscle
If vascular may pulsate and feel warm
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Osteosarcoma
X-Rays
Variable with combination of
bone destruction and bone
formation
Sun ray spicules (Radial
ossification) and
Codmans triangle (lifting of
periosteum) may be evident
Cortical breach common
Adjacent soft tissue mass
Joint space rarely involved
25% Lytic
35% Sclerotic
40% Mixed
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Osteosarcoma
Pathology
Pleomorphic and
anaplastic cell population
with abundant fibrous
and chondroid matrix
Stroma of spindle cells
with numerous mitoses.
Most are high grade
aggressive tumours
usually about 10cm
diameter at diagnosis (~
10/12 growth)
50% -> osteoblastic
25% -> chondroid
25% -> fibroblastic
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Osteosarcoma
Consider osteosarcoma as a systemic disease therefore
remove the primary site by en bloc excision and treat
microscopic disease by chemotherapy
Chemotherapy T10 regimen (methotrexate, vincristine,
adriamycin)-> 60-75% survival
Chemotherapy continues for 12 months in 4/52 cycles
Radiotherapy -> palliation of local pain and to treat surgically
inaccessible lesions and painful metastatic deposits
radiotherapy may also be used pre-operatively to decrease
the size and vascularity of the tumour.
Surgery:
Wide resection / Amputation
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Chondrosarcoma
Primary malignant tumour whose
cells produce cartilage matrix
May arise de novo or secondarily
to an existing benign
cartilaginous tumour
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Chondrosarcoma
Incidence
17% of primary malignant
bone tumours
Peak incidence 30 - 60
years
Male : Female 2:1
Sites:
Pelvis 30%
Femur 20%
Femoral head 10%
Ribs 10%
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Chondrosarcoma
Clinical Presentation
Usually occurs in the metaphysis or
diaphysis
Presents with constant ache or
increased size of a pre-existing lump
Metastatic deposits are infrequent
and usually go to lung
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Chondrosarcoma
X-Rays
Variable appearance with 60 - 70% have calcification and
50% have sub periosteal new bone
May be a large cystic lesion with cortical destruction and
central calcification, endosteal scalloping and cortical
expansion
Chondrosarcoma can also be classified as:
Intramedullary, which generally arise from
enchondromas
Patients with Ollier's disease (multiple
enchondromatosis) or Maffucci's syndrome (multiple
enchondromas and hemangiomas) are at much higher risk
of chondrosarcoma than the normal population
Surface which arise from osteochondromas/ exostoses.
Malignant change in an osteochondroma: increased size,
fuzzy outline, cartilage cap more than 1cm thick, base
more than 6cm diameter
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Chondrosarcoma
Treatment
These tumours tend to metastasise late
therefore attempt wide local excision initially
Radiotherapy useful for the treatment of
surgically inaccessible sites however are
relatively resistant to chemotherapy and
radiotherapy
Prognosis
Dependant on grade:
Low grade -> 65 - 85% 5 year survival
High grade -> 15 - 25% 5 year survival
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Multiple Myeloma
(MM)
Malignant tumor of plasma cells that
causes widespread osteolytic bone
damage
May affect any bone with
haematopoietic red marrow (spine,
skull, ribs, sternum and pelvis)
Age 50-80 year
M:F 2:1
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Multiple Myeloma
(MM)
Presentation
Bone pain related to the deposits
Pathological fractures
Constitutional symptoms related to anaemia, thombocytopenia
and renal failure
Other symptoms may include cachexia, spinal cord compression
Amyloidosis in 20%
Bacterial infections are common because of a lack of normal
immunoglobulin production.
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Multiple Myeloma
(MM)
Investigations
FBC normochromic, normocytic anaemia
ESR raised ++ (Often >100mm/hour)
Hypercalcaemia (20-40%)
Monoclonal immunoglobulin found on serum
electrophoresis (90%)
Bence Jones proteins (light chain subunits of
immunoglobulin) prese
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Multiple Myeloma
(MM)
Radiology
The radiological appearance of multiple
myeloma is characterised by irregular lytic
defects of different sizes, often described as
"punched out" and have no periosteal reaction
Skeletal survey is the most sensitive
investigation as a bone scan can fail to have
increased uptake in 25%
MRI is useful for delineating spinal lesions
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Multiple Myeloma
(MM)
Histology
Biopsy reveals sheets of
densely packed plasma
cells
The degree of cytological
atypia of these cells has
no prognostic value
The osteolytic lesions are
caused by increased
osteoclastic resorption
that is stimulated by
cytokines released by the
plasma cells
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Multiple Myeloma
(MM)
Treatment
Radiotherapy
MM is sensitive to Radiotherapy, and reossification of tumour
defects may occur within several months
Radiotherapy is recommended for intractable bone pain, it can be
dramatically effective in relieving symptoms
Chemotherapy
Palliative only
Bisphosphonates useful in the treatment of hypercalcaemia.
Surgery
Prophylactic IM nails for femoral, humeral deposits
ORIF of other pathological fractures
15% may need spinal decompression due to deposits or fracture
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Multiple Myeloma
(MM)
Prognosis
Untreated, a patient with bony lesions will only survive an
average of 6-12 months with the cause of death usually by
infection or haemorrhage
Improved survival following chemotherapy
With treatment a survival time of 3-5 years is not uncommon
Solitary lesions - 60% 5 year survival
Multiple lesions - 5% 5 year survival
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Unicameral (Simple)
Bone Cyst (UBC)
Benign lesion which occurs during growth
20% of benign bone lesions
Age 5-15 years
Not found in adults
Sex male to female is 3:1
The most common location is the proximal humerus (67%) followed by
the proximal femur (15%)
UBC's may be found in unusual sites (e.g. calcaneum, pelvis) in patients
>17 years
Cysts may be Active or Latent: Active cysts are located near the
growth plate,
but they move further away as the child grows and become inactive
(latent)
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Unicameral (Simple)
Bone Cyst (UBC)
Presentation
Asymptomatic
Usually presents as a pathological
fracture (~ 65%)
Aetiology
Unknown
Venous obstruction leading to a
transudate of fluid
Fluid contains high levels of IL-1 &
IL-6, which stimulate osteoclasts
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Unicameral (Simple)
Bone Cyst (UBC)
Radiology
Well defined, central
osteolytic area with a
thin sclerotic margin
(‘Fallen Leaf’ appearance)
Metaphyseal in young -
moves towards diaphysis
with growth
It fills and slightly
expands the juxta
epiphyseal metaphysis
CT not helpful unless the
UBC is in the pelvis
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Unicameral (Simple)
Bone Cyst (UBC)
Pathology
Histologically UBC's are thin walled
cavities filled with blood tinged fluid.
The lining cells are cuboidal, but are
not an endothelium
There is endosteal osteoclastic
activity and periosteal new bone
formation
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Unicameral (Simple)
Bone Cyst (UBC)
Treatment
Treatment goal is to minimise fracture risk until the cyst heals
(but this can take years)
Steroid injection
1-3 percutaneous injections repeated at 2 monthly intervals
60-80% success rate
Curettage and bone graft - 50% recurrence rate and
possibility of damage to the growth plate
Bone marrow aspirate has recently been used
Intramedullary fixation / stabilization with flexible nails may
help stabilize the lesion and may also promote resolution
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Aneurysmal Bone
Cyst (ABC)
Benign solitary, expansile and erosive
lesion of bone
1% of benign bone lesions
Age most frequent in children (85%
cases <20 years old)
Sex female to male is 2:1
ABC's can be found in any bone in
the body
The most common location is the
metaphysis of the lower extremity
long bones, more so than the upper
extremity
The vertebral bodies or arches of
the spine may be involved
Approximately one-half of lesions in
flat bones occur in the pelvis
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Aneurysmal Bone
Cyst (ABC)
Presentation
Swelling, tenderness and pain
Occasionally there is limited range of motion
due to joint obstruction
Spinal lesions can cause neurological symptoms
secondary to cord compression
Pathological fractures are rare due to the
eccentric location of the lesion
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Aneurysmal Bone
Cyst (ABC)
Differential diagnosis
Depending on the location, the
differential includes
UBC,
chondromyxoid fibroma,
giant cell tumor,
osteoblastoma
highly malignant telangiectatic
osteosarcoma.
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Aneurysmal Bone
Cyst (ABC)
Radiographic features
On plain film, an ABC is normally placed
eccentrically in the metaphysis and
appears osteolytic
The periosteum is elevated and the
cortex is eroded to a thin margin
The expansile nature of the lesion is
often reflected by a "blow-out" or
"soap bubble" appearance
CT scan can help delineate lesions in the
pelvis or spine where plain film imaging
may be inadequate
CT scan can narrow the differential
diagnosis of ABC by demonstrating
multiple fluid-fluid levels within the
cystic spaces
MRI can also confirm the multiple fluid-
fluid levels
65. TUMOURS 65
Aneurysmal Bone
Cyst (ABC)
Pathology
Macroscopically, an ABC is like a blood filled sponge
with a thin periosteal membrane. Soft, fibrous walls
separate spaces filled with friable blood clot.
Microscopically, the ABC has cystic spaces filled
with blood. The fibrous septa have immature woven
bone trabeculae as well as macrophages filled with
haemosiderin, fibroblasts, capillaries and giant
cells.
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Aneurysmal Bone
Cyst (ABC)
Treatment
The treatment approach will vary depending of the location and
aggressiveness of the lesion
A slow growing, indolent ABC has been observed to regress
spontaneously
Most lesions can be treated with curettage and application of a high-
speed burr
Local recurrence rates vary widely,
Recurrence statistically related to young age and open growth
plates, and may be less likely following wide excision than following
curettage
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Fibrous Dysplasia
(FD)
Aetiology
? developmental hamartoma
Incidence
5 - 20% benign bone lesions
Relatively common and usually monostotic
Affects children and adolescents
Median age at onset 8 years
Male > Female ( Albrights - Female > Male)
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Fibrous Dysplasia
(FD)
Sites
Ribs commonest (40%)
Lower limbs more than upper limbs
Craniofacial -> skull deformity
Epiphyses usually spared
Polyostotic -> pain, fracture (85%), deformity and skin
pigmentation
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Fibrous Dysplasia
(FD)
X-Rays
Lucent lesion in
medullary space
Sclerotic margin with
no discernable matrix.
Ground glass
appearance typical
No periosteal reaction
Shepherds crook
deformity of proximal
femur caused by
repeated # through
proximal femur lesions
71. TUMOURS 71
Fibrous Dysplasia
(FD)
Pathology
Bone replaced by firm, whitish tissue of gritty
consistency
Vascular tumour with poorly orientated bone
trabeculae separated by fibrous tissue.
Bone is woven rather than lamellar
lack of osteoblastic rimming of trabeculae
73. TUMOURS 73
Fibrous Dysplasia
(FD)
Treatment
Monostotic -> curettage and grafting if
symptomatic
Polyostotic -> symptomatic treatment
May require osteotomy for deformity or
lengthening / shortening procedures
74. TUMOURS 74
Fibrous Dysplasia
Prognosis
Monostotic lesions cease activity at
puberty but may be reactivated by
pregnancy
Polyostotic - 85% -> pathological
fracture
malignant change occurs after
radiotherapy
75. TUMOURS 75
Summary
Primary bone tumours are relatively rare
Osteosarcoma is the Commonest malignant primary
bone tumour
Exostoses are the most common benign bone
tumours
Patients with bone tumours need a thorough work
up.