Tumors of

Musculoskeletal
Tutorial Ortopaedic Surgery

Dr. Yazid Achari SpB,SpOT,FICS

 Definition
Neoplasm : abnormal new cell formation,
through the rest of life
Hereditary mechanism of the cell change 
irreversible  no cell maturity
Neoplasm
- Primary
- Secondary (metastatic)
Benign neoplasm : reactive lesion,
hamartoma  progressive growth  better
prognosis
 Incidence
Of all tumors : 1,5 %
Bone tumors : Benign  65,8%
Malignant  34,2 %
Most common :
– Benign : osteoma (9,3 %), osteochondroma
(32,5 %), chondroma (9,8 %)
– Malignant : Osteosarcoma (48,8 %), GCT
(17,5 %), chondrosarcoma (10 %)
 Incidence of
Benign Primary Bone Tumors

Other Benign
Tumors; 18,4
Osteoma; 39,3
Chondroma; 9,8

Osteochondroma;
32,5
 Incidence of
Malignant Primary Bone Tumors

Other Malignant
Tumors; 23,7
Osteogenic
sarcoma; 48,8
Chondrosarcoma;
10

Giant Cell Tumors;

17,5
 Classification of Neoplasia
Based on :
Histogenesis : tissue of origin & cell type
Biologic behaviour : benign / malignant
Anatomic site
Degree of differentiation
Common primary bone tumors

CELL TYPE BENIGN MALIGNANT

Bone Osteoid osteoma Osteosarcoma

Cartilage Chondroma Chondrosarcoma

Osteochondroma

Fibrous Tissue Fibroma Fibrosarcoma

Marrow Haemangioma Angiosarcoma

Uncertain GCT Malignant GCT

 Clinical Presentation
History
Chief complaint : usually pain,
deep seated, dull
first at night
Soft tissue tumors : lump / mass
Pathological #
Seek medical adv when pain persist or
lump got bigger
Neurologic symptoms : pressure / stretching
peripheral nerve
Age : important clue,
certain age group related

AGE MALIGNANT BENIGN

Birth - 5 Years Leukemia Osteomyelitis
Metastatic neuroblastoma Osteofibrous dysplasia
Metastatic rhabdomyosarcoma
10 - 25 Years Osteosarcoma Eosinophilic granuloma
Ewing's Tumor Osteomyelitis
Leukemia Enchondroma
Fibrous dysplasia
40 - 80 Years Metastatic bone disease Hyperparathyroidism
Myeloma Paget's disease
Lymphoma Mastocytosis
Paget's sarcoma
Postradiation sarcoma
 Physical Examination

Examined :
 Mass : border, firm or soft, tenderness
pulsating, warm, rubor
 DD/ with infection or haematoma

Near joint : effusion, limited ROM

Spinal lesion : muscle spasm, painful scoliosis
Look for metastase : lymph nodes, thyroid glands
abdomen, prostate, breast
 Radiography

Plain X Ray  still most useful

Watt ( 1985 ) Guideline for Examination

Lesion multiple / solitary

Type of bone involved
Location in the bone
Margin : well or ill defined
Bony reaction
Calcification
 Radiography
Anatomic site : origin of cell
Borders
 Benign : well define, sclerotic reaction
 Malignant : ill define, wide transition, min sclerotic
Bone destruction
 geographic, moth eaten, permeative
Matrix calsification
Periosteal reaction
 malignancy : non continous, thin, multiple lamination
Radiography

Pictures : Osteolisis
Osteosclerosis
Periosteal elevation
 ie. Codmann’s Triangle
Onion skin
 Other modalities of Imaging

Tomografi
CT Scan
MRI
Bone scan
 Laboratory

Blood exam : to exclude other cause of lump

 infection
metabolic bone disease
brown tumor
Anemia, elevated ESR, elevated Alk Phospatase
 not spesific, help DD/ malignancy
Ab normal globulin fraction
Bence Jones protein in urine
Acid phosphatase serum elev in Prostate Ca
 Biopsy

Needed for diagnosis & treatment planning

Needle biopsy
 less accurate
Open biopsy
 small incision, on margin area
normal, pseudocapsule, abnormal tissue
Benign : excision biopsy
Cyst : curretage
Performed after imaging exam completed
 Staging & Grading Tumour
Staging : evaluation of the extent of the
tumor at the time of diagnosis
Grading : an evaluation of the degree of
differentiation
Useful for :
- developing evaluation strategies
- planning treatment
- predicting prognosis
 Staging Musculo-skeletal
Neoplasms (Enneking)
Classification based on:
1. Histological grade (G)
2. Site (T)
3. Metastases (M)
Enneking staging system was tested
retrospectively on 397 cases of bone &
soft tissue tumours
The system aids in assessing prognosis &
planning management
Surgical Stages
 Grade (assessment of biological
aggressiveness)
– G0 Histologically benign (well
differentiated and low cell to
matrix ratio)
– G1 Low grade malignant (few
mitoses, moderate differentiation
and local spread only); Have low
risk of metasases
– G2 High grade malignancy (frequent
mitoses, poorly differentiated); High
risk of metasases
LOW (G1) HIGH (G2)
Parosteal osteosarcoma classic osteosarcoma
low-grade medullary radiation osteosarcoma
osteosarcoma
Secondary Chondrosarcoma Paget's osteosarcoma
Fibrosarcoma Primary Chondrosarcoma
Giant Cell Tumour Malignant Fibrous
Myxoid Liposarcoma Histiocytoma
Clear cell tumour of tendon Pleomorphic Liposarcoma
sheath Neurofibrosarcoma
Chordoma (Shwannoma)
Adamantinoma Rhabdomyosarcoma
Synovioma
Features of aggressive tumours

Cellular atypia
Frequent mitoses
Extensive necrosis
Significant vascularity
Small amounts of immature matrix
Site (anatomic setting of the lesion)

– T0 Intracapsular
– T1 Intracompartmental (eg cortical
bone, joint capsule or fascia)
– T2 Extracompartmental (spreads
beyond 'fascial' plane without
longitudinal containment)
Intracompartmental Extracompartmental
intraosseous soft tissue extension
intra-articular deep fascial
Intrafascial extension
compartments Extrafascial
planes/spaces:
(neurovascular
containing spaces)
 Metastasis (nodal or
blood borne tumour spread)

– M0 No evidence of regional or distant

metastases
– M1 Regional or distant metastases
evident
Malignant Primary
Tumors of Bone
 OSTEOSARCOMA

Primary malignant tumour arising from

bone and producing bone
Highly malignant spindle sarcoma
variants depending on the appearance of
the prominent cell type (may look like a
fibrosarcoma, chondrosarcoma or
myxosarcoma)
 Incidence

Affects about 1/200,000 population

Accounts for 21% of malignant primary bone
tumours
Third most common malignancy in
adolescents, after leukaemia & lymphoma.
75% occur in the distal femur or around the
knee
90% are metaphyseal in long bones
10% present with macroscopic metastatic
disease
 Types

Intramedullary (classical or ordinary)

osteosarcoma
Surface osteosarcomas:
– Parosteal osteosarcoma
– Periosteal osteosarcoma
Secondary osteosarcomas:
– Paget's
– postradiation
Telangiectatic osteosarcoma
 Clinically

Pain which is constant and worse at night

Pathological fracture rare
May have a tender lump which may lack a
definite edge and may be attached to muscle
If vascular ,pulsate and warm
fracture following minimal trauma as in
secondary to osteosarcoma often -> late
diagnosis of the tumour
 Differential Diagnosis

Post traumatic callus or myositis ossificans

Stress fracture - pathology may look similar
Osteomyelitis or syphilis
Benign bone tumour may have a similar
appearance especially if X-Rayed early
Ewings
 Prognosis
Untreated -> 95% death in 2 years
10% have macro-metastases at
presentation; 90% have micro-metastases
Even with metastatic (Stage 3) disease 5
year survival now 30 - 40% (10-20% with
surgery alone)
100% of bilateral retinoblastomas ->
osteosarcoma (? genetic factors)
 Prognostic Factors

Age - adults do worse

Size of primary tumour (big is bad)
Type
Location (proximal worse than distal)
– Parosteal - tend to be more low grade tumours
? -> better prognosis
– Intra osseous (classical) osteosarcoma -> good
prognosis
Stage
 Prognostic Factors

Origin of tumour in pre-existing lesion -> worse

prognosis
Response to chemotherapy
80 - 90% 5 year disease free survival in good
response patients
60% 5 year disease free survival in patients
with poor response rates
more than 16 metastatic deposits -> poor
prognosis
Pathological fracture does not affect prognosis
 CHONDROSARCOMA
Primary malignant tumour whose cells produce
cartilage matrix
May arise de novo or secondarily to an existing
benign cartilaginous tumour( osteochondroma or
enchondroma )
17% of primary malignant bone tumours
 Third most common malignancy after myeloma
and osteosarcoma
 CHONDROSARCOMA

•Peak incidence 30 - 60 years

•Male : Female 2:1
•Sites:
Pelvis 30%
Femur 20%
Femoral head 10%
Ribs 10%
 CHONDROSARCOMA

Clinically

Most common malignant tumour of the

hands and face in middle aged patients
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
 CHONDROSARCOMA

Clinically

 Pain
 mass in cartilago
 late growthly
 prognosis is better from osteosarcoma
MULTIPLE MYELOMA (MM)
MM is a malignant tumor of plasma cells that
causes widespread osteolytic bone damage
Most common primary malignant tumor of bone
(~ 40%)
May affect any bone with haematopoietic red
marrow (spine, skull, ribs, sternum and pelvis)
Age 50-80 year
M:F = 2:1
 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.
 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) present in urine
(50%)

Solitary lesions - 60% 5 year

survival
Multiple lesions 5% , 5 year
survival
 MALIGNANT FIBROUS
HISTIOCYTOMA
5% of primary malignant bone tumours
Age more than 30 (often more than 50)
Male > Female
80% are primary bone tumour
20% occur secondary to a pre-existing condition
Cell of origin controversial-options- histiocytic cell;
fibroblasts; multipotent mesenchymal cell
Can produce the osteolitik lession that spread to soft
tissue
 MALIGNANT FIBROUS
HISTIOCYTOMA

Clinically
Present with pain, swelling
15% present with a pathological fracture
May arise in previously abnormal bone eg Pagets,
fibrous dysplasia, long standing osteomyelitis or
irradiated bone
40% occur around the knee
Metastasises to the lung, and other bones via the
blood
 MALIGNANT FIBROUS
HISTIOCYTOMA

X-Rays
Usually metaphyseal
around knee
bone often mottled or moth eaten with extension
into soft tissue
Osteolytic lesion may be surrounded by reactive
bone
Destructive appearance radiologically
 FIBROSARCOMA
25% have metastasized at presentation and rare
2% primary malignant bone tumors
Male = Female

X-Rays
Osteolytic lesion
Margins can range from well-defined to ragged and
moth-eaten
Periosteal reaction is seen with cortical destruction
Extension into the soft tissue is common
 FIBROSARCOMA

Differential Diagnosis:
Metastatic carcinoma
multiple myeloma
MFH
leiomyosarcoma
 EOSINOPHILIC GRANULOMA
Part of a spectrum of Langerhan's cell
histiocytosis, formerly known as histiocytosis X.
Sub-types:
Hand Schuller Christian disease
– occurs in children > 3 yrs
– traid of skull lesions, exophthalmos, & diabetes
insipidus
– a minority of patients will have wide spread
visceral involvement (liver, spleen, pituitary)
– cranial lesions are always present in this
disease
 EOSINOPHILIC GRANULOMA

Letterer-Siwe disease
– <3 years old
– liver, spleen, skin, CNS involvement
– Rapidly fatal
– look for recurrent bacteremia, diffuse
lymphadenopathy, & skin lesions
 EOSINOPHILIC GRANULOMA

1 - 5% benign bone tumours

Rare
80% are less than 10 years old (usually
4 - 7 years)
Male = Female
 EOSINOPHILIC GRANULOMA

Clinically
Can affect just about any bone but
skull (10%), femur and spine most
commonly
Metaphyseal or diaphyseal
 EWINGS TUMOUR
Described by James Ewing in 1921
as a diffuse endothelioma of bone It
is a peripheral primitive
neuroectodermal tumour

5% of all primary malignant bone

tumours
 EWINGS TUMOUR

Clinically

60% occur in long tubular bones (also

pelvis ribs and scapula,femur,tibia,ulna and
metatarsal)
Considered a systemic disease
Present with pain + limp. Pain is throbbing,
worse at night and often severe
Age : child , adolescent,youngest
Prognosis : bad
 EWINGS TUMOUR

Clinically

Patient may be ill and sometimes

pyrexial
May have a palpable lump, which is
tender with an ill defined edge
Pathological fracture is rare
30% have metastatic deposits at
presentation - Lung & Lymph Nodes most
common
 EWINGS TUMOUR

Serology
Anaemia
Increased ESR & WCC
Increased serum Alkaline
Phosphatase
X-Rays
"onion skin" periosteal reaction
 EWINGS TUMOUR

Differential diagnosis

–Chronic osteomyelitis
–Eosinophilic granuloma
 LYMPHOMA OF BONE
(NON-HODGKIN'S)

reticulum cell sarcoma

> 20% of patients with lymphoma have
secondary bone involvement
Femur,tibia,humerus,vertebrae and pelvis
in patients twenty years of age and older
40 -50% occur around the knee
Present with pain and swelling
Pathological fracture may occur cause by
osteolytic defect
 LYMPHOMA OF BONE
(NON-HODGKIN'S)

Differential Diagnosis:
Osteosarcoma
Ewing's sarcoma
Osteomyelitis
Metastatic Ca
 LYMPHOMA OF BONE
(NON-HODGKIN'S)

Pathology
Histologically sheets of poorly
differentiated cells with irregular nuclei
Hodgkins -> Reed-Sternberg cells
histologically
Prognosis
Lymphoma of bone has the best
prognosis of all primary malignant bone
tumors
44% 5 year survival
Pure Hodgkins disease or lymphocytic
disease -> worse prognosis
 GIANT CELL TUMOUR
( Osteoclastoma )

Growth at the cancellous

long bone in youngest
Proliferate at the ephypise
area after close ephypiseal
plate
 GIANT CELL TUMOUR
( Osteoclastoma )

Predilection :
distal of radius,proximal of
tibia,distal of femur and
proximal of humerus
Spread to articular cartilage
2/3 benign,1/6 local agresive,1/6
malignant
 GIANT CELL TUMOUR
( Osteoclastoma )

Clinical feature :
Haemorhagea arround the
skin lession
Pain and tenderness
Joint disfunction
 GIANT CELL TUMOUR
( Osteoclastoma )

Microscopic feature :
Appear the osteoclastoma
cell that content vascular
tissue of the stroma cell
and most found multi
nucleated giant cell
Benign Primary Tumors of Bone
 OSTEOCHONDROMA
(Ivory Exostosis)
Cartilage capped bony projection / exostosis
Commonest benign tumour of bone
(45%)
Developmental abnormality of the metaphyseal
area of any bone formed in cartilage
(endochondral ossification)
most become evident under 20 years
May be solitary or multiple (diaphyseal aclasis)
Tx/ Observ only - excision
 ENCHONDROMA
Benign tumour of cartilage originating within the medullary cavity
Periosteal form originates in periosteum & erodes into the cortex
Usually metaphyseal
75% Solitary
60% present as fractures
Present with pathological fracture, lump or as incidental finding
Cortex remains intact unless fracture
Ollier's disease – multiple enchondromatosis; 50% ->malignant
transformation
Mafucci's disease - associated with multiple haemangiomata and
associated with nearly 100% malignant change somewhere
Tx/ Curretage + bone graft
 Chondroblastoma
About 1% of benign bone tumours
Male : Female 2:1
the adult counterpart of chondroblastoma is
giant cell tumour
Present with ache of increasing severity
Usually affects proximal humerus, proximal tibia
or femur
Epiphyseal but may expand into metaphysis
Tx/ curretage + graft
 Chondromyxoid Fibroma
2% of benign bone tumours
Present with a chronic ache
Usually eccentric metaphyseal lesions
75% lower extremity and 50% tibia
Tx/ Curretage + bone graft
 Osteogenic
Osteoid osteoma
Benign osteoblastoma

CHARACTERISTICS
Recognise matrix as woven bone
(osteoid)
osteoid osteoma & osteoblastoma have
similar histology, but different clinical,
radiological & gross pathological findings
 Osteoid Osteoma
Small, benign, solitary painful lesion of bone seen mainly in
children and adolescents
Etiology - Unknown
Accounts for 10% of benign bone tumors
Pain is the commonest presentation
Pain often worse at night and relieved by aspirin
10% occur in the spine and may -> scoliosis
Other sites may -> joint effusion, LLD, synovitis
Runs a self limiting course but usually -> surgery for pain
relief
 Benign Osteoblastoma
Less than 1% of primary bone tumours
Peak age 10 - 35 years, 80% are less than 30
years old
40 - 50% are vertebral
Less intense pain than osteoid osteoma
Occurs in the spine (post elements), often
associated with scoliosis and may be
neurological signs
May occur in long bones or phalanges
Tx/ Excisi + bone graft
X Ray
Well demarcated
osteolytic lesion
Less reactive bone
than osteoid osteoma
May have aggressive
features
metaphyseal
enlarges bone
 Unknown Origin
Unicameral (Simple) Bone Cyst (UBC)
Aneurismal Bone Cyst (ABC)
Giant Cell Tumor
Fibrous Histiocytositoma
 UNICAMERAL (SIMPLE)
BONE CYST (UBC)
Benign lesion which occurs during growth
20% of benign bone lesions
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)
Asymptomatic
Usually presents as a pathological fracture (~ 65%)
Etiology : Unknown
Venous obstruction leading to a transudate of fluid
Fluid contains high levels of IL-1 & IL-6, which stimulate osteoclasts
 Aneurismal Bone Cyst (ABC)
Benign solitary, expansile and erosive lesion of bone
1% of benign bone lesions
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
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
Tx/ curretage + bone graft/cement
 Giant Cell Tumor (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)
Nearly always located at the very end of a long bone
(metaphyseal / epiphyseal)
Pathological fracture occurs in 10 - 15%
Neighbouring joint often irritated (effusion)
Pain, swelling
Tx/ excisi/resection+ graft
 Fibrogenic
Fibrous Cortical defect (Non-ossifying
Fibroma)
Fibrous Dysplasia
Desmoplastic fibroma
 Fibrous Cortical defect
(Non-ossifying Fibroma)
20% of benign bone tumours
Male more than Female
Usually an incidental finding in children
Most heal spontaneously
Larger ones may -> pathological fracture
(common presentation)
Tx/ - unnecessary
- curretage+bone graft
 FIBROUS DYSPLASIA
Normal medullary bone is replaced by variable amounts of
structurally weak fibrous & osseous tissue
Aetiology :? developmental hamartoma
5 - 20% benign bone lesions
Relatively common and usually monostotic
McCune - Albrights Syndrome
Polyostotic disease (unilateral usually)
Skin pigmentation
– cafe au lait spots with serrated borders (called "coast of Maine") that
tend to stop abruptly at the midline of the body
Precocious puberty (endocrinopathy)
usually presents earlier, may be unilateral or widespread, affecting
long bones, hands, feet & pelvis
Malignant transformation (fibrosarcoma) is about 5-10 %;
 Other origin
Notochordal vascular : hemangioma
Lipogenic : Lipoma
Neurogenic : Neurilemoma
HAEMANGIOMA OF BONE
Haemangiomas are benign lesions
characterised by vascular spaces lined with
endothelial cells
Common, with approximately 10% of autopsy
cases having vertebral haemangiomas
M:F 1:2
Age 30 - 50 years
 HAEMANGIOMA OF BONE
Presentation
Usually asymptomatic and solitary discovered on x-ray
or at post mortem
Vertebral haemangiomas may present chronic back
ache and can cause neurological symptoms if they
extend into the epidural space
May present as a pathological fracture
Long bones may over grow due to increased blood
supply
 Soft Tissue Tumors
Rarely malignant
Chief complaint : lump / mass
Benign
Malignant
Tumor like reactive reaction
Suggest malignacy if :
pain lump, rapid increase in size
poor demarcation & attachment
 Fibrous tissue
Malignant :
Malignant Fibrous histiocytoma
Fibrosarcoma
Clinical & radiological similar
Age 30 – 80 years
Clinical : painful mass, could reach > 10 cm
ill defined
X ray : usually normal
bone erosion / destruction  severe
 Fibrous tissue
Clinical :
MFH : spindel cells , histiocytes
in cartwheel formation
small fasicle , concentric around
vascular
Fibrosarcoma :fasicular growth patern, spindel
cells, ill defined, lack citoplasma
 Lipoma
Common benign tumor of mature fat
In subcutaneus, intramuscular or intermuscular
Mostly not painful
3 types :
1. Spindel cell lipomas
2. Pleomorphic lipoma
3. Angiolipoma ( painful when palpated )
 Liposarcoma
Sarcoma with differentiation toward fat cell
Rage : Low grade – High grade
Propensity to recurent & metastase
 Neurofibroma
Solitary or multiple
Histological : elongated cell bundles
wavy nuclei, dark stain
Neurofibromatosis
autosomal dominant,
café au lait sport
varrying degree of skeletal condition
( scoliosis, non ossifying fibroma, long bone bowing )
 Rhabdomyosarcoma
Malignant
Young people ( < 20 years )
Grow rapidly
Hsitology : spindel cell in paralel bundles,
multinucleated giant cell
racquet shaped cells
cross striation ( rhabdomyoblast )
Sensitive to multiagent chemo th/