7.

 Bone  Tumours  

Cell type Benign Malignant

Bone § Osteoid osteoma § Osteosarcoma
Cartilage § Enchondroma § Chondrosarcoma
§ Osteochondroma
Fibrous tissue § Fibroma § Fibrosarcoma
Marrow § Eosinophilic § Ewing’s sarcoma
granuloma § Myeloma
Vascular § Haemangioma § Angiosarcoma
Uncertain § Giant-cell tumour § Malignant giant-
cell tumour

FACTORS IN ASSESSMENT
1. Age of patient – Young (<30) vs Old (>30)
a. Malignancy commoner in adults (except osteosarcoma
and Ewing’s sarcoma in young)
b. ***NOTE: Osteosarcoma has bimodal distribution; occurs in elderly because of Paget’s disease
c. NOTE MCQ: child w cystic lesion at epiphysis of long bone, most likely diagnosis is osteoblastoma
2. Location in long bones
a. Epiphysis
i. Young: Chondroblastoma, infection
ii. Over 20: GCT
iii. Older: Geode
b. Metaphysis: Osteosarcoma (esp knee and proximal humerus), non-ossifying fibroma (cortex), SBC, ABC, GCT (proximal tibia, distal
radius, distal femur), osteochondroma, chondromyxoid fibroma, chondrosarcoma, infections
c. Diaphysis: Ewing’s sarcoma, fibrous dysplasia, osteoblastoma
d. Non-specific: enchondroma, osteoid osteoma, metastatic
3. Location of lesion in skeleton
a. Finger: Enchondroma
b. Calcaneum: GCT
c. Spine: ABC, GCT, Multiple myeloma, mets (note that disc space not eroded in spinal mets)
d. Pelvis: Mets
e. Flat bones: Chondrosarcoma, Ewing’s sarcoma
f. Skull: Multiple myeloma
g. Red marrow populated areas: multiple myeloma, metastatic
h. Cartilage cap of osteochondroma OR central medullary: chondrosarcoma
4. Morphology – Malignant looking vs Benign looking
a. Transition zone: Applies only to osteolytic lesions
i. Narrow zone: Benign
ii. Broad zone: Malignant
b. Periosteal reaction: Left to right à Solid, lamellated, spiculated (sunburst), Codman’s

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 c. Soft tissue mass
d. Cortical destruction
5. Single vs Multiple
a. Commonly mutiple: Metastasis, multiple myeloma, osteochondromatosis (multiple exostosis), enchondromas (Maffucci syndrome)

à NOTE DDx for tumours

• Chronic osteomyelitis
o Systemic features may have been suppressed by Abx
o Submit tissue for histological and bacteriological examination
o Ewings and osteomyelitis can be confused due to: swelling, diaphyseal location, periosteal bone formation, exploration reveals
liquefied material like pus
• Stress # (young adult w localized pain near a large jt)

DIAGNOSIS OF MSK TUMOURS

• Imaging
o Plain XR
§ Description:
1. Type of X-ray
2. Age (skeletally immature vs mature)
3. Solitary or multiple
4. LUCENCY (lytic) vs DENSITY (blastic)
5. Site (bone and area – metaphysis, diaphysis, epiphysis)
6. Features
a. Zone of transition: Well definted or ill-defined (ie. Narrow or widened zone of transition)
b. Periosteal reaction: Any periosteal new bone formation & extension of tumour into soft tissues (malignant!)
i. Note that periosteal hypertrophy may also mean infx, stress #, soft tissue bruising
c. Any cortical destruction or cortical thickening
7. NOTE: stipled calcification inside a vacant area is characteristic of cartilage tumours
o CT & MRI
§ Useful to assess the true extent of tumour and its relationship to surrounding structures
§ Assuming that there are no mets, e local extent of e tumour is e most impt factor in deciding how much tissue to remove
o Radionuclide scanning w technetium
§ Shows non-specific reactive changes in the bone
§ Useful to reveal site of small tumour (eg. osteoid osteoma) or the presence of skip lesions/ silent secondary deposits
• Biopsy: essential for diagnosis and planning of Rx
o Open Bx more reliable
o Site is selected so that it can be included in any subsequent ablative operation
o As little as possible of the tumour is exposed; block of tissue is removed (ideally in the boundary zone), so as to include normal
tissue, pseudocapsule and abnormal tissue
o Frozen section examined; several samples taken if necessary
o If bone removed, the raw area is covered w bone wax or methlmethacrylate cement
o Drains shld be avoided to minimize risk of tumour seeding
o For tumours that are almost certainly benign: excisional biopsy can be done (remove entire lesion)
o For cysts: careful curettage to remove representative tissue

CLASSIFICATION
Epiphysis Metaphysis Diaphysis
Young Benign Malignant Benign Malignant Benign Malignant
(<20) Chondroblastoma SBC (simple bone cyst) Osteosarcoma Osteoid osteoma Ewing’s
Infection Non-ossifying fibroma sarcoma
Enchondroma (arise at puberty,
presents ~30yo)
Osteochondroma
Osteoid osteoma
Adult (20- Benign Malignant Benign Malignant Benign Malignant
40) GCT ABC (aneurysmal bone cyst) Osteoid osteoma
GCT (to epiphysis)
Enchondroma
Osteoid osteoma (<30)
Middle/ Benign Malignant Benign Malignant Benign Malignant
elderly Geode Osteosarcoma (~50yo)
(>40) (degen cyst) Chondrosarcoma
Multiple myeloma (solitary
type/non-specific)
NOTE: consider 1)infections, 2)metastasis from primary sites esp in older age groups
• Head and neck: thyroid, NPC
• Thorax: breast, lung
• Abdomen: kidneys, GIT (except rectum), prostate, testes
*Midline & paired structures
*Most are osteolytic except prostatic CA (blastic) & breast (blastic & lytic
Malignant bone tumours by frequency: 1) metastatic 2) multiple myeloma 3) osteosarcoma 4) chondrosarcoma 5) ewing’s sarcoma

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TREATMENT

Intra-compartmental Extra-compartmental
Low grade Wide excision (w/o exposing tumour) Radical incision +/- bone graft or prosthetic replacement
High grade Radical incision and prosthetic replacement or
amputation +/- chemo to reduce risk of mets
1. Osteosarcoma: neoadjuvant chemotherapy given before surgical resection
2. Mets (ELDERLY)
a. Wheelchair and admit patient (avoid weight bearing) – even if not yet fractured; fracture risk
b. Possible differential infection

STAGING FOR MALIGNANT BONE TUMOUR

1. MSTS Staging
a. Stage IA: Low grade, intracompartmental
b. Stage IB: Low grade, extracompartmental
c. IIA: High grade, intracompartmental
d. IIB: High grade extracompartmental
e. III: Any grade, metastasis

DETAILS ON SOME TUMOURS

Multiple Myeloma
• Pathology
o Malignant B-cell lymphoproliferative disorder of marrow, with plasma cells predominating
o Effects on bone due to marrow cell proliferation and increased osteoclastic activity
• Clinical features
o Osteoporosis and discrete lytic lesions throughout skeleton
§ Pain: Bone pain, back ache
§ Pathological fracture
o Plasmacytoma: large colony of plasma cells forming solitary tumour
o Blood: Plasma protein abnormalities, Anemia, Increased blood viscosity
o Hypercalcemia: Due to bone resorption
o Renal Dysfunction
o Spinal cord/root compression: Due to vertebral collapse
• X-Ray
o Generalised osteoporosis
o Classic lesion of punched out defects with soft margins in skull, pelvis, proximal femur
o Crushed vertebra
o Solitary lytic tumour in large bone metaphysis
• Biochemistry
o Mild anemia
o High ESR
o Raised creatinine
o Hypercalcemia
o Bence-Jones protein in urine
o Plasmacytosis with typical myeloma cells
• Prognosis: Poor (median survival of 2-3 years)

Fibrous Dysplasia
• Pathology
o Developmental disorder in which trabecular bone is replaced by fibrous tissue containing flecks of osteoid and woven bone
• Clinical Features (mono or polyostotic)
o Pathological Fractures
o Deformities
o Albright’s syndrome: Café-au-lait spots, precocious sexual development in girls
o Malignant transformation: 5-10% in patients with polyostotic lesions
• X-Ray
o ‘Ground glass’ cystic lesions in metaphysis or shaft
o Shepherd’s crook deformity of proximal femur

Osteosarcoma risk factors PRIMARY: Paget's, Radiation, Infaction of bone, Male, Alcohol, poor diet, sedentary lifestyle [adults only]
Retinoblastoma, Li-Fraumeni syndrome, Young [10-20 yrs].
Osteosarcoma is the most common primary malignant tumor of bone.

Osteosarcoma: features PEARL HARBOR:

Paget's disease (10-20%)*, Early age (10-20 yrs), Around knee, Raised periosteum by expanding tumor: "sunburst pattern", Lace-like
architecture, Hyaline arteoriosclerosis, Alkaline phosphatase increased, Retinoblastoma*, Boys predominantly, Osteomyelitis DDx, Radiation*
· Sunburst pattern was Japanese Navy emblem during WWII.
*: Predisposing factors.

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Osteochondroma
Pathophysiology
• Lump that sticks out of bone, mainly cancellous bone with a covering of cortical bone and a cartilaginous cap
• Derived from small pieces of metaphyseal cartilage which were not remodelled during growth of bone and have become separated from the
main cartilaginous epiphyseal plate
• Continue to grow and ossify and produce a bony knob just above the epiphyseal line, sometimes have an adventitious bursa over their cap
• Usually single, may be congenital and multiple

On inspection, I note a ___ by ___ cm spherical lump over the medial aspect of the (left / right) knee. There are no overlying skin changes or
scars noted in the surrounding skin. The surface is smooth, edges are distinct and the lump feels bony hard (can be masked by soft, fluctuant bursa
overlying cap). It is non-tender and not warmth to touch. The overlying skin is not attached to the lump; it is attached to the deep structures and is
not freely mobile. (move joint and feel)

ROM of the knee joint (is / is not) limited by the lump

My diagnosis will be an exostosis.
I would like to ask the patient for the presence of similar lumps over other sites of the body.
Differentials: gouty tophi / enchondroma

Hereditary Multiple Exostosis (Diaphyseal Aclasis/ Osteochondromatosis)

• Pathology
o Unrestrained transverse growth of cartilaginous physis
o Only affects endochondral bones
o Autosomal dominant inheritance
• Clinical Features
o Hard lumps at end of long bones (ulna, fibula) and along apophyseal borders of scapula and pelvis
o Deformities: Ulnar deviation of wrist, bowing of radius, subluxation of radial head, valgus kness, valgus ankles, large lumps often
around knee
o Neurovascular compromised by pressure
o Malignant change: Cartilage cap of exostoses transform to chondrosarcoma (a sign is continued growth after normal growth period
of bone)
• X-Ray
o Broad and poorly modeled long bone metaphyses
o Pedunculated or sessile exostoses arising from cortices

SOLITARY EXOSTOSIS / OSTEOCHONDROMA MULTIPLE EXOSTOSES

• Present when large enough to cause symptoms à • Hereditary condition (AD) – family hx
teenage & early adult life • Widespread generalised abn of bone remodelling at
• p/w lump, disfiguring, interference with movement of joint + epiphyseal line (failure of growth plate remodelling)
tendons (limited ROM a/w clicks or jumps as tendons slip • All bones that ossify in cartilage can be affected with
over lump), overlying bursa can become enlarged and exception of spine & skull
History

inflamed • Sites: end of long bones, apophyseal borders of scapula

• Continues enlarging till end of normal growth period, • Long bones may be shorter than normal (slightly short child
subsequently, consider malignant change with bowing of forearms & valgus knees)
• Site: usually adjacent to epiphyseal line of bone, just on • Occasionally lumps become tender / cause trouble with
diaphyseal side, majority at lower end of femur and upper pressure on a tendon
end of tibia (fast growing ends of long bone + crest of ilium) • Grows only when child grows (subsequent enlargement:
malignant change to chondrosarcoma)
• X-ray: well defined bony protuberance emerging from • X-ray: broadening & lumpiness of metaphyses
metaphysis, smaller (cartilage don’t show up) / surrounded
by blotches of calcified material (if cartilage gets calcified)
Investigation

• Excise if symptomatic, recent á in size + pain (malignant • Remove if troublesome + start to grow after parent bone
change) stops growing
Management

• Risk of malignant change: esp with pelvic exostoses (may • Risk of malignant change: 6% for multiple lesions
go unnoticed for long periods even with enlargement); 1%
for solitary lesions

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