Download MBBS Orthopaedics PPT 12 Malignant Bone Tumors Lecture Notes

Malignant bone tumors

Classification (W.H.O.)

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? Bone-forming tumours
? Cartilage forming tumours
? Giant-cell tumour
? Marrow tumours
? Vascular tumours

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? Secondary malignant tumours of bone
Bone forming tumors

? Benign-

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Osteoma

Osteoid osteomaor osteoblastoma

? Intermediate - Aggressive osteoblastoma

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? Malignant ?

Osteosarcoma Central (Medullary) and Peripheral (Surface)

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Parosteal

Periosteal

High grade surface

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Cartilage forming tumors

Benign ?

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Malignant -

? Chondroma/Enchondroma

? Chondrosarcoma

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? Osteochondroma

? Differentiated chondrosarcoma

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? Chondroblastoma

? Juxtacortical chondrosarcoma

? Chondromyxoid fibroma

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? Mesenchymal chondrosarcoma
? Clear cell chondrosarcoma
Giant cell tumor

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? Osteoclastoma

Marrow tumors

? Ewing's sarcoma

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? Neuroectodermal tumour

? Malignant lymphoma of bone (Primary/secondary)

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? Myeloma
Vascular tumors

Benign

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? Haemangioma

? Lymphangioma

? Glomus tumour

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Intermediate

? Haemangio endothelioma

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? Haemangio pericytoma

Malignant

? Angiosarcoma

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? Malignant haemangio pericytoma

Secondary

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Metastasis to bone from
? Thyroid
? Breast
? Bronchus
? Kidney

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? Prostate
APPROACH TO DIAGNOSIS OF MALIGNANT

BONE TUMOR
Multi phased work up

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Steps
? History
? Local examination
? Laboratory test
? Radiological test

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? Histopathological examination

History

? Age- some tumors are very age specific. Eg- Ewing sarcoma 10-20yrs;

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osteosarcoma 15-25yrs and > 45yrs (bimodal); chondrosarcoma

>45yrs; multiple myeloma >50yrs.

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? Non specific

? Dull aching painful lump

? Pathological fracture

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? Sometimes as incidental finding

? H/o exposure to radiation and chemical carcinogens

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? History of any malignancy anywhere in body or treatment history for

any malignancy at present or past
Examination

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? Swelling- tenderness, location, shape, consistency, fixity to skin and

adjacent structure, mobility, skin over swelling , dilated or engorged

veins.

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? Joint range of movement limitation
? Sign of inflammation may be present
? Any other skin lesion anywhere else in the body
? Regional lymph node

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? Systemic examination to diagnose primary tumor in case of

metastasis

Laboratory

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? Hemogram

? Lactate dehydrogenase

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? Erythrocyte sedimentation rate

? Parathormone

? C- reactive protein

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? Urinary Bence Jones protein

? Serum calcium

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? Urinary 24hrs calcium

? Serum phosphorus

? Electrophoresis

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? Alkaline phosphatase

? Bone marrow examination
Hemogram and ESR

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? Anaemia may be seen
? To rule out infection, myeloma and leukemia.
? ESR is raised particularly in
Metastasis

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Ewing's sarcoma
Lymphoma
Leukemia

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S calcium and phosphorus

? Hypercalcemia is most common metabolic complication of metastatic

bone disease.

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? Increased level than normal indicates

Metastasis
Myeloma

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Hyperparathyroidism
S alkaline phosphatase

? Raised alkaline phosphatse indicates high turn over of the bone
? Raised in osteoblastic lesion

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Blastic metastasis from prostate and breast
Active Paget's disease
Hyperparathyroidism

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Radiological tests

? X-ray

? Chest X-ray

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? CT scan

? CT thorax, abdomen and pelvis

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? MRI

? Mammography

? Bone scan

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? Thyroid scan

? PET scan

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? Arteriogram
OSTEOSARCOMA

o Characterized by the production of osteoid by malignant cel s.

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o It is the second most common primary malignant tumor of bone, accounting for approximately 20% of

primary bone cancers.

o The most common nonhematologic primary malignancy of bone

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o Onset can occur at any age; however, primary high-grade osteosarcoma occurs most commonly in the second

decade of life.

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o Parosteal osteosarcoma has a peak incidence in the third and fourth decades.
o Arise from multipotent mesenchymal cel s
Clinical y

? Radiographic appearance of osteosarcoma can vary

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? lesion can be either predominantly blastic or predominantly lytic

? The lesion usually is quite permeative, and the borders are ill defined.

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? May take the form of a "Codman triangle," or it may have a "sunburst" or

"hair-on-end" appearance.

? Magnetic resonance imaging (MRI)

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? They may be primarily osteoblastic, fibroblastic, or chondroblastic

Plain X-ray (Most valuable)

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Lytic

sclerotic

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Mixed (most common)
Plain X-ray

22

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Lesions are usual y permeative

Associated with destruction of the cancel ous a n

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d

cortical elements of the bone

Ossification within the soft tissue component, if

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tumour has broken through cortex

Intra medullary
Borders are ill defined

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Plain X-ray

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Periosteal reaction may appear as the characteristic Codman triangle.

Classification:

PRIMARY OSTEOSARCOMAS are

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Conventional /classic osteosarcoma (high grade, intra medullary)
Low-grade intramedul ary osteosarcoma

Parosteal osteosarcoma

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Periosteal osteosarcoma

High-grade surface osteosarcoma

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Telangiectatic osteosarcoma, and

Small cell osteosarcoma.

Classification:

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SECONDARY OSTEOSARCOMAS

Osteosarcomas occurring at the site of another disease process.
more common in >50 years of age

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The most common causes are

Paget disease
Previous radiation treatment

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Other associated conditions are

Fibrous dysplasia Bone infarcts
Osteochondromas Chronic
osteomyelitis

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Dedifferentiated chondrosarcomas
Osteogenesis imperfecta
Conventional Osteosarcoma

? High grade

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? Common type of Osteosarcoma

? Common radiographic appearance is

aggressive lesion producing osteoid matrix

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? Periosteal reaction may take the form of

"codmans triangle" or "sunbrust" or "hair

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on end" appearence

Periosteal Osteosarcoma

? Intermediate grade

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? Arises from surface of bone

? Commonly on femur and tibia.
Intramedul ary Osteosarcoma

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q Rare
q Low grade

Parosteal Osteosarcoma

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? Low grade malignancy
? Rare
? Arises on surface of bone and invades

medulary cavity only at later stages.

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? It has peculiar tendency to occur as a

lobulated mass on the posterior aspect of
femur

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High grade surface Osteosarcoma

? High grade
? Least Common
? Radiographs show invasive lesions with

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ill defined borders

Telangectic Osteosarcoma

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Lytic lesion
Smal cell Osteosarcoma

? Rare
? High grade

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? Resemble Ewing sarcoma or Lymphoma

Treatment

Current standard of care

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27

Radiological staging

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Biopsy to confirm diagnosis Preoperative

chemotherapy

Repeat radiological staging (access chemo response, finalize surgical treatment plan)

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Surgical resection with wide margin

Reconstruction using one of many

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techniques

Post op chemo based on preop response
Chondrosarcoma

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? 9% of primary malignancies of bone
? Age: broad, primary chondrosarcoma peak around 40 ? 60yrs, secondary chondrosarcoma

25 ? 45 yrs.

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? Any location but common around pelvis, proximal femur, proximal humerus.
? Most common malignancy in hand.
? Clinical y: increasing pain and palpable mass.
? Pain in absence of pathological fracture is helpful to dif erentiate between enchondroma

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and low grade chondrosarcoma

Secondary Chondrosarcoma

? Olliers disease (multiple enchondromatosis)

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? Maffuci syndrome (multiple enchondromatosis + soft tissue haemangioma)
? Multiple hereditary exostoses
? Solitary osteochondroma
? Synovial chondromatosis

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? Chondromyxoid fibroma
? Periosteal Chondroma
? Chondroblastoma
? Previous radiation treatment Firbours dysplasia
Chondrosarcoma - xrays

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? Appearence similar to enchondroma,
it is a lesion arising in medulary cavity with irregular
matrix calcification.
? Pattern is described as "punctate," "popcorn," or

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"comma-shaped".

? Compared with enchondroma,
chondrosarcoma has more aggressive appearence

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with bone destruction and cortical erosions,
periosteal reaction, and rarely soft-tissue mass.
Mesenchymal chondrosarcoma
Clear cell chondrosarcoma

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Epiphyseal Gaint Cel Tumor

Chondroblastoma

Clear cel chondrosarcoma

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Dedifferentiated chondrosarcoma

Radiographic features of dedif erentiated chondrosarcoma often

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show a more aggressive radiolucent area juxtaposed on a

otherwise typical chondrosarcoma.
POP CORN CALCIFICATION

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DIFFERENCE FROM ENCHONDROMA

o Endosteal scal oping of more than 2/3rd of cortical thickness- chondrosarcoma

o Aggressive changes such as cortical erosion , bone destruction, periosteal reaction, soft

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tissue mass- chondrosarcoma

o size> 5 cm in axial skeleton- predictor of malignancy

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o Apperance of lysis in previously calcified area- malignancy
Intramedullary ,soft

tiisue extention of tumour

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with calcification

Chondrosarcomas Treatment

? Low grade ? Extended curettage with use of intraoperative adjuvant

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treatment.

? High grade ? Wide or radical resection or amputation.

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? Radiotherapy as palliative for inaccessible lesions.

Ewing's Sarcoma

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Ewing sarcoma, a highly malignant neoplasm

Third most common nonhematologic primary malignancy of bone

The second most common in patients younger than 30 years of age and the

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most common in patients younger than 10 years of age

Ewing's sarcoma

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Approximately 90% of Ewing sarcomas occur before age 25

Histogenesis: neurally derived small round cel malignancy very similar to the

so-called primitive neuroectodermal tumor (PNET)

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Disease is extremely rare in black persons
Areas of

involvement

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Clinical presentation

Present as a localized painful mass

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With systemic symptoms such as fever, malaise, weight loss, and

An increased erythrocyte sedimentation rate.

These systemic symptoms may lead to an erroneous diagnosis of osteomyelitis.

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Radiological features

lesion is poorly defined,

Marked by a permeative or moth-eaten type of bone destruction,

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Associated with an aggressive periosteal response that has an onionskin (or

"onion peel")

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less commonly, a "sunburst" appearance,

Large soft tissue mass, Occasionally, the bone lesion itself is almost

imperceptible, with the soft-tissue mass being the only prominent radiographic

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finding

Radiological

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Features

Onion skin appearance

MRI

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1. T1 : low to

intermediate signal

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2. T1 C+ (Gd) :

heterogeneous but

prominent

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enhancement

3. T2 : heterogeneously

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high signal, may see

hair on end low signal

striations

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Nuclear medicine:

Ewing sarcomas demonstrate increased uptake on both

Gallium-citrate and

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Technetium99m methylene diphosphonate scans
Differential diagnosis

other Ewing sarcoma family of tumours

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pPNET : large soft tissue component with extension into bone

Askin tumour : chest wall

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osteosarcoma (ALP is not elevated in Ewing sarcoma)

Leukemia

Multiple myeloma

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Ostyeomyelitis
Ewing sarcoma ? treatment
? Radiosensitive

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? Large central unresectable mass ? radiotherapy.

? Smaler more accessible lesions surgery.

? Neo adjuvant and adjuvant chemotherapy

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