Malignant bone tumors

Classification (W.H.O.)

• Bone-forming tumours

• Cartilage forming tumours

• Giant-cell tumour

• Marrow tumours

• Vascular tumours

• Secondary malignant tumours of bone

Bone forming tumors
• Benign-
Osteoma
Osteoid osteomaor osteoblastoma
• Intermediate - Aggressive osteoblastoma
• Malignant –
Osteosarcoma Central (Medullary) and Peripheral (Surface)
Parosteal
Periosteal
High grade surface
Cartilage forming tumors
Benign – Malignant -
• Chondroma/Enchondroma • Chondrosarcoma
• Osteochondroma • Differentiated chondrosarcoma
• Chondroblastoma • Juxtacortical chondrosarcoma
• Chondromyxoid fibroma • Mesenchymal chondrosarcoma
• Clear cell chondrosarcoma
Giant cell tumor
• Osteoclastoma
Marrow tumors

• Ewing’s sarcoma

• Neuroectodermal tumour

• Malignant lymphoma of bone (Primary/secondary)

• Myeloma
 Vascular tumors
Benign
• Haemangioma
• Lymphangioma
• Glomus tumour
Intermediate
• Haemangio endothelioma
• Haemangio pericytoma
Malignant
• Angiosarcoma
• Malignant haemangio pericytoma
Secondary
Metastasis to bone from
• Thyroid
• Breast
• Bronchus
• Kidney
• Prostate
APPROACH TO DIAGNOSIS OF
MALIGNANT BONE TUMOR
Multi phased work up
Steps
• History
• Local examination
• Laboratory test
• Radiological test
• Histopathological examination
History
• Age- some tumors are very age specific. Eg- Ewing sarcoma 10-20yrs;
osteosarcoma 15-25yrs and > 45yrs (bimodal); chondrosarcoma >45yrs;
multiple myeloma >50yrs.
• Non specific
• Dull aching painful lump
• Pathological fracture
• Sometimes as incidental finding
• H/o exposure to radiation and chemical carcinogens
• History of any malignancy anywhere in body or treatment history for
any malignancy at present or past
Examination
• Swelling- tenderness, location, shape, consistency, fixity to skin and
adjacent structure, mobility, skin over swelling , dilated or engorged
veins.
• Joint range of movement limitation
• Sign of inflammation may be present
• Any other skin lesion anywhere else in the body
• Regional lymph node
• Systemic examination to diagnose primary tumor in case of
metastasis
Laboratory
• Hemogram • Lactate dehydrogenase
• Erythrocyte sedimentation rate • Parathormone
• C- reactive protein • Urinary Bence Jones protein
• Serum calcium • Urinary 24hrs calcium
• Serum phosphorus • Electrophoresis
• Alkaline phosphatase • Bone marrow examination
Hemogram and ESR
• Anaemia may be seen
• To rule out infection, myeloma and leukemia.
• ESR is raised particularly in
Metastasis
Ewing’s sarcoma
Lymphoma
Leukemia
S calcium and phosphorus
• Hypercalcemia is most common metabolic complication of metastatic
bone disease.
• Increased level than normal indicates
Metastasis
Myeloma
Hyperparathyroidism
S alkaline phosphatase
• Raised alkaline phosphatse indicates high turn over of the bone
• Raised in osteoblastic lesion
Blastic metastasis from prostate and breast
Active Paget’s disease
Hyperparathyroidism
Radiological tests
• X-ray • Chest X-ray
• CT scan • CT thorax, abdomen and pelvis
• MRI • Mammography
• Bone scan • Thyroid scan
• PET scan • Arteriogram
 OSTEOSARCOMA
o Characterized by the production of osteoid by malignant cells.

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

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

o Parosteal osteosarcoma has a peak incidence in the third and fourth decades.
o Arise from multipotent mesenchymal cells
 Clinicall
y
• Radiographic appearance of osteosarcoma can vary

• l esion can be either predominantly blastic or predominantly lytic

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

• May take the form of a “Codman triangle,” or it may have a “sunburst” or

“hair-on-end” appearance.

• Magnetic resonance imaging (MRI)

• They may be primarily osteoblastic, fibroblastic, or chondroblastic

 Plain X-ray (Most
valuable) 21

  sclerotic Mixed (most common)

Lytic
 Plain X-
ray 22
 Lesions are usually permeative

 Associated with destruction of the cancellous a n d

cortical elements of the bone

 Ossification within the soft tissue component, if

tumour has broken through cortex
 Intra medullary
 Borders are ill defined
 Plain X-
ray 23
 Periosteal reaction may appear as the characteristic Codman
triangle
.
 Classification:
 PRIMARY OSTEOSARCOMAS are
 Conventional /classic osteosarcoma (high grade,
intra medullary)
 Low-grade intramedullary osteosarcoma

 Parosteal osteosarcoma

 Periosteal osteosarcoma

 High-grade surface osteosarcoma

 Telangiectatic osteosarcoma, and

 Small cell osteosarcoma.

 Classificatio
n:
SECONDARY OSTEOSARCOMAS
Osteosarcomas occurring at the site of another disease process.
more common in >50 years of age
The most common causes are
Paget disease
Previous radiation treatment

Other associated conditions are

Fibrous dysplasia Bone
infarcts
Osteochondromas
Chronic osteomyelitis
Dedifferentiated chondrosarcomas
Osteogenesis imperfecta
Conventional
Osteosarcoma
• High grade
• Common type of Osteosarcoma
• Common radiographic appearance is
aggressive lesion producing osteoid
matrix
• Periosteal reaction may take the form of
“codmans triangle” or “sunbrust” or
“hair on end” appearence
Periosteal
Osteosarcoma
• Intermediate grade

• Arises from surface of bone

• Commonly on femur and tibia.

Intramedullary Osteosarcoma

 Rare
 Low grade
Parosteal Osteosarcoma
• Low grade malignancy
• Rare
• Arises on surface of bone and invades
medullary cavity only at later stages.
• It has peculiar tendency to occur as a
lobulated mass on the posterior aspect
of femur
High grade surface Osteosarcoma

• High grade
• Least Common
• Radiographs show invasive
lesions with ill defined
borders
Telangectic Osteosarcoma

Lytic lesion
Small cell Osteosarcoma
• Rare
• High grade
• Resemble Ewing sarcoma or Lymphoma
 Treatme
nt of care
 Current standard
27
Radiological staging

Biopsy to confirm

diagnosis Preoperative

chemotherapy

Repeat radiological staging (access chemo response,

finalize surgical treatment plan)

Surgical resection with wide margin

Reconstruction using one of many

techniques
Chondrosarcoma
• 9% of primary malignancies of bone

• Age: broad, primary chondrosarcoma peak around 40 – 60yrs, secondary

chondrosarcoma 25 – 45 yrs.
• Any location but common around pelvis, proximal femur, proximal humerus.

• Most common malignancy in hand.

• Clinically: increasing pain and palpable mass.

• Pain in absence of pathological fracture is helpful to differentiate between enchondroma

and low grade chondrosarcoma
Secondary Chondrosarcoma
• Olliers disease (multiple enchondromatosis)

• Maffuci syndrome (multiple enchondromatosis + soft tissue haemangioma)

• Multiple hereditary exostoses
• Solitary osteochondroma
• Synovial chondromatosis
• Chondromyxoid fibroma
• Periosteal Chondroma
• Chondroblastoma
• Previous radiation treatment Firbours dysplasia
 Chondrosarcoma -
xrays
• Appearence similar to enchondroma,
it is a lesion arising in medullary cavity with
irregular matrix calcification.
• Pattern is described as “punctate,” “popcorn,”
or “comma-shaped”.
• Compared with enchondroma,
chondrosarcoma has more aggressive appearence
with bone destruction and cortical erosions,
periosteal reaction, and rarely soft-tissue mass.
Mesenchymal
chondrosarcoma
 Clear cell
chondrosarcoma

Epiphyseal Gaint Cell Tumor Chondroblastoma Clear cell chondrosarcoma

 Dedifferentiated
chondrosarcoma

Radiographic features of dedifferentiated chondrosarcoma often

show a more aggressive radiolucent area juxtaposed on a
otherwise typical chondrosarcoma.
POP CORN CALCIFICATION
 DIFFERENCE FROM
ENCHONDROMA
o Endosteal scalloping of more than 2/3 r d of cortical thickness- chondrosarcoma

o Aggressive changes such as cortical erosion , bone destruction, periosteal reaction, soft
tissue mass- chondrosarcoma

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

o Apperance of lysis in previously calcified area- malignancy

Intramedullary ,soft tiisue
extention of tumour with
calcification
Chondrosarcomas Treatment
• Low grade – Extended curettage with use of intraoperative adjuvant
treatment.

• High grade – Wide or radical resection or amputation.

• Radiotherapy as palliative for inaccessible lesions.

 Ewing’s
Sarcoma
 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
most common in patients younger than 10 years of age
 Ewing’s
sarcoma
 Approximately 90% of Ewing sarcomas occur before age 25

 Histogenesis: neurally derived small round cell malignancy very similar to the
so-called primitive neuroectodermal tumor (PNET)

 Disease is extremely rare in black persons

 Areas
of
involvement
 Clinical
presentation
 Present as a localized painful mass

 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.
Radiological
features
 lesion is poorly defined,

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

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

“onion peel”)

 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
finding
Radiologic
al
Features
Onion skin
appearance
 MR
I
1. T1 : low to
intermediate signal
2.T1 C+ (Gd) :
heterogeneous but
prominent
enhancement
3. T2 : heterogeneously
high signal, may see
hair on end low signal
striations
Nuclear
medicine:

 Ewing sarcomas demonstrate increased uptake on both

Gallium-citrate and

 Technetium99m methylene diphosphonate scans

 Differential
diagnosis
 other Ewing sarcoma family of tumours
 pPNET : large soft tissue component with extension into bone
 Askin tumour : chest wall

osteosarcoma (ALP is not elevated in Ewing sarcoma)

 Leukemia

 Multiple myeloma

 Ostyeomyelitis
Ewing sarcoma – treatment
• Radiosensitive

• Large central unresectable mass – radiotherapy.

• Smaller more accessible lesions surgery.

• Neo adjuvant and adjuvant chemotherapy