? Cartilage forming tumours
? Giant-cell tumour
? Marrow tumours
? Vascular tumours
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? Secondary malignant tumours of boneBone forming tumors
? Benign-
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OsteomaOsteoid osteomaor osteoblastoma
? Intermediate - Aggressive osteoblastoma
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? Malignant ?
Osteosarcoma Central (Medullary) and Peripheral (Surface)
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ParostealPeriosteal
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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? OsteoclastomaMarrow tumors
? Ewing's sarcoma
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? Neuroectodermal tumour
? Malignant lymphoma of bone (Primary/secondary)
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? MyelomaVascular tumors
Benign
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? Haemangioma? Lymphangioma
? Glomus tumour
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Intermediate
? Haemangio endothelioma
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? Haemangio pericytomaMalignant
? 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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? ProstateAPPROACH 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 examinationHistory
? 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 forany malignancy at present or past
Examination
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? Swelling- tenderness, location, shape, consistency, fixity to skin andadjacent 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 ofmetastasis
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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HyperparathyroidismS 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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? ArteriogramOSTEOSARCOMA
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% ofprimary 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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21Lytic
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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dcortical 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
23
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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, andSmall 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 arePaget disease
Previous radiation treatment
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Other associated conditions areFibrous dysplasia Bone infarcts
Osteochondromas Chronic
osteomyelitis
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Dedifferentiated chondrosarcomasOsteogenesis 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" appearencePeriosteal 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 lesionSmal cell Osteosarcoma
? Rare
? High grade
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? Resemble Ewing sarcoma or LymphomaTreatment
Current standard of care
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27
Radiological staging
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Biopsy to confirm diagnosis Preoperativechemotherapy
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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techniquesPost 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 chondrosarcomaSecondary 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 TumorChondroblastoma
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 aotherwise typical chondrosarcoma.
POP CORN CALCIFICATION
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DIFFERENCE FROM ENCHONDROMAo 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- malignancyIntramedullary ,soft
tiisue extention of tumour
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with calcificationChondrosarcomas 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 neoplasmThird 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 25Histogenesis: 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, andAn increased erythrocyte sedimentation rate.
These systemic symptoms may lead to an erroneous diagnosis of osteomyelitis.
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Radiological featureslesion 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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FeaturesOnion 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 seehair 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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