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An Approach To Malignant Bone Tumors

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Title: An Approach To Malignant Bone Tumors


1
An Approach to Malignant Bone Tumors
  • Dr.Suhas B
  • MD
    Radio-Diagnosis

2
Introduction
  • Bone tumors are classified into
  • Primary bone tumors
  • Secondary bone tumors ( Metastasis)
  • Most are classified according to the normal cell
    of origin and apparent pattern of
    differentiation.
  • Forms 0.2 of human tumor burden.
  • Primary malignant bone tumors make 1 of all
    malignant tumors.
  • Commonest bone tumour is secondaries from other
    sites.
  • Commonest primary bone tumour is multiple
    myeloma, second osteosarcoma.

3
Occurence
4
Classification
  • Bone-forming tumours
  • Cartilage forming tumours
  • Giant-cell tumour
  • Marrow tumours
  • Vascular tumours
  • Other connective tissue tumours
  • Other tumours
  • Secondary malignant tumours of bone

5
  • Bone-forming tumors (malignant)
  • Osteosarcoma
  • Central (medullary)
  • Peripheral (surface)
  • Paraosteal
  • Periosteal
  • High grade surface
  • Cartilage-forming tumors (malignant)
  • Chondrosarcoma
  • Differentiated chondrosarcoma
  • Juxta-cortical chondrosarcoma
  • Mesenchymal chondrosarcoma
  • Clear cell chondrosarcoma
  • Marrow tumors (malignant)
  • Ewings sarcoma
  • Neuroectodermal tumour
  • Malignant lymphoma of bone (Primary/secondary)
  • Myeloma
  • Vascular tumors (malignant)
  • Angiosarcoma
  • Malignant haemangio pericytoma
  • Other tumors (malignant)
  • Chordoma
  • Adamantinoma

6
  • Other connective tissue tumours (malignant)
  • Fibrosarcoma
  • Malignant fibrous histiocytoma
  • Liposarcoma
  • Malignant mesenchymoma
  • Leiomyosarcoma
  • Undifferentiated sarcoma
  • Secondary malignant tumours of bone
  • Osteoblastic
  • Osteolytic
  • Mixed Lesions

7
Evaluation
  • Age Sex
  • Symptomatology
  • Pain
  • Swelling
  • History of trauma
  • Neurological symptoms
  • Restriction of movement
  • Other constitutional sympotms (cough, chest pain,
    backache, loss of appetite etc)
  • Pathological fracture
  • Associated conditions
  • Prior surgeries/investigations

8
Age distribution of various bone tumors
9
Imaging
  • Plain radiography
  • CT scan
  • MRI
  • Radionuclide scanning
  • PET

10
Plain Radiography
  • Information yielded by radiography includes
  • Size
  • Site of the Lesion
  • Borders of the lesion/zone of transition
  • Type of bone destruction
  • Periosteal reaction
  • Matrix of the lesion
  • Nature and extent of soft tissue involvement
  • Multiplicity

11
Plain radiography (contd.)
  • Size
  • It helps us in pre treatment staging of the
    tumor.
  • The larger the lesion the more likely to be
    aggressive or malignant.

12
Plain radiography (contd.)
  • Site
  • Type of bone
  • Long bone / Flat bone
  • Intramedullary / Eccentric / Cortical lesion
  • The epicenter of the tumor helps to determine the
    origin.
  • Epiphysis / Metaphysis / Diaphysis

13
Distribution of various bone tumors
14
Distribution of various lesions in a long tubular
bone before skeletal maturity
  • Distribution of various lesions in a long
    tubular bone after skeletal maturity

15
Distribution of various lesions in a vertebra
  • Benign lesions predominate in its posterior
    elements.
  • Osteoblastoma
  • Osteoid osteoma
  • Aneurysmal Bone cyst
  • Osteochondroma
  • Chondromyxoid fibroma
  • Malignant lesions are seen predominantly in
    its anterior part (body)
  • Lympohoma
  • Myeloma
  • Osteosarcoma
  • Ewing
  • Chondrosarcoma
  • Metastases

16
(No Transcript)
17
  • Zone of Transition
  • The zone of transition is the most reliable
    indicator in determining whether an osteolytic
    lesion is benign or malignant.
  • The zone of transition only applies to osteolytic
    lesions since sclerotic lesions usually have a
    narrow transition zone.
  • A small zone of transition results in a sharp,
    well-defined border and is a sign of slow growth.
    A sclerotic border especially indicates poor
    biological activity.
  • An ill-defined border with a broad zone of
    transition is a sign of aggressive growth.

18
Narrow zone of transition
  • Wide zone of transition

19
Patterns of Bone Destruction
  • Mechanisms of bone destruction
  • Direct effect of tumor cells
  • Increased osteoclastic activity
  • Cortical bone is destroyed less rapidly than
    trabecular bone.
  • Loss of cortical bone appears earlier on
    radiography
  • trabecular bone must be destroyed (about 70 loss
    of mineral content) before the loss becomes
    radiographically evident
  • Bone destruction can be described as
  • geographic (type I) - benign lesions
  • moth-eaten (type II) and
  • permeative (type III) - rapidly growing
    infiltrating tumors

20
Patterns of Destruction
Geographic
Moth-eaten
Permeative
Less malignant More malignant
21
Types of Periosteal reaction
  • Benign
  • None
  • Solid/Continuous
  • More aggressive or malignant
  • Interrupted
  • Lamellated or onion-skinning
  • Sunburst
  • Codmans triangle

22
Periosteal Reactions
Sunburst
Codman's Triangle
Solid
Lamellated
Less malignant More malignant
23
Types of matrix osteoblastic
The matrix of a typical osteoblastic lesion is
characterized by the presence of the following
features
B. presence of the wisps of tumor-bone
formation, like in this case of osteosarcoma of
the sacrum
A. fluffy, cotton-like densities within the
medullary cavity, e.g in this case of
osteosarcoma of the distal femur
C. by the presence of a solid sclerotic mass,
such as in parosteal osteosarcoma
24
Types of matrix chondroid
matrix
A Schematic
representation of various
appearances of chondroid matrix calcifications.
B Enchondroma displays a typical chondroid matrix
C Chondrosarcoma with characteristic chondroid
matrix
25
Radiographic features differentiating primary
soft tissue tumor invading bone from
primary bone tumor invading soft tissues
26
Radiographic features that may help differentiate
benign from malignant lesions
27
CT
  • Features are similar to that of plain radiograph,
    however CT scanning may be helpful locally when
    the radiographic appearances are confusing,
    particularly in areas of complex anatomy.
  • Very useful in early diagnosis.
  • Cross-sectional images provide a clearer
    indication of bone destruction, as well as the
    extent of any soft tissue mass, than the
    radiographs.
  • CT scanning may depict small amounts of
    mineralized osseous matrix not seen on
    radiographs.
  • The modality may be particularly helpful in
    visualizing flat bones, in which periosteal
    changes may be more difficult to appreciate.
  • Early detection of pulmonary secondaries
  • Exact measurement for limb salvage procedures
    (Prosthesis/allograft)
  • Used for prognostic follow-up of the patient.

28
MRI
  • Investigation of choice to assess intra-medullary
    extension and soft tissue involvement.
  • Defines the relationship to the nearby
    neurovascular bundles.
  • Ambiguous and inconspicuous cases.
  • Helps in staging of the tumor and to plan its
    surgical management.
  • Radio-nuclide bone scan
  • For pre biopsy staging
  • Dissemination of tumour
  • Silent secondaries and skip lesions
  • Arteriogram
  • Planning limb sparing surgery
  • Therapeutic embolization
  • To assess vascularity of tumour

29
Osteosarcoma
  • Osteosarcoma is a primary bone-producing
    malignant mesenchymal tumor.
  • It is the most common primary malignant tumor of
    bone, excluding plasma cell myeloma.
  • Osteosarcoma represents 20 of all primary
    malignant bone tumors.
  • Osteosarcoma is encountered most commonly in the
    age group from 10 to 25 years (75 of cases) few
    cases occur before age 5 or after age 30.
  • MF21
  • The metaphyseal lesion abutting the physis is the
    classic location in 75 of cases.
  • Although usually in the long bones, may occur in
    other places
  • Craniofacial
  • Small bones
  • Extraskeletal (soft tissue)
  • Associations - Irradiation, Pagets disease,
    Rothmund-Thompson syndrome

30
Osteosarcoma (contd.)
  • Conventional Radiography
  • medullary and cortical bone destruction
  • wide zone of transition, permeative or moth-eaten
    appearance
  • aggressive periosteal reaction
  • sunburst type
  • Codman triangle
  • lamellated (onion skin) reaction less frequently
    seen
  • soft-tissue mass
  • tumour matrix ossification/calcification
  • variable reflects a combination of the amount of
    tumour bone production, calcified matrix, and
    osteoid
  • ill-defined "fluffy" or "cloud-like" cf. to the
    rings and arcs of chondroid lesions
  • Radiography

31
Osteosarcoma (contd.)
  • CT
  • Cross-sectional images provide a clearer
    indication of bone destruction, as well as the
    extent of any soft tissue mass, than do
    radiographs.
  • CT scanning may depict small amounts of
    mineralized osseous matrix not seen on
    radiographs. The modality may be particularly
    helpful in visualizing flat bones, in which
    periosteal changes may be more difficult to
    appreciate

32
Osteosarcoma (contd.)
  • MRI
  • T1WI - Low/heterogenous signal intensity
  • T2WI - High signal intensity
  • Constrast - enhancing medullary cavity and solid
    components.
  • STIR - High signal intensity and helps in
    assessing involvement of neurovascular bundles
    and muscles.

33
Multiple Myeloma
  • Multiple myeloma is the most common primary
    malignant neoplasm of the skeletal system. The
    disease is a malignancy of plasma cells.
  • Average age is 60-70
  • Much more common in men than women
  • Most have an elevated serum protein with 80-90
    in the globulin fraction, especially IgG
  • Bence-Jones protein in 40-60 of patients
  • Most commonly affected bones are vertebrae
    (66), ribs (45), skull (40), shoulder (40),
    pelvis (30), and long bones (25).

34
Multiple Myeloma (contd.)
  • Plain Radiography
  • A full skeletal survey is required for proper
    evaluation.
  • No. of lytic lesions is directly proportional to
    the tumor load.
  • Classical lesions are well defined
    rounded/punched out lytic lesions scattered
    diffusely among the involved bones.
  • Such lesions in skull gives 'pepper pot' or
    'swiss cheese' appearance, however the occipital
    bone is spared.
  • Multiple deformed vertebral bodies.
  • Diffuse osteopenia
  • No periosteal bone formation.
  • Medullary involvement in the form of endosteal
    scalloping.

35
Multiple Myeloma (contd.)
  • CT
  • Computed tomography (CT) scanning readily depicts
    osseous involvement in myeloma.
  • CT allowed a more accurate evaluation of areas at
    risk of fracture.
  • Tool of choice utilised in image guided spinal
    or pelvic bone biopsy.
  • MRI
  • Most sensitive imaging modality at detecting
    diffuse and focal multiple myeloma in the spine,
    as well as the extra-axial skeleton
  • Mainly bone marrow based lesions.
  • T1WI - Low signal intensity
  • T2WI and STIR - High signal intensity.
  • Show enhancement on contrast enhanced images.

36
Chondrosarcoma
  • malignant cartilaginous tumour and second most
    frequent primary malignant tumor of bone,
    representing approximately 25 of all primary
    osseous neoplasms.
  • Typical presentation is in the 4th and 5th
    decades and there is a slight male predominance
  • Further differentiated into Primary and secondary
  • Most common bones involved are long bones (45),
    pelvis (25), ribs (8), spine (7), scapula
    (5), sternum (2)
  • Patients with multiple enchondromas like in
    Ollier's disease and Mafucci's syndrome are at
    risk.

37
Chondrosarcoma (contd.)
  • Plain radiography
  • large mass at the time of diagnosis, usually over
    4 cm in diameter in 50 of cases.
  • lytic (50)
  • intralesional calcification(s) 60-78 (rings and
    arcs calcification or popcorn calcification)
  • endosteal scalloping
  • affects more than two thirds of the cortical
    thickness (c.f. less than 2/3 in enchondromas)
  • moth eaten appearance or permeative appearance in
    higher grade tumours
  • cortical remodelling, thickening and periosteal
    reaction are also useful in distinguishing
    between an enchondroma and low grade
    chondrosarcoma

38
Chondrosarcoma (contd.)
  • CT
  • In as many as 90 of cases, tumors appear as
    lucent areas containing chondroid matrix
    calcification. Endosteal scalloping and cortical
    destruction are frequently easier to appreciate
    on CT scans than on radiographs.
  • CT scanning may be used to guide percutaneous
    biopsy
  • MRI
  • T1 low to intermediate signal
  • T2 very high intensity in non mineralised/calcifi
    ed portions
  • gradient echo/SWI blooming of mineralised/calcifi
    ed portions
  • T1 C (Gd)
  • most demonstrate heterogeneous moderate to
    intense contrast enhancement.
  • enhancement can be septal and peripheral rim-like
    corresponding to fibrovascular septation between
    lobules of hyaline cartilage

39
Ewings Sarcoma
  • Ewing sarcoma, a highly malignant primary bone
    tumor that is derived from red bone marrow and
    second most common primary bone tumour of
    childhood.
  • This tumor is most frequently observed in
    children and adolescents aged 4-15 years and
    rarely develops in adults older than 30 years.
  • Affected bones include, long bones 50-60,
    femur 25, tibia 11, humerus 10, flat bones
    40, pelvis 14, scapula, ribs 6
  • As far as location within long bones, the tumor
    is almost always metaphyseal or diaphyseal.
  • It is the most lethal bone tumor. An association
    exists between Ewing sarcoma and primitive
    peripheral neuroectodermal tumor (PNET).

40
Ewing's Sarcoma (contd.)
  • Plain Radiography
  • Typical presentation ill-defined osteolytic
    lesion with a moth-eaten or permeative type of
    bone destruction, irregular cortical destruction
    and aggressive periostitis.
  • reactive sclerosis, irregular periosteal reaction
    and soft tissue mass.
  • Ewing sarcomas tend to be large poorly marginated
    tumours, with over 80 demonstrating extension
    into adjacent soft tissues.
  • laminated (onion skin) periosteal reaction 57

41
Ewings Sarcoma (contd.)
  • CT
  • CT scanning helps to define the bone destruction
    that is associated with Ewing sarcoma.
  • Tumor size can be evaluated with
    contrast-enhanced CT scanning, which may be used
    in follow-up evaluation during chemotherapy.
  • MRI
  • MRI is essential to elucidate soft-tissue
    involvement
  • T1 low to intermediate signal
  • T1 C (Gd) heterogeneous but prominent
    enhancement
  • T2 heterogeneously high signal, may see hair on
    end low signal striations

42
Lymphoma
  • Primary lymphoma of bone (PLB) is a rare,
    malignant, neoplastic disorder of the skeleton.
    Also known as Reticulum cell sarcoma.
  • Primary bone lymphoma occurs in a broad range of
    patients, aged 1 year 6 months to 86 years
    (median range, 3652 years) (5), with a peak
    prevalence among patients in the 6th to 7th
    decades of life.
  • Presentation usually pain and palpable mass.
  • Preferential sites femur, tibia, humerus and
    iliac bone.
  • Plain Radiography
  • The features vary widely in appearance. The
    most common ones are as below
  • Permeative, lytic pattern of bone destruction
    (74)
  • Metadiaphyseal location (69)
  • Periosteal reaction (58)
  • Soft-tissue mass (80-100)

43
Lymphoma (contd.)
  • CT
  • useful adjuncts to conventional radiographs
  • pattern appears as extensive evidence of disease
    within the marrow cavity associated with a
    surrounding soft-tissue mass but without
    extensive cortical destruction
  • Cortical breakthrough is well appreciated.
  • MRI
  • T1WI - low signal intensity within the marrow
  • T2WI - High signal intensity
  • Contrast - diffuse heterogenous/homogenous
    enhancement

44
Adamantinoma
  • rare primary malignant bony tumour, only
    approximately 200 cases have been reported.
  • The tumor occurs almost exclusively in the long
    bones tumors in the tibia account for more than
    80 of cases. The diaphyseal region is the area
    most commonly affected. Other bones affected are
    the jaw, ulna, humerus, femur, and fibula.
  • Typically presents in the 2nd to 3rd decades as a
    locally aggressive mass 3-15 cm in diameter.
  • Differentiated lt20 years of agegt Classic
  • 10-year survival rate is believed to be 10
  • Adamantinoma may present as a solitary focus or
    multicentric lucencies or slightly expansile
    osteolytic lesion
  • May extend into the marrow cavity.
  • Lesions tend to have an eccentric epicenter and
    a lack of periosteal reaction.
  • usually no periosteal reaction is noted in the
    surrounding bone
  • Long-standing tumors produce marked cortical
    thickening and spool-shaped bulges of the outer
    cortex in an eggshell fashion.

45
Adamantinoma
  • MRI
  • two morphologic patterns are seen
  • - a solitary lobulated focus
  • - multiple small nodules in one or more
    foci.
  • In some patients separated tumour foci may be
    seen, defined as foci of high signal intensity on
    either T2- or T1-weighted contrast-enhanced
    images, interspersed with normal-appearing
    cortical or spongious bone.
  • Fluid-fluid level may occasionally be seen.
  • C(Gd) tends to show intense and homogeneous
    static enhancement, although there is no uniform
    dynamic enhancement pattern.

46
Metastases
  • Metastatic bone tumors are the most common
    malignant tumors of the skeleton. Approximately
    70 of all malignant tumors are metastatic in
    origin.
  • metastases are usually found in Vertebrae -
    especially posterior vertebral body, extending
    into pedicle, pelvis, proximal femur,
    proximal humerus and skull.
  • Metastases distal to the elbow and knee are
    distinctly uncommon.
  • Types
  • - osteolytic metastases
  • - sclerotic/osteoblastic metastases
  • - mixed lytic and sclerotic metastases
  • Osteoblastic metastases Osteolytic
    metastases Mixed metastases
  • prostate carcinoma (most common) RCC
    Lung carcinoma (25)
  • breast carcinoma (may be mixed) Thyroid
    carcinoma Breast carcimoma
    (15)
  • transitional cell carcinoma (TCC)
    Pheochromocytoma carcinoma of
    cervix
  • carcinoid
    Wilms tumor testicular
    tumors
  • medulloblastoma
    Ewings sarcoma Prostatic
    carcinoma (15)
  • neuroblastoma
    Carcinomas of GIT
  • mucinous adenocarcinoma of GIT Melanoma
  • lymphoma HCC

  • SCC of skin

47
Metastases (contd.)
  • Imaging Findings
  •  little or no soft tissue mass associated with
    them
  • Usually no periosteal reaction
  • May appear as moth-eaten, permeative or
    geographic lesions
  • Indistinct zones of transition, no sclerotic
    margins and may be sharply circumscribed or have
    indistinct borders
  • Lesions distal to elbows and knees - 50 are from
    lung and breast
  • Diffuse skeletal sclerosis or multiple round,
    well-circumscribed sclerotic lesions - Prostate
    Breast
  • Expansile and lytic (soap-bubbly) - RCC
  • Cookie-bite lesions of the cortices of long bones
    - Lung
  • Bone scans are extremely sensitive but not very
    specific
  • 10-40 of lesions will not be visible on plain
    film but will be positive on bone scans
  • CT or MRI can be used to show findings in
    patients with negative conventional radiographs
    and positive bone scans

48
Metastases (contd.)
49
Fibrosarcoma
  • Fibrosarcoma is a tumor of mesenchymal cell
    origin that is composed of malignant fibroblasts
    in a collagen background..
  • It can occur as a soft-tissue mass or as a
    primary or secondary bone tumor.
  • Primary fibrosarcoma - central (arising within
    the medullary canal) or peripheral (arising from
    the periosteum)
  • Secondary fibrosarcoma of bone arises from a
    preexisting lesion or after radiotherapy to an
    area of bone or soft tissue.
  • Fibrosarcomas of bone are typically seen between
    the third and sixth decades of life.
  • most commonly in the metaphysis or metadiaphysis
    of the long tubular bones.

50
Fibrosarcoma (contd.)
  • Plain Film
  •   Highly destructive with a wide zone of
    transition and often expansile. Periosteal
    reaction is uncommon.  The lesion usually has not
    matrix mineralization, but may have areas of
    sequested bone. Often associated with a large
    soft tissue mass.
  • CT
  •   CT scanning is used to delineate bone
    involvement, bone destruction, or bone reaction.
  • MRI
  • best modality overall for examining
    soft-tissue masses and for detecting the
    intraosseous and extraosseous extent of many bony
    sarcomas.
  • T1WI - Isointense
  • T2WI - Hyperintense
  • Shows strong enhancement on contrast images

51
Pleomorphic undifferentiated sarcoma
  • Previously known as malignant fibrous
    histiocytoma.
  • high grade tumor composed of fibroblasts,
    myofibroblasts, and histiocytes.
  •  most frequent soft tissue tumor in adults.
  •  found in the extremities 70-75 of the time and
    50 of all cases are in the lower extremity.
  • highest incidence is during the fifth decade of
    life and there is a male to female ratio of 1.5
    to 1
  • Plain Film
  • Plain x-rays will demonstrate a soft tissue
    mass and if arising from bone, then an aggressive
    destructive bony lesion. In some cases,
    curvilinear or punctate regions of calcification
    may be demonstrated.

52
Pleomorphic undifferentiated sarcoma
  • CT
  • The density of MFH is typically similar to
    adjacent muscle, with heterogeneous lower density
    areas if haemorrhage, necrosis or myxoid material
    is abundant.
  • The soft tissue component enhances.
  • In up to 15-20 of cases some mineralisation is
    present.
  • MRI
  •   typically relatively well circumscribed,
    located within or adjacent to muscle, exerting
    positive mass effect on surrounding structures.
  • T1WI - Highly variable, isointense (muscle),
  • T2WI - intermediate to high signal intensity
  • Heterogeneous and shows enhancement of solid
    components.

53
Chordoma
  • Chordomas are uncommon malignant tumours that
    account for 1 of intracranial tumours and 4 of
    all primary bone tumours.
  • They originate from embryonic remnants of the
    primitive notochord.
  • Occurs between the ages of 30-70 with a 21
    malefemale ratio
  • 50 occur in sacrum, in  4th or 5th sacral
    segment.
  • 35 at skull base around clivus
  • vertebral body 15-30
  • Plain Film
  • Large presacral mass (gt10cm), destruction of
    multiple sacral and coccygeal segments.
  • Sclerotic rim in 50,  amorphous calcifications (
    mainly peripherally)
  • May cross the sacroiliac joint
  •  Pattern is lytic, with sequestered bone
    fragments.

54
Chordoma (contd.)
  • CT
  • CT is helpful in defining bone destruction and
    calcification within lesion.
  • With contrast, the pseudocapsule may enhance.
  • Usually low attenuation soft tissue mass with
    destruction of the sacrum and/or coccyx,
    sometimes with marginal sclerosis.
  • May show sequestered bone fragments or
    calcifications within tumor.
  • MRI
  • T1WI intermediate to low signal intensity with
    a small foci of hyperintensity (hemorrhage or
    mucus)
  • T2WI - most exhibit very high signal .
  • T1 C (Gd) heterogeneous enhancement with a
    honeycomb appearance corresponding to low T1
    signal areas within the tumour

55
  • Bibliography
  • Essentials of skeletal radiology 3rd edition -
    Yochum Rowe
  • CT and MRI of whole body 5th edition
    John.R.Haaga
  • Musculoskeletal Imaging The Requisites 3rd
    edition B.J.Manaster
  • Musculoskeletal MRI 2nd edition Helms
  • Bone Tumors and Tumor like Conditions Analysis
    with Conventional Radiography Miller
  • Expert DDX Musculoskeletal system B.J. Manaster
  • Pathologic and Radiologic features of primary
    Bone tumors - Update.com
  • Imaging in various primary and secondary bone
    tumors - Medscape
  • Bone tumors - Radiology assistant Radiopedia
  • Study in various primary bone tumor - Journal of
    Bone Oncology PubMedRSNAKJRIJR
  • Metastatic diseases of bone Learning Radiology

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