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.

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Occurence
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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

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• 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

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• Other connective tissue tumours (malignant)
• Fibrosarcoma
• Malignant fibrous histiocytoma
• Liposarcoma
• Malignant mesenchymoma
• Leiomyosarcoma
• Undifferentiated sarcoma

• Secondary malignant tumours of bone
• Osteoblastic
• Osteolytic
• Mixed Lesions

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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

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Age distribution of various bone tumors
9
Imaging

• Plain radiography
• CT scan
• MRI
• Radionuclide scanning
• PET

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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

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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.

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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

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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

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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

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• 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.

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Narrow zone of transition

• Wide zone of transition

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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

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Patterns of Destruction
Geographic
Moth-eaten
Permeative
Less malignant More malignant
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Types of Periosteal reaction

• Benign
• None
• Solid/Continuous
• More aggressive or malignant
• Interrupted
• Lamellated or onion-skinning
• Sunburst
• Codmans triangle

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Periosteal Reactions
Sunburst
Codman's Triangle
Solid
Lamellated
Less malignant More malignant
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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
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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
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Radiographic features differentiating primary
soft tissue tumor invading bone from
primary bone tumor invading soft tissues
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Radiographic features that may help differentiate
benign from malignant lesions
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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.

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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

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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

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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

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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

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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.

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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).

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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.

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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.

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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.

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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

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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

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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).

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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

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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

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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)

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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

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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.

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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.

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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

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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

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Metastases (contd.)
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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.

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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

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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.

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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.

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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.

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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

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• 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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