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Imaging in malignant bone tumors
1. JSS Medical College, Mysuru
IMAGING IN MALIGNANT BONE
TUMOURS
-Dr Suman T P
Resident,
JSS Medical College,Mysuru
2. JSS Medical College, Mysuru
INTRODUCTION
• Divided into two broad categories:
Benign & malignant (primary and secondary).
• Plain radiographs are initial and most important imaging
modality for the diagnosis.
• CT scan is particularly useful in evaluating the cortex and
matrix.
• MRI is useful in determining the intramedullary extent,
soft tissue extent of focal bone lesion and also play vital
role in detecting skip lesions.
• Commonest bone tumour is secondaries from other sites.
• Commonest primary bone tumour is multiple myeloma,
second osteosarcoma.
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CLASSIFICATION OF PRIMARY MALIGNANT
BONE TUMORS
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Staging
Enneking staging AJCC- International Union against Cancer (UICC)
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SYSTEMATIC APPROACH
Patient’s age
Location of the lesion
LONGITUDINAL PLANE (epiphysis, metaphysis, diaphysis)
TRANSVERSE PLANE (intramedullary, intracortical, surface).
Solitary or multiple
Pattern of bone destruction
Edge of lesion/zone of transition
Matrix mineralization
Cortical response
Periosteal reaction
Extraosseous extension/soft tissue mass
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AGE AT PRESENTATION
Edeiken classified malignant tumors into the
following age groups:
DIFFERENTIAL DIAGNOSIS CAN BE NARROWED
CLUE TO AGE : FROM EPIPHYSEAL FUSION
7. JSS Medical College, Mysuru
1. In the transverse plane:
a) Central – Enchondroma
b) Eccentric -GCT, osteosarcoma,
chondromyxoid fibroma
c) Cortical - Non-ossifying fibroma,
osteoid osteoma
d) Parosteal - Parosteal osteosarcoma,
osteochondroma
LOCATION
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Patterns of Bone Destruction
• Bone destruction can be described as
– Geographic (type I) - benign lesions
– Moth-eaten (type II)
– Permeative (type III) - rapidly growing infiltrating tumors
CHONDROBLASTOMA,
BONE CYST
Myeloma, EG,
Fibrosarcoma
Osteosarcoma,
osteomyelitis etc.
Osteosarcoma, GCT
etc.Ewings, Osteosarcoma,
Lymphoma etc.
Less malignant More malignant
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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.
SBC, ABC, GCT, FD etc. Osteosarcoma, Fibrosarcoma etc.
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Patterns of mineralization of cartilaginous tumor
matrix
Stippled
Flocculent
Ring and arc
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Solid
Patterns of mineralization of osseous matrix
Ivory-like opacityCloudlike
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Types of Periosteal reaction
• Benign
– None
– Solid/Continuous
• More aggressive or malignant
Interrupted
– Lamellated or onion-skinning
– Sunburst
– Codman’s triangle
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Periosteal Reactions
Less malignant More malignant
Solid
Eg: Post
traumatic, HPOA
Sunburst
Eg: Osteosarcoma,
Ewings sarcoma
Codman's Triangle
Eg: Osteosarcoma,
Ewings sarcoma
Lamellated
Eg: Ewings,
Osteomyelitis
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Malignant Bone Neoplasms: Differentiating Radiologic
Features between Primary and Secondary Lesions
Feature Primary Secondary
Incidence 30% 70%
Expansion of bone +++ +
Length of lesion > 6 cm 2-4 cm
Periosteal response +++ +
Solitary lesion +++ +
Multiple lesions + +++
Soft tissue mass +++ +
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OSTEOSARCOMA
• It commonest primary malignant lesion of bone after myeloma.
• Classified as
– PRIMARY OSTEOSARCOMA
a) CENTRAL(75%)
b) INTRACORTICAL or SURFACE
i. Parosteal
ii. Periosteal
iii. High-grade
– SECONDARY OSTEOSARCOMA: Paget's disease (Paget's sarcoma), Radiotherapy
(post-radiation sarcoma) or as the dedifferentiated part of chondrosarcoma.
Osteosarcoma may
occasionally be multicentric
(osteosarcomatosis) or
arise in the soft tissues
(extraskeletal).
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PLAIN FILM FEATURES OF OSTEOSARCOMA
Most common primary bone tumor of adolescence.
Peak incidence ages 8-25, and 40+ secondary to
Paget’s Disease.
• Commonly found in the metaphyses of long bones.
• Appearance can be lytic(25%), sclerotic(50%) or
mixed(25%).
• Trabecular pattern typically permeative with wide zone of
transition.
• Periosteal reactions include: sunburst, hair on end, and
Codman’s triangle.
• Typically associated with a soft tissue mass.
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Increase in bone density in the metaphysis and distal diaphysis of the femur. There is a
sunburst or malignant spiculated periosteal response present surrounding the entire distal
metaphysis of the femur. This sunburst appearance, the soft tissue mass, and the destructive
lesion of the medullary portion of the femur are the cardinal radiographic features of
osteosarcoma.
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OSTEOSARCOMA
Tibia.
Diffuse ivory
osteosarcoma in the
proximal tibia.
Approximately 50%
of the
osteosarcomas
present as a sclerotic
lesion, rendering an
ivory or sclerotic
appearance.
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Sclerotic osteosarcoma affecting the ilium near the acetabulum. Note
the lobulated margins of the medial surface of this osteosarcoma
(arrows) called the CUMULUS CLOUD APPEARANCE.
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Osteosarcoma with metastases: CANNONBALL METASTASES
Extensive metastasis to the lungs.
NOTE: Spontaneous pneumothorax is relatively common because
subpleural nodules that have undergone excavation lead to rupture
into the pleural space.
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• Slow growing rare tumor with low grade malignancy.
• Arises from periosteum, grows outwards & tends to
surround the host bone - Dense mass of new bone away
from the cortex with lucent area in the periphery.
• Fine radiolucent line between cortex & dense
tumour mass with central stalk(string sign ) –
Characteristic.
Paraosteal OS (Juxta cortical OS)
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Osteosarcoma (contd.)
MRI
• T1WI - Low/heterogenous signal intensity
• T2WI - High signal intensity
• Contrast - enhancing medullary cavity and solid
components.
• STIR - High signal intensity and helps in assessing
involvement of neurovascular bundles and
muscles.
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Bone scintigraphy and sagittal T2-weighted
MR images showing a large primary osteosarcoma of
the distal femur with multiple proximal skip
metastases.
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• Distinct from conventional OS.
• Adolescence.
• High grade – poor prognosis, more
aggressive than paraosteal variety.
• Sessile elliptical growth on periosteal
aspect of the diaphysis.
• Short spicules of bone perpendicular
to shaft / laminated periosteal
reaction.
• Base of tumor attached to entire
extent of cortex.
• Absence of medullary invasion.
Periosteal OS
POORLY DEFINED OSSIFICATION OF
MASS ATTACHED TO THE SURFACE
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TELANGIECTATIC OSTEOSARCOMA
• Second to third decade
• High degree of vascularity
• Large cystic spaces filled
with blood.
• Osteolytic lesion with
absence of sclerosis.
• Telangiectatic OS shows that
the tumor usually arises
from the metaphysis of a
long bone and is expansile
and lytic. It contains multiple
fluid levels due to layering
haemorrhage.
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Radiograph of the shoulder shows the expansile lesion of the scapula with a thick curvilinear
band of mineralized matrix (osteoid) superolaterally
Posterior bone scintigraphic image shows marked heterogeneous uptake of radionuclide
peripherally and central photopenia (donut sign).
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SECONDARY OSTEOSARCOMA
Malignant degeneration of benign disorders,
such as Paget’s disease, polyostotic fibrous
dysplasia, hereditary multiple exostosis
(osteochondromas), and enchondromatosis
(Ollier’s disease), may lead to osteosarcoma.
The radiographic features of permeative, moth-
eaten bone destruction with periosteal reaction
and soft tissue mass is largely indistinguishable
from those osteosarcomas arising de novo.
PAGET’S SARCOMA. Humerus.
Pathologic fracture and extensive destruction of the
proximal humerus and extensive cortical thickening
of the visualized diaphysis of the humerus.
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Chondrosarcoma
• Chondrosarcoma is a malignant tumor of chondrogenic origin that
remains essentially cartilaginous throughout its evolution.
Primary chondrosarcoma arises de novo.
Secondary chondrosarcoma arises in a pre-existing cartilaginous
lesion (enchondroma, osteochondroma).
Central chondrosarcoma arises intramedullary.
Peripheral chondrosarcoma arises on the surface of the bone.
• Age of presentation is 40-60 years, with 2:1 male predominance.
• Common sites include pelvis (30%), proximal and distal femur, ribs,
proximal humerus, and proximal tibia.
• Patients present with pain or mass.
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Two distinct appearances:
• Osteolytic lesion with short transitional zone
& sclerotic rim
– Irregular, fine calcific specks
• Osteolytic lesion without sclerotic rim
– “Snow-flake” calcified chondroid matrix
– Scalloping of cortex
Chondrosarcoma – Central Type
Site : Neck of femur, pubic rami, prox. end of humerus
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• Arise from cartilagenous cap of osteochondroma.
• Insidious growth.
• Unusually large soft tissue mass (with calcification) &
attached to bone
• Later underlying bone destroyed
• Dense radiopaque center with calcific streaks radiating
towards periphery
Peripheral Chondrosarcoma
Site : Pelvis, Scapula, sternum, ribs
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CT
• Lucent areas containing chondroid matrix
calcification. Endosteal scalloping and cortical
destruction.
• CT scanning may be used to guide
percutaneous biopsy
MRI
• T1: low to intermediate signal
• T2: very high intensity in non
mineralised/calcified portions
• gradient echo/SWI: blooming of
mineralised/calcified portions
T1 C+ (Gd)
• 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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Multiple Myeloma
• The most common primary malignant bone tumor.
• 75% of patients are between 50 and 70 years of
age; 2:1 male preponderance.
• Tumor made up of malignant monoclonal plasma
cells.
• Classic radiographic appearance is multiple lytic
“punched out” areas in bone.
• Lesions often do not show uptake of isotope on
bone scan, making a skeletal survey the most
important radiographic test.
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Gross osteoporosis throughout the lumbar spine. There is a
compression fracture of L2 (arrow).
Diffuse osteoporosis throughout the entire lumbar spine. There are
pathologic fractures of the T12 and L2 vertebral bodies.
OSTEOPOROSIS:
THE EARLIEST
RADIOGRAPHIC
SIGN OF MYELOMA.
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PUNCHED-OUT LESIONS OF MULTIPLE MYELOMA.
Radiologic hallmark of multiple myeloma is the sharply circumscribed
osteolytic defect that is clearly demonstrated on these radiographs.
The lesions are multiple, round, and purely lytic.
The most frequent sites are bones with hematopoietic potential.
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MULTIPLE MYELOMA
Collapse of the T7 vertebral body (arrow). The pedicles have not been destroyed
and are visualized on this frontal radiograph (arrowheads). Uniform collapse of the
T7 vertebral body, collapse of the posterior third of the vertebral body, which
strongly suggests a pathologic fracture.
Characteristic
appearance for a
wrinkled vertebra
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MULTIPLE MYELOMA: RAINDROP
SKULL
Diffuse, permeative, or punched-
out lesions throughout the
calvaria.
NOTE:
Multiple myeloma of the skull may
be differentiated from metastatic
carcinoma by the more uniform
size of the lytic lesions in
myeloma.
The co-existence of both large and
small lesions is often the mode of
presentation of metastatic
disease.
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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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Metastatic Disease
• Most common sites for bony metastases
include thoracic and lumbar spine, pelvis,
femur, rib, proximal humerus and skull
THE VERTEBRAL VEIN SYSTEM
(BATSON’S VENOUS PLEXUS).
This venous network is a common
two-way avenue of metastatic spread
of pelvic, abdominal, and thoracic
tumors. A large portion of bony
metastases results from
dissemination of neoplastic cells
through the vertebral vein system.
Pathways of Metastasis:
Cancers metastasize through one of
three routes:
direct extension, lymphatic channels,
or hematogenous dissemination.
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•Technetium bone scans (99mTc-MDP) are more accurate in detecting early
bone metastases than are conventional radiographs.
•80% of all metastases are located in the central or axial skeleton, with the
spine and pelvis being the most common sites
•75% of all metastatic lesions are osteolytic, creating a moth-eaten or
permeative pattern of bone destruction.
•15% of all metastatic lesions are osteoblastic in nature, creating a snowball
pattern with diffuse lesions.
•10% of all metastatic lesions are solitary.
•Vertebral body and pedicles are the most common sites for metastatic
deposits.
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BLASTIC METASTASIS:
LATERAL SKULL. Numerous well-defined radiopacities scattered throughout the
calvaria. These represent osteoblastic metastatic deposits secondary to carcinoma
of the prostate gland.
NOTE: Approximately 10% of all metastatic lesions affect the calvaria, with > 90% of
the calvarial metastatic deposits being lytic and only 10% being blastic.
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ISCHIAL METASTASIS:
Loss of bone density and a moth-eaten pattern of bone destruction scattered
throughout the ischium.
The poor zone of transition around the lytic lesion suggests an aggressive
disorder of bone.
This lytic metastasis is secondary to carcinoma of the breast.
Degenerative changes surrounding the pubic articulation can be seen.
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SPINAL METASTASIS
Ivory Vertebra. Diffuse, homogeneous radiopacity of the T10 vertebral body, representing
blastic metastatic disease from carcinoma of the prostate gland.
B. Ivory Vertebra and Mixed Metastasis. homogeneous radiopacity of the L4 vertebral body.
The visualized segments of L3, L5, and the sacrum demonstrate mixed lytic and blastic changes.
A pathologic fracture is present in the L5 vertebral body.
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NOTE:
Sclerosis and enlargement of the pedicle
adjacent to a missing pedicle are positive
radiographic signs of congenital absence
rather than of osteolytic metastatic disease.
Congenital Absence:
Agenesis of the pedicle of L4 (arrow), with
compensatory sclerosis and hypertrophy
of the contralateral pedicle (arrowhead).
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Technetium-99m bone scan of a
patient with prostate cancer
demonstrating abnormal isotope
uptake in multiple skeletal
metastases.
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METASTATIC NEUROBLASTOMA
Striking sunburst spiculation of the skull
tables, along with gross widening of the
sutures and permeative destructive
lesions affecting the calvari(arrowhead).
NOTE:
• Characteristic roentgen appearance
of lytic lesions and diffuse widening
of the sutures.
• A soft tissue mass and striking
sunburst spiculation on the skull
tables are frequently encountered
and virtually pathognomonic of
metastatic neuroblastoma.
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Primitive small and round blue cell tumor possibly
related to primitive neuroectodermal cells.
•The most common primary malignant bone tumor to
metastasize to bone.
•Fourth most common primary malignant bone tumor.
•Most cases occur in the 10- to 25-year age range; rare <
5 years and > 30 years.
•Classic presentation is a diaphyseal, permeative lesion
with a delicate onion skin or peel periosteal response.
•Cortical saucerization is a characteristic sign.
EWING’S SARCOMA
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EWING’S SARCOMA: CORTICAL
SAUCERIZATION AND ONION SKIN
PERIOSTEAL RESPONSE.
Permeative destruction within the
medullary portion of the mid-diaphysis
of the femur.
Disruption in the cortex, creating a
saucerization appearance, is
characteristic of Ewing’s sarcoma .
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EWING’S SARCOMA:
GROOMED OR TRIMMED
WHISKERS EFFECT.
A fine and delicate periosteal
pattern of new bone formation
is present around the
metaphysis and diaphysis of the
first metacarpal bone, causing
the groomed or trimmed
whiskers appearance (arrows).
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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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METASTASIS TO BONE.
Aggressive permeative
pattern of bone destruction
present throughout the
distal phalanx of the index
finger.
CHEST: CANNONBALL
METASTASES
Cannonball metastases,
with sarcomatous growth
within the lung
parenchyma
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CHORDOMA
A rare primary malignant bone
tumor arising from the vestigial
remnants of the notochord.
Chordoma affects males more
commonly than females in a
ratio of approximately 2:1
Lytic destruction, along with a
soft tissue mass, is the most
common roentgen sign.
This destruction occurs in the
sacral or base of skull area.
Age 40 -70 years
Location Features
Sacrococcygeal (50%) Soft tissue mass
Clivus (35%)
Matrix calcification
(50%)
Vertebrae (15%) Ivory vertebra (rarely)
Majority central
Lytic destruction
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SACROCOCCYGEAL CHORDOMA:
Grossly destructive lesion in the midportion of the sacrum.
Flocculent calcification is scattered throughout the tumor matrix.
NOTE: Amorphous calcification of the tumor mass may be noted on plain films in approximately
50% of chordomas.
SPHENO-OCCIPITAL CHORDOMA:
Extensive destruction in the area of the clivus. Considerable flocculent calcification is present
within the destructive soft tissue mass
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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
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HODGKIN’S LYMPHOMA OF BONE
• Hodgkin’s lymphoma of bone may occur as a
secondary manifestation of systemic Hodgkin’s
lymphoma or, rarely, as a primary bone lesion in
the absence of nodal involvement.
• The lytic lesions are usually more symptomatic
than the sclerotic ones.
Location: The primary site of skeletal(lower thoracic
and upper lumbar spine) involvement in Hodgkin’s
lymphoma is the vertebral body.
• Other bones may be involved, such as
the innominate, scapula, sternum, ribs, and femur.
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There is scalloping of the anterior vertebral body (arrows). Diffuse radiopacity
throughout the entire vertebral body (ivory vertebra).
NOTE:
• The anterior and lateral scalloping of the vertebral body is characteristic in
Hodgkin’s lymphoma of the spine.
• When this radiographic sign is present in an ivory vertebra, it inevitably eliminates
Paget’s disease and osteoblastic metastatic disease from the differential diagnosis.
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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.
• 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 decade.
• Adamantinoma may present as a solitary focus
or multicentric lucencies or slightly expansile
osteolytic lesion.
• Lesions tend to have an eccentric epicenter
and a lack of periosteal reaction.
• Long-standing tumors produce marked cortical
thickening and spool-shaped bulges of the
outer cortex in an eggshell fashion.
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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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Notas do Editor
Nonaggressive vs aggressive pattern on radiograph is
not always equivalent to benign vs malignant, e.g.
osteomyelitis can have an aggressive pattern on
radiograph.
Any skeletal lesion detected on well penetrated
radiograph rule out infection, eosinophilic granuloma,
subchondral degenerative cyst, lesion related to
metabolic bone disease such as brown tumor, posttraumatic
changes such as stress fracture or is it normal
variant.
IT IS AN ASSESSMENT OF THE BIOLOGICAL ACTIVITY
GEOGRAPHIC OR TYPE 1 (LODWIG)
EX: CHONDROBLASTOMA, BONE CYST
TYPE 1B:
BIOLOGIC ACTIVITY IS SLIGHTLY MORE: NO SCLEROTIC RIM
EX: GIANT CELL TUMOR
EX: CHONDROSARCOMA, FIBROSARCOMA
Typically metaphyseal in location.
More than half occur around the knee.
Typically affects patients in their second or third decade, however, there is a second peak in patients >60 years old ( 1-30 age group ).
Male to female ratio is 2:1.
Plain films reveal permeative lesion with cortical destruction. “Codman’s triangle” of bone appears as tumor elevates periosteum from underlying bone.
Cortical soft tissue extension may produce radiating spicules of bone called “sunray” appearance.
Associations - Irradiation, Pagets disease, Rothmund-Thompson syndrome
The cleavage plane referred to previously actually represents a periosteal fibrous tissue layer separating the tumor from the cortical surface. This has also been referred to as the string sign of parosteal sarcoma and is present in only 30% of cases.
Bone scintigraphy and (B) sagittal T2-weighted
MR images showing a large primary osteosarcoma of the distal
femur with multiple proximal skip metastases. Although MR imaging contributes little to the imaging
diagnosis, it is mandatory for local surgical staging. It
accurately defines the extent of the tumour within bone,
in the soft tissues and any involvement of the adjacent
joint and neurovascular structures. It is essential to have
a least one MR sequence of the whole affected bone to
confirm or exclude skip metastases (Fig. 48-15). These
are foci of tumour, seen in approximately 5% of cases,
arising within the same bone but separate from the main
tumour or, on occasion, across the adjacent joint (transarticular
skip metastasis). Static sequences following the
intravenous injection of a gadolinium chelate do not contribute
to local staging. However, sequential dynamic
contrast-enhanced sequences obtained pre- and postadjuvant
chemotherapy can be used to assess tumour
response to therapy. FDG-PET has a complimentary
role to other imaging in the initial staging of osteosarcoma
but has an established role in the evaluation of
response to adjuvant chemotherapy and for recurrent
disease, both locally and remote.