Osteosarcomas
●2nd most common primary bone tumor aftermultiple myeloma
●Male predominance
●Serum alkaline phosphatase (ALP) may be raised
Location
●metadiaphysis of tubular bones in the appendicular skeleton.
Common sites include:
●femur: MC
●tibia
●humerus
●fibula
●innominate bone (i.e. os coxae)
●mandible (gnathic osteosarcoma)
●maxilla
●vertebrae
Primary osteosarcoma:
●in young patients (10-20 years)
●with 75% taking place before the age of 20 because the growth
centers of the bone are more active during adolescence
Secondary osteosarcoma:
●occurs in the elderly
●usually secondary to malignant degeneration ofPaget disease,
extensivebone infarcts, post-radiotherapy for other
conditions,osteochondroma, andosteoblastoma
SUBTYPES
●intramedullary:~80%
○conventional high-grade: most common and discussed in this article
○telangiectatic osteosarcoma
○low-grade osteosarcoma
●surface or juxtacortical:~10-15%
○intracortical osteosarcoma
○parosteal osteosarcoma
○periosteal osteosarcoma
●extraskeletal:~5%
○extraskeletal osteosarcoma
Associations
●Paget disease of bone
●irradiation
●fibrous dysplasia(more common in the polyostotic form)
●Mazabraud syndrome(rare)
CONVENTIONAL HIGH -GRADE
OSTEOSARCOMA
Plain radiograph
Typical appearances of conventional high-grade osteosarcoma include:
●medullary and cortical bone destruction
●wide zone of transition,permeativeor moth-eaten appearance
●aggressiveperiosteal reaction
○sunbursttype
○Codman triangle
○lamellated (onion skin) reaction: less frequently seen
●soft-tissue mass
●tumor matrix ossification/calcification
○variable:reflects a combination of the amount of tumor bone production,
calcified matrix, and osteoid
○ill-defined "fluffy" or "cloud-like" compared to therings and arcsof chondroid
lesions
An ill-defined sclerotic area affecting the metaphysis of distal
fibula with an aggressive periosteal reaction (sunburst type).
This lesion involves the adjacent posterior aspect of the tibia
and the calcaneum inferiorly.
It is associated with a moderately large soft tissue swelling.
The distal half of the femur is occupied and expanded with a heterogeneous mass with
areas of bone formation. Posterior the periosteum is elevated (Codman's triangle).
The role of CT
●in assisting biopsy and staging.
●CT adds little to plain radiography and MRI in the direct
assessment of the tumor.
●The exception to this rule is predominantly lytic lesions in
which small amounts of mineralized material may be
inapparent on both plain film and MRI.
MRI
●Preoperative evaluation and staging of osteosarcoma
●To determine accurate local staging and assessment for limb-
sparing resection, particularly for evaluation of intraosseous
tumor extension and soft-tissue involvement.
●Evaluation of the growth plate -up to 75-88% of metaphyseal
tumors do cross the growth plate into the epiphysis
MRI
○soft tissue non-mineralized component: iso in T1 & hyper in T2
○mineralized/ossified components: low signal intensity in T1 & T2
○peritumoral edema: intermediate signal intensity
○scattered regions of hemorrhage will have a variable signal
○enhancement: solid components enhance
●T1 -Low signal , with small high signal foci likely hemorrhage
●T2 -heterogenous signal
●STIR -hyper
●Enhancement ( + ).
●There is associated cortical destruction, extraosseous soft
tissue extension, periosteal infiltration and marrow edema and
microinvasion of the adjacent area of distal femur.
T1
T2 FS, STIR
At bone scintigraphy, marked uptake of radiotracer is seen on blood flow, blood pool, and
delayed images
In evaluating for distant metastases (Both osseous and extraosseous metastatic disease)
Differential considerations include:
●Osteomyelitis
●other tumors
○metastatic lesion to bone
○Ewing sarcoma
○aneurysmal bone cyst
When the lesion is at the posteromedial distal femur consider:
●cortical desmoid
On MR images, joint involvement is
suggested when
●the hyaline cartilage is penetrated or
●more commonly when tumor extends through the capsule,
(such as into the suprapatellar bursa anteriorly or posteriorly
to encompass the cruciate ligaments )
●Fat-suppressed T1 + C -for delineating extension of tumor
into the joint
Enhancing synovium may mimic tumor spread.
Although invasion of the joint is unlikely in the absence of an
effusion, the presence of an effusion does not allow an accurate
prediction of intraarticular invasion.
Skip metastases
●Foci of tumor within the marrow of the affected
bone that are distinctly separate from the primary
lesion
Importance
●For defining the extent of disease
●Prognostical significance since the decrement in 5-
year survival for patients with skip metastases is
similar to that for patients with distant metastatic
disease.
Skip metastases are best identified with MR imaging, and
a study of the entire length of an affected bone should be
performed at the time of primary evaluation
Telangiectatic Osteosarcoma
●is an uncommon histopathologic subtype
●The term Malignant bone aneurysmis to describe a
hemorrhagic, poorly ossified telangiectatic osteosarcoma.
●Characterized by a malignant osteoid-forming sarcoma of
bone with large blood-filled vascular channels.
●Geographic bone destruction with a wide zone of
transition
●Marked aneurysmal expansion of bone is frequent
●Metaphyseal lesions often extend into the epiphysis
●Aggressive periosteal reaction, cortical destruction,
associated soft-tissue mass, and pathologic fracture
The cystic consistency of telangiectatic osteosarcoma is
reflected by its radiologic appearance.
●Bone scintigraphy -peripheral increased radionuclide
uptake with central photopenia (donut sign).
●CT -attenuation of the central portion of the lesion is often
lower than that of muscle
●This central region also shows hyperintense signal on
T2W . At MR imaging, hemorrhage is frequently observed
as areas of high signal intensity
●Fluid-fluid levels may be seen on CT or MR
Expansile and mostly cystic extraosseous mass with
multiple fluid levels
Radiologic differential diagnoses
●ABC
●giant cell tumor (GCT) of bone
●metastases
DIFFERENCES B/W
Telangiectatic Osteosarcoma
●Nodular septal thickening with viable tissue
showing enhancement
●Osteoid matrix mineralization in a soft-tissue
mass
●Cortical destruction and infiltrative margins -
aggressive growth pattern
Aneurysmal bone cyst
●Only an enhancing thin peripheral rim
and septa without nodularity or
osteoid matrix mineralization.
●The pattern of growth in ABC -less
aggressive, with expansile remodeling
and a well-defined encapsulated
margin
Telangiectatic OS GCT
●located at the end of the bone, close
to the subchondral bone
●is a solid mass
●Metaphyseal lesion
●Entirely cystic appearance
Treatment
●is similar to that of conventional
●Chemotherapy followed by
wide surgical resection / limb salvage or amputation
Low-Grade Intraosseous Osteosarcoma
●Within the medullary canal of the distal femur and proximal tibia
●The lesion may show well-defined margins, a sclerotic rim, prominent internal
trabecubation, and diffuse sclerosis, and it may cause expansile remodeling of
bone -simulate a benign process
●Prognosis is substantially better than that for patients with conventional OS
●Aggressive features such as cortical or medullary bone destruction and an
extraosseous mass suggest the malignant nature, even if it is focal and subtle
The most common radiographic
appearance
●Well-defined margins
●Expansile lytic bone destruction with coarsely thick or thin
incomplete trabeculation
●A dense sclerotic pattern is less common
●Cortical disruption and soft-tissue extension
●Expansile remodeling of bone
Differential radiologic diagnoses
Benign fibro-osseous lesions
●fibrous dysplasia
●nonossifying fibroma
●desmoplastic fibroma
●The presence of aggressive imaging features such as cortical destruction,
soft-tissue extension, and periosteal reaction is a helpful clue for
differentiation of low-grade central OS from the above benign lesions
Small Cell Osteosarcoma
●The pathologic characteristics of this tumor are similar to
those of Ewing sarcoma: Both lesions are composed of small
round blue cells
●Predominantly permeative
●lytic medullary lesion
●cortical breakthrough
●aggressive periosteal reaction,
Osteosarcomatosis
●also known as multifocal osteosarcoma or multiple sclerotic
osteosarcoma
●represent rapidly progressive metastatic disease
●Younger, skeletally immatune patients -have rapidly
appearing, usually symmetric, sclerotic lesions
●Older patients -fewer, asymmetric sclerotic lesions.
●Ill-defined margins
●Aggressive periosteal reaction
●Cortical disruption
●Adjacent soft-tissue extension
●Although lesions usually contain cloudlike osteoid, purely lytic
dominant lesions may be seen.
●In contrast to the dominant lesions, the secondary foci are often
smaller, more sclerotic, and better defined and lack periosteal
reaction or cortical destruction
Juxtacortical or surface OS
●Refers to OS originating from the surface of bone.
●It is primarily associated with the periosteum, with variable
medullary canal involvement.
SUBTYPES
●Parosteal
●Periosteal
●High-grade surface OS
Parosteal OS
●MC type of juxtacortical OS
●Metaphysesof long bones
●Posterior aspect of the distal femur is the most frequent site
●Parosteal OS originates from the outer fibrous layer of the
periosteum
●Lobulated and exophytic mass with central dense ossification
adjacent to the bone
●A cleavage plane seen separating the tumor and adjacent
normal cortex (THE STRING SIGN). This cleavage plane
corresponds histologically to the periosteum interposed
between the cortex and the tumor mass
●Dedifferentiation correlates radiographically with increased
lysis and the presence of a soft-tissue mass without
ossification
Differential diagnosis
benign entities such as
●osteochondroma,
●myositis ossificans,
●periosteal chondroma
malignant entities such as
●fibrous malignancy
●periosteal chondrosarcoma
●other subtypes of juxtacortical OS
D/D
Parosteal OS
●Lacks corticomedullary
continuity
●The cartilage cap -
irregular, incomplete, and
thick
Osteochondroma
●corticomedullary continuity
●The cartilage cap -high
signal on T2-weighted /PD
(high water content)
●The cartilage cap -Smooth,
continuous, and relatively
thin cartilage cap
Parosteal OS
●The ossification pattern -
Radiographic inverse of that seen in
myositis ossificans
( with the densest ossification in the
center of the lesion and the least
radiopaque bone at the periphery)
Myositis ossificans
●The ossification pattern -Gradual
ossification of the lesion from the
periphery toward the center of the
mass
●Prominent surrounding edema
Periosteal OS
●2
nd
MC type of juxtacortical OS
●along the diaphyses of long bones
●most commonly the tibia
●Periosteal OS arises from the inner, germinative layer of
periosteum
Common radiographic findings
●soft-tissue mass with periosteal reaction -Periosteal reaction
extends perpendicularly from the inner cortex to the outer
margin of the tumor
●cortical erosion
●cortical thickening
XRAY
●Lesion in the femoral diaphysis with a
Codman triangle with perpendicular
periosteal reaction
CT & MR
●The predominantly chondroid matrix -results in a lesion that is
low in attenuation on CT images
●Hyperintense on T2-weighted MR images with smaller foci of
low signal intensity representing calcified matrix or hair-on-
end periosteal reaction.
Difference b/w
Parosteal OS
●densely ossified juxtacortical mass
that lies outside the cortex
●occurs in metaphyses
Periosteal OS
●More lytic in appearance, causing
cortical erosion and periosteal
reaction
●occurs in diaphyses
High-Grade Surface Osteosarcoma
●2nd and 3rd decades of life
●the diaphyses and metaphyses of long bones
●the femur being the most common site
Radiographic findings
●dense ossification and periosteal reaction
●Cortical erosion and thickening
●surrounds the femur circumferentially and invades the medullary canal
focally
D/D
●parosteal OS
●periosteal OS
●conventional OS.
Similarity & difference B/W
Periosteal OS
●both can occur in diaphyses and
cause periosteal reaction and bone
destruction.
●medullary invasion is less common
High-grade surface OS
●involves the entire circumference of
the cortex and is more likely to show
medullary invasion
EWING SARCOMAS
Ewing sarcomas
●are the 2nd most common malignant primary bone tumors of
childhood afterosteosarcoma
●Typically arising from medullary cavity with invasion of the
Haversian system.
●They usually present as moth-eaten destructive permeative
lucent lesions in the shaft of long bones with large soft tissue
component without osteoid matrix and typical onion skin
periostitis
●10 -20 years of age
●M > F
C/F
●local pain
●Pathological fractures also occur.
●Systemic symptoms including fever may be present. ESR is
also elevated.
Ewing sarcoma family of tumors(ESFT)
●Ewing sarcoma (small round blue cell tumorwith regular
sized primitive appearing cells)
●Soft tissue tumorspPNET
●Askin tumor
●Neuroepithelioma
Location -tumor is almost always metadiaphyseal
or diaphyseal
●lower limb
○femur most common
●pelvis
●upper limb
●spine and ribs
○sacrococcygeal region most common
Plain radiograph and CT
●have clearly aggressive appearance.
Common findings include:
●Wide zone of transition
●Permeative bone destruction
●Laminated (onion skin) periosteal reaction
●Sclerosis may be present
They occasionally demonstrate other appearances, includingCodman
triangles, spiculated (sunburst) or thick periosteal reaction and even
bone expansion or cystic components.
●Soft tissue calcification is uncommon
Permeative lytic bone lesion in the distal fibular diaphysis with aggressive hair-on-
end periosteal reaction and Codman triangles
MRI
●T1:low to intermediate signal
●T1 C+ (Gd):heterogeneous but prominent enhancement
●T2:heterogeneously high signal, may see hair on end low
signal striations
Nuclear medicine
Ewing sarcomas demonstrate increased uptake on both
●Gallium
67
-citrate and
●all three phases of the Technetium
99
m methylene
diphosphonate bone scans.
Mid-diaphyseal left humeral lesion which has a permeative
appearance andlamellated periosteal reaction.
Multiple intramedullary bone lesions involving both
femora and the pelvis bilaterally
.
T2W FS
T1W FS + C
The right iliac bone demonstrates
●ill-defined lytic lesion
●a wide zone of transition
●The medial part of the
acetabular cortex is
poorly seen, potentially
representing extension
into the joint.
Large areas of focal cortical destruction and continuity between the medullary and
soft-tissue components are common
CHONDROSARCOMAS
Chondrosarcomas
●4
th
and 5
th
decades
●M>F
●3
rd
MC malignant tumour
Typical features
●ring-and-arc chondroid matrix mineralization
●lytic pattern
●deep endosteal scalloping
●soft-tissue extension
C/F
●pain
●pathological fracture
●palpable lump or local mass effect
●Hyperglycemiacan occur as aparaneoplastic syndrome.
TYPES
Chondrosarcomas are either
●Primary-arisingde novo
●Secondary -arise from a pre-existent cartilaginous mass
Primary
●conventional intramedullary chondrosarcoma(or central
chondrosarcoma) : low, intermediate or high grade
●juxtacortical chondrosarcoma : low, intermediate or high grade
●clear cell chondrosarcoma
●myxoid chondrosarcoma
●mesenchymal chondrosarcoma
●extraskeletal chondrosarcoma
●dedifferentiated chondrosarcoma
Locations
●long bones: 45% (the reason is that the cartilage is more abundant in the long,
tubular bones)
●pelvis
●ribs
●spine
●scapula
●sternum
●head and neck
●craniofacial
●hands and feet
Depending on their osseous location
●Central -intramedullary
●Peripheral / Juxtacortical (periosteal) lesions
Numerous categories (incorporating both location
and histologic characteristics)
●Conventional intramedullary
●Clear cell
●Juxtacortical
●Myxoid
●Mesenchymal
●Extraskeletal
●Dedifferentiated
Plain radiograph
●lytic
●intralesional calcifications: ~70% (rings and arcs calcification orpopcorn
calcification)
●endosteal scalloping
●moth-eaten appearance orpermeative appearancein higher grade tumors
●cortical remodelling, thickening andperiosteal reactionare also useful in
distinguishing between an enchondroma and low-grade chondrosarcoma
Solitary, expansile, lytic lesion in the right
middle finger metacarpal head.
Minor cortical breach in its inferior medial
aspect.
No periosteal reaction.
Typicalring and arc
calcification
An intramedullary lesion is noted with associated
endosteal scalloping and chondral calcification.
CT
●Matrix calcification
●Endosteal scalloping
●Cortical breach
●Soft tissue mass: tumor cellularity, and therefore density,
increases with increased grade of the tumor
●Heterogenous contrast enhancement
MRI
●T1:low to intermediate signal
●T2:high intensity in non-mineralized/calcified portions
●Gradient :blooming of mineralized/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
Low T1, bright T2
and STIR signal
STIR
Nuclear medicine
●Typically chondrosarcomas demonstrate increased uptake
onbone scan
●This is useful in helping to distinguish a low-grade
chondrosarcoma from an enchondroma as the latter has
increased uptake in ~20% of cases, and usually to a lesser
degree
MULTIPLE MYELOMA
Multiple myeloma
●Most commonprimary malignant bone neoplasmin adults.
●It arises from red marrow due to monoclonal proliferation of
plasma cells
●Manifests in a wide range of radiographic abnormalities.
Four main patterns are recognized:
●Disseminated form: multiple well-defined "punched out" lytic
lesions
●Disseminated form: diffuse skeletal osteopenia
●Solitaryplasmacytoma: a single large/expansile lesion most
commonly in a vertebral body or in the pelvis
●Osteosclerosing myeloma
Clinical presentation
can be recalled with the mnemonicCRAB:
●Calcium elevation
●Renal insufficiency related to multiple myeloma
●Anemia
●Bone abnormalities including lytic lesions and osteopenia
MR imaging patterns of multiple myeloma
infiltration
These include
●Normal marrow
●A micronodular pattern (also termed variegated or salt-and-
pepper)
●A focal pattern
●A diffuse pattern
Durie and Salmon Plus System for Staging of
Multiple Myeloma
Radiographic features
●numerous, well-circumscribed, lytic bone lesions
○punched out lucencies (Raindrop skull )
○endosteal scalloping
●generalized osteopenia (less common)
○often associated withvertebral compression fractures / vertebra
plana
Multiple foci ofendosteal scallopingwithin this long bone, consistent
with lytic myelomatous lesions
Xray
Askeletal surveyis essential
Bilateral AP and PA projections of
●Femur, pelvis, spine and skull
●Hands
●Forearms
●Humerus
●Feet
●Leg
MRI
Five patterns have been described:
●normalbone marrowsignal
●diffuse involvement
●focal involvement
●combined diffuse and focal involvement
●variegated ("salt and pepper")
MRI
●T1 -low signal
●T2 withfat-suppression -high signal
○infiltration of the ribs is probably best appreciated on T2 images
with fat suppression, appearing bright -‘white ribs sign’
●T1 C+ (Gd) –
■type 4 curve -asteep wash-inof contrast medium, due to the
high vascularization, followed by anearly wash-out
●Sclerosis is usually seen only with treatment or fracture
○Bone scans may typically be negative and many hotareas on scan may be
healing fractures
○Solitary plasmacytoma produces “soap-bubbly” expansile, septated lesion,
when characteristic
Diff b/w
MM
●Vertebral body is the MC site
●Involve mandible, distal axial skeleton
●May have normal appearance in bone
scan
METS
●more commonly affect the vertebral
pedicles rather than vertebral bodies
●Rare
●extensive bony metastases rarely
have a normal appearance in bone
scan
Oil-droplet appearance.
●Metastases are generally
fewer in number and of
different sizes and shapes.
D/D:
●mets and disuse osteoporosis
METASTASIS
METASTASIS
●Most common malignancy in bone.
●Must be considered in the differential diagnosis of any bone
lesion in a patient > 40 years.
●May present as well-defined osteolytic, ill-defined osteolytic
and also as sclerotic bone lesion.
●Majority of osteolytic metastases
LOCATION
●vertebrae
○especially the posterior vertebral body, extending into the pedicles
○pelvis
●proximalfemur
●proximalhumerus
●skull
General rule
●Unlike primary bone tumors, metastases incite no or only
limitedperiosteal reaction.
●The occasional exception to this general rule
includesprostate cancer, some gastrointestinal
malignancies,retinoblastomaandneuroblastoma.
Lytic bone metastases( more common)
●thyroid cancer
●renal cell cancer
●adrenal gland carcinomaandpheochromocytoma
●uterine carcinoma
●gastrointestinal carcinomas
●Wilms tumor
●Ewing sarcoma
●melanoma
●hepatocellular carcinoma
●squamous cell carcinoma of the skin
●lung cancer
●Two geographical areas of osteolytic mets
involving left iliac bone with gross thinning of
bony cortex and cortical breach.
Scleroticorblasticbone metastases
●prostate carcinoma(most common)
●breast carcinoma(may bemixed)
●transitional cell carcinoma (TCC)
●carcinoid
●medulloblastoma
●neuroblastoma
●mucinous adenocarcinoma of the gastrointestinal tract (e.g.colon carcinoma,gastric carcinoma)
●lymphoma
●small cell lung cancer
●post-treatment appearance of any lytic metastasis
●pulmonary adenocarcinoma
●medullary thyroid carcinoma
Osteoblastic mets from Ca Prostate
sclerotic metastasesfrom neuroblastoma
Mixed lytic and sclerotic bone
metastases
●breast carcinoma
●lung carcinoma
●carcinoma of the cervix
●testicular tumors
●prostate carcinoma: typically sclerotic but 15% are mixed
●ganglioneuroblastoma
Mixed lytic and sclerotic vertebral
metastatic lesions
Bone scans
●are the most sensitive routine imaging modality to identify
both sclerotic and lytic lesions
●Main differential ismultiple myeloma.
OSSEOUS LYMPHOMA
Primary bone lymphoma
●“lymphoma presenting in an osseous site with no evidence of
disease elsewhere for at least 6 months after diagnosis
●Lytic-Destructive Pattern -MC
●Blastic-Sclerotic Pattern -rare in primary bone lymphoma
compared with metastatic bone lymphoma
●Subtle or “Near-Normal” Findings
Lytic permeative pattern
After irradiation and combination
chemotherapy shows sclerotic
changes
Radiographic features
●solitary lytic lesion near the end of a long bone
●permeative or moth-eaten pattern of destruction
●aggressive periosteal reaction
MR Imaging Characteristics
1.Bone Marrow Replacement
●T1 -are the best for demonstrating marrow changes ( as T1-
weighted images reveal areas of low signal intensity within the
marrow).
●On T2 -these areas generally appear bright.
●Peritumoral edema & reactive marrow changes-hyper on T2
.
●However, if fibrosis is present -it may show low signal
intensity
●PC -areas of enhancement within the lesion.
2. Soft-Tissue Involvement
●Cortical break with soft tissue masses on MR images.
●Extensive marrow disease and surrounding soft-tissue
masses but without extensive cortical destruction has also
been reported .
(One explanation for this finding is the spread of tumor cells from
the marrow through small vascular channels that run through the
cortex into the surrounding soft tissue )
3. Cortical Erosion
●Both MR imaging and CT demonstrate cortical erosion,
although the former permits early detection
T1W T2W
Analysis of the biopsy specimen revealed primary Hodgkin
lymphoma of the bone.