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BONE FORMING
TUMOURS &
FIBROUS
DYSPLASIA
Presented by: ARUN KUMAR
Moderator: Dr. SHIKHA PRAKASH
NORMAL BONE
Parts of bones
1. Epiphysis : ends of bone
2. Diaphysis : shaft
3. Metaphysis : epiphysial end of
diaphysis
Macroscopically the architecture of bone
may be
1. Compact
2. cancellous/spongy/trabeculae
NORMAL BONE
On longitudinal
section the bone is
composed of :
1. Periosteum
2. Cortex
3. endosteum
4. Medullary cavity
 Unmineralised matrix laid down by osteoblasts
 Stains homogenously pink on H&E
 On polarized light demonstrates criss cross or
woven pattern of collagen fibers
 Can be confused with fibrin, collagen or
chondroid.
OSTEOID
NORMAL BONE
Woven bone : In these, collagen is deposited in a
random weave manner.
Lamellar bone : Collagen is deposited in a order. It
replaces woven bone during growth ,deposited much
slowly & stronger than woven bone
Woven bone (top) deposited on the surface of
preexisting lamellar bone(bottom)
WHO CLASSIFICATION OF
BONE TUMOURS
Histologic Type Benign Malignant
Chondrogenic (22%) Osteochondroma Chondrosarcoma
Chondroma
Dedifferentiated
chondrosarcoma
Chondroblastoma
Mesenchymal
chondrosarcoma
Chondromyxoid
fibroma
clear cell
chondrosarcoma
Osteogenic (19%) Osteoid osteoma Osteosarcoma
Osteoblastoma
Fibrogenic
Desmoplastic
fibroma
notochordal tumors Chordoma
Histiocytic origin
Benign Fibrous
histiocytoma
Malignant fibrous
histiocytoma
Ewing’s
sarcoma/PNET
Giant cell tumor
Malignant Giant cell
tumor
Hematopoietic (40%) Myeloma
Malignant lymphoma
Joint lesions
Synovial
chondromatosis
Vascular Hemangioma
Hemangioendotheliom
a
Hemangiopericytoma,
angiosarcoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma
Smooth muscle tumors Leiomyoma Leiomyosarcoma
Miscellaneous tumors Adamantinoma
Metastatic malignancy
Tumours of
unidentified neoplastic
nature
Aneurysmal bone cyst
Simple cyst
Fibrous dysplasia
Langerhan’s cell
histiocytosis
Chest wall hamartoma
Osteofibrous dysplasia
BONE FORMING TUMOURS
1. OSTEOID OSTEOMA
2. OSTEOBLASTOMA
3. OSTEOSARCOMA
 CONVENTIONAL OS
 TELANGIECTATIC OS
 SMALL CELL OS
 LOW GRADE CENTRAL OS
 SECONDARY OS
 PAROSTEAL OS
 PERIOSTEAL OS
 HIGH GRADE SURFACE OS
OSTEOID
OSTEOMA
OSTEOID OSTEOMA
 Benign lesion characterized by less than 2 cm pea like
mass of abnormal bone-nidus
 Limited growth potential
 Clinical features
1. children & adolescents, occasionally in older individuals
2. more common in males
3. any bone except sternum.
1. most common in the long bones (metaphysis or shaft).
2. The tumour tend to involve the cortex rather than medulla
OSTEOID OSTEOMA
 Signs & symptoms:
1. Pain-characteristically relieved by salicylates and
NSAIDS
2. Swelling & redness
3. Joint effusion
4. Scoliosis
5. Bone growth abnormalities
 Genetics : involvement of 22q13 & loss of 17q
OSTEOID OSTEOMA:
RADIOLOGIC FINDINGS
 X-RAY : Dense
cortical sclerosis
surrounding a
radiolucent nidus,
 CT SCAN : best
imaging study to
demonstrate OO, help
OSTEOID OSTEOMA:
RADIOLOGIC FINDINGS
 MRI SCAN: Useful in demonstrating medullary
or periarticular lesions and peri-tumoural oedema,
also help to localize the lesion
BONE SCAN: associated with hot bone scan,
helpful in localizing the lesion
OSTEOID OSTEOMA : GROSS
FINDINGS
Small , cortically
based, red, gritty
or granular round
lesion
surrounded (well
circumscribed)
by ivory white
sclerotic bone
MICROSCOPIC FINDINGS
 The tumour consists of a central area of
vascularised connective tissue within which
differentiating osteoblasts are engaged in the
production of osteoid and sometime bone
 if actual bone is present osteoclast may also
be seen engaged in remodelling
OSTEOID OSTEOMA
Low power
view
showing
wedge
shaped
nidus
protruding
slightly
above the
surface and
surrounded
by sclerotic
bone
Central nidus with
dense sclerotic
bone
Osteoid osteoma shows anastomosing irregular
bony trabeculae with osteoblastic rimming
embedded in a hypocellular fibrovascular stroma
PROGNOSIS
 Prognosis is excellent
 Recurrence is rare
DIFFERENTIAL DIAGNOSIS
 SOLITARY ENOSTOSIS
 OSTEOMYELITIS
 BRODIE’S ABSCESS
 OSTEOBLASTOMA
 OSTEOSARCOMA
Osteoid osteoma v/s Solitary
enostosis
SOLITARY
ENOSTOSIS : does
not cause pain or
host bone sclerosis.
Usually consists of
lamellar bone with
haversian systems
Osteoid osteoma v/s
Osteomyelitis
Chr osteomyelitis : showing necrotic bone,chr
inflammation & fibrosis
Osteoid osteoma v/s Brodie’s
abscess
Image shows fibrosis , degenerating bony spicules, &
subacute inflammation
OSTEOBLASTOMA
OSTEOBLASTOMA
 A rare benign tumor, usually larger than 2 cm,
characterized by osteoid and woven bone
production.
 Age: 10-30 yrs
 M:F is 2.5:1
 Skeletal distribution: predilection for spine
particularly posterior elements and sacrum, others-
long bones-proximal and distal femur, proximal
tibia
 Site: majority intra-osseus but a small percentage
can occur on the surface of the bone in a
OSTEOBLASTOMA
Symptoms: dull, aching, nocturnal pain not relieved
by aspirin
Genetics : 1. chromosomal rearrangements have
been described in 4 cases, with chr no. ranging
from hypodiploid to hyperdiploid.
2. MDM2 amplification
3. TP53 deletion in aggressive
osteoblastoma
OSTEOBLASTOMA:
RADILOGICAL FINDINGS
 SPINAL LESION
1. Main mass centered in
vertebral arch
2. Round to ovoid lytic lesion
with bone expansion
3. Periosteal new bone
formation
4. Intra-lesional bone forming
X-ray showing expansion of the left fourth lumbar pedicle :
transeverse process and superior facet
OSTEOBLASTOMA: GROSS
FINDINGS
 Lesion is well
demarcated,
 Round to oval with thin
cortex
 Mostly granular, gritty, &
deep red on cutting
 Whitish yellow if bone
Osteoblastoma: excised specimen of femoral lesion
showing a well demarcated fleshy tumour
OSTEOBLASTOMA:
MICROSCOPIC FINDINGS
 Tumour is composed of woven bone spicules
or trabeculae which are arranged haphazardly
& lined by single layer of osteoblast
 Rich vascularity
 Osteoblast may have mitosis but not atypical
 Scattered multinucleated giant cells often
present
OSTEOBLASTOMA:
MICROSCOPIC FINDINGS
 Tumour has a pushing border
 Secondary ABC like changes may be seen
 Some osteoblastomas show multiple small
nidi
Osteoblastoma shows trabeculae of woven bone
lined by layer of polygonal osteoblast and the well
vascularised inter-trabecular stroma
OSTEOBLASTOMA:
MICROSCOPIC FINDINGS
Malignant osteoblastoma : histologically same but are
more cellular, has large no. of giant cells & large amount
of spiculated blue bone.
locally aggressive & does not develop distant
metastasis
Aggressive osteoblastoma : characterised by presence
of epitheloid osteoblasts, trabecular or sheet-like osteoid &
osteosclerotic resorption. locally aggressive & does not
develop distant metastasis
Pseudomalignant osetoblastoma : tumor cells have
hyperchromatic nuclei. However, the nuclei have the
Osteoblastoma : prominent epithiloid appearance
OSTEOBLASTOMA :
PROGNOSIS
Prognosis is excellent
Recurrence is unusual and may occur in a bone
which has difficult surgical access
OSTEOBLASTOMA:D/D
1.Osteoid osteoma
2.Osteosarcoma
3.Giant cell tumor
4.Aneurysmal bone cyst
0steoid osteoma osteoblastoma
1. involvement Every bone except
sternum
Predilection for spine
2. size < 2cm > 2cm
3. pain Characteristically
relieved by NSAIDs
Not relieved by
NSAIDs
3. imaging Dense, cortical
sclerosis surrounding
a radiolucent nidus
Lytic, well
circumscribed ,
confined by a
periosteal shell of
reactive bone
4. gross Gritty or granular
round lesion
surrounded by ivory
white sclerotic bone
Round to oval, thinned
cortex & periosteal
reactive bone
5. microscopy Central nidus with
dense sclerotic bone
Composed of woven
bone trabeculae
arranged haphazardly
Osteoblastoma v/s GCT
GIANT CELL
TUMOR: located
in epiphysis,
packed with giant
cells and stromal
cells
OSTEOBLASTOMA: D/D
ABC Osteoblastoma
1. Age First 2 decade 10-30yr
2. Sex No sex predilection M>F
3. Site Metaphysial Majority intraosseus
4. Gross Well demarcated,
multiloculated mass of
blood filled cystic
spaces separated by
tan white gritty septa
Well demarcated,
round to oval.
Granular, gritty & deep
red on cutting
5. Microscopy
6. Recurrence
Well circumscribed,
blood filled cystic
spaces, separated by
fibrous septa rich in
collagen producing
fibroblasts & callus like
tissue
Tumor composed of
haphazardly arranged
woven bone spicules
or trabeculae
Unusual
Osteoblastoma v/s ABC
ABC with blood filled cystic space surrounded by wall containing
proliferating fibroblasts, reactive woven bone, & osteoclast type
giant cells
OSTEOSARCOMA
OSTEOSARCOMAS
 Osteosarcomas is a mesenchymally derived malignant
tumor that produces osteoid and/or bone.
 They can be:
1. INTRAMEDULLARY OS
2. INTRACORTICAL OS
3. JUXTACORTICAL OS
OSTEOSARCOMA
 PRIMARY OSTEOSARCOMA
1. CONVENTIONAL OSTEOSARCOMA
a) Osteobalstic
b) Chondroblastic
c) Fibroblastic
2. SMALL CELL OSTEOSARCOMA
3. TELANGIECTATIC OSTEOSARCOMA
4. LOW GRADE CENTRAL OSTEOSARCOMA
OSTEOSARCOMA
5. SURFACE OSTEOSARCOMA
a) Parosteal osteosarcoma
b) Periosteal osteosarcoma
c) High grade surface osteosarcoma
 SECONDARY OSTEOSARCOMA
1. Osteosarcoma in paget’s disease
2. Postirradiation osteosarcoma
3. Osteosarcoma in other benign precursors
age/sex site c/f prognosis
Convention
al OS
2nd decade
M>F
Metaphysis
(91%) of
long bones
Pain,swellin
g,path #
If untreated
fatal
Telangiectat
ic OS
(<4%)
2nd decade
M>F
Metaphysis
of long
bones
Pain,swellin
g ,path #
As above
Small cell
OS(1.5%)
2nd decade,
slightly mc
in female
>50%
metaphysis
of long
bones
Pain,swellin
g
Slightly
worse than
convention
al OS
Low grade
central
OS(1-2%)
2nd-3rd ,
M=F
80% in long
bones
Pain,swellin
g
excellent
age/sex site c/f prognosis
OS sec. to
paget ds
65yr
M>F,2:1
Long
bones,
pelvis, skull
Pain,
swelling,
path#
Poor
Post-
radiation
OS (3.4%-
5%)
Children
are at
greatest
risk
Any bone,
mc in pelvis
& shoulder
region
Pain,
swelling
5yr survival
rate for
extremity
lesion is
68.2% &
axial lesion
27.3%
Parosteal
OS
Young
adult, slight
female
predominan
ce
Long bones Pain,
swelling,
inability to
flex the
knee
excellent
age/sex site c/f prognosis
Periosteal
OS
2nd-3rd
decade
Slightly mc
in male
Diaphysial
or dia-meta
region of
long bones
Painless
swelling
initially,
later pain
excellent
High grade
surface OS
2nd decade,
slightly mc
in male
Long bones Mass &/or
pain
Depend
upon
response to
chemothera
py
CONVENTIONAL OSTEOSARCOMA
CONVENTIONAL
OS:RADIOLOGIC FINDINGS
 Metaphysial location
 It may be pure oseoblastic or
osteolytic. In most cases it is mixed
lytic/blastic lesion accompanied by
cortical destruction and extension
into soft tissue
 Tumours tend to be eccentric.
Mixed blastic/lytic lesion
involving the femoral metaphys
CONVENTIONAL
OS:RADIOLOGIC FINDINGS
 Periosteal reaction:
1. Codman’s triangle
2. Longitudinal laminations
3. Perpendicular spiculations
 Soft tissue mass
CONVENTIONAL OS :GROSS
FINDINGS
 OS often a large (over
5cm), metaphysial centered , fleshy
or hard tumour which may contain
cartilage
 It frequently transgresses the
cortex & is associated with a soft
tissue mass
CONVENTIONAL OS
:MICROSCOPIC FINDINGS
1. It is highly anaplastic , pleomorphic tumor.
2. The tumour cells may be epithelioid ,plasmacytoid ,
fusiform, ovoid, small round cells, clear cells, giant
cells or spindle cells. Most cases are mixture of 2 or
more of these cell types.
3. Osteoid and/or bone production by tumor cells.
dense, pink, amorphous,curvilinear with small
nubs,arborisation,abortive lacunae formation,
intercellular material.
On the basis of production of predominant matrix
by tumors the conventional OS is subdivided into:
1. OSTEOBLASTIC (50%)
2. CHONDROBLASTIC (25%)
3. FIBROGENIC (25%)
OSTEOBLASTIC
OSTEOSARCOMA
1. Bone &/or osteoid are
the predominant
matrix
2. The matrix may be
thin, arborising
osteoid (i.e.,
filigree) to dense
compact osteoid &
Osteoblastic OS: showing
osteoid
OSTEOBLASTIC
OSTEOSARCOMA
Filigree osteoid
comprises thin,
randomly arborizing
lines of osteoid
interweaving b/w
neoplastic cells
Flat and thick osteoid
CHONDROBLASTIC
OSTEOSARCOMA
 Chondroid matrix is predominant.
 It tends to be high grade hyaline cartilage.
 Myxoid and other forms of cartilage are
uncommon, except in the jaws & pelvis.
 Osteoid is seen between spindle cells or in center of
chondroid lobules.
CHONDROBLASTIC OSTEOSARCOMA
Lobules of malignant cartilage. There is spindling at the
periphery and osteoid formation.
FIBROBLASTIC
OSTEOSARCOMA
 25% of all conventional osteosarcoma
 A high grade spindle cell malignancy
 Minimal matrix production
Unusual forms of osteosarcoma
1.Epitheloid osteosarcoma
2.Ostoblastic osteosarcoma – sclerosing type
3.Osteosarcoma resembling osteoblastoma
4.Chondromyxoid fibroma-like osteosarcoma
5.Chondroblastoma-like osteosarcoma
6.Clear cell osteosarcoma
7.Malignant fibrous histiocytoma-like osteosarcoma
8.Giant cell rich osteosarcoma
Not associated with specific biologic behaviour that
differs from conventional osteosarcoma.
TELANGIECTATIC
OSTEOSARCOMA
TELANGIECTATIC
OSTEOSARCOMA
Lytic lesion , poorly
marginated,
Cortical bone
destruction without
distinct surrounding
bony sclerosis
Periosteal reaction
frequent
TELANGIECTATIC OSTEOSARCOMA
Tumor has hemorrhagic red-brown appearance
resembling blood clot
TELANGIECTATIC
OSTEOSARCOMA
Resmbles aneurysmal
bone cyst at low power
TELANGIECTATIC
OSTEOSARCOMA
Shows malignant osteoid
TELANGIECTATIC OSTEOSARCOMA
Hypercellular pleomorphic stromal cells within solid septa
adjacent to cystic space. Mitosis seen
SMALL CELL OSTEOSARCOMA
Small cell OS of distal
femur : X-ray image
showing lytic and
blastic tumour tissue
at the soft tissue
compartment of the
lesion.
SMALL CELL
OSTEOSARCOMA
SMALL CELL
OSTEOSARCOMA
GROSS : Indistinguishable from those of conventional
OS
HISTOPATHOLOGY
•Small cells associated with osteoid production.
•Tumours are classified according to predominant cell
type : round cell type or spindle cell type.
•The nuclear diameter of round cells can be very
small(comparable to Ewing sarcoma) to
large(comparable to large cell lymphoma).
Small cell osteosarcoma, small cell type. Osteoid
production at lower right.
CENTRAL LOW GRADE
OSTEOSARCOMA
CENTRAL LOW
GRADE
OSTEOSARCOM
A
Cut surface shows a grey
white tumour with a firm
& gritty texture arising
from the medullary cavity.
CENTRAL LOW GRADE
OSTEOSARCOMA
Microscopy
 Tumor is composed of hypocellular spindle
cell proliferation.
 The osteogenic matrix is produced as well
formed trabeculae of bone.
 The spindle cell show minimal cytolgical
atypia
 Mitotic figures are sparse.
 The tumor tend to permeate marrow fat &
surrounding bony trabeculae.
CENTAL LOW GRADE OSTEOSARCOMA
Bony trabeculae surrounded by a hypocellular
spindle cell stroma with minimal cytologic atypia.
OS SECONDARY TO
PAGET'S DISEASE
OS SECONDARY TO PAGET'S
DISEASE
Grossly : destructive tumors along with fleshy soft
tumors
 Microscopically: high grade OS, mostly osteoblastic or
fibroblastic. A great number of osteclast like giant cell
may be found.
Genetics : linked to 18q
PAGET’S SARCOMA
Proximal femur shows
thickened cortical and
medullary bone
characteristic of underlying
paget’s disease with a large
hemorrhagic tan-white
sarcomatous tumor that has
broken through cortex
PAGET’S SARCOMA
High grade osteosarcoma permeating abnormal bone
seen in Paget’s disease which is thickened and lined by
osteoclasts
POST-IRRADIATION
SARCOMA
POSTIRRADIATION
SARCOMA
Criteria for diagnosis
1. The affected bone may have been normal/benign
tumour/non-bone forming malignancy.
2. History of prior radiation therapy & tumour
developed in the path of radiation beam.
3. A symptom free latent period must follow.
4. Sarcoma must be biopsy proven.
DIFFERENTIAL DIAGNOSIS
OF OS
 CALLUS
 OSTEOBLASTOMA
 ANEURYSMAL BONE CYST
 CHONDROBLASTOMA
 GCT
 EWING’S SARCOMA
 MALIGNANT LYMPHOMA
 CHONDROSARCOMA
 FIBROSARCOMA
 FIBROUS DYSPLASIA
HISTOLOGIC
FEATURES
CALLUS OSTEOBLASTOM
A
OSTEOSARCOM
A
Osteoblastic
rimming
+++ 0-++ 0
Trapping of host
lamellar bone
0-++ 0 ++-+++
Circumscribed 0 +++ 0
Lesional fibrous
tissue
0-+++ No ++-+++
Lesional hyaline
cartilage
0-+++ Very rare 0-++++
Scattered atypical
nuclei
0 Rare 0-++++
Atypical mitotic
figures
0 0 0-++++
Extensive necrosis 0-+ 0 0-+++
Osteosarcoma v/s callus
HISTOLOGIC FEATURES ANEURYSMAL BONE
CYST
TELANGIECTATIC
OSEOSARCOMA
Osteoblastic rimming 0-++ 0
Trapping host lamellar bone - 0-++++
Pure woven bone Yes or no Yes
Lesional fibrous tissue 0-+ 0-++
Scattered atypical nuclei Rare +-+++
Atypical mitotic figures 0 0-+++
Large blood filled cysts +-++++ +++
Extensive necrosis 0-+ +-++++
Anaplasia - Yes
Osteosarcoma v/s ABC
ABC with blood filled cystic space surrounded by wall containing
proliferating fibroblasts, reactive woven bone, & osteoclast type
giant cells
HISTOLOGIC FEATURES FIBROUS DYSPLASIA LOW GRADE OS
Osteoblastic rimming 0 0-+
Trapping host lamellar
bone
0 0-++
Pure woven bone Yes Usually no
C &Y spicules of bone Yes No
Lesional fibrous tissue ++-+++ +-++
Lesional hyaline cartilage Rare 0-++
Scattered atypical nuclei Rare +-++
Atypical mitosis 0 0-+
Extensive necrosis 0 0-+
DIFFERENTIAL DIAGNOSIS
OF OS
Chondroblastoma : Epiphyseal centering , it is rarely
occur in OS
Fibrosarcoma : Not associated with any tumor osteoid,
bone, or cartilage
Chondrosarcoma : >50yr, cartilage forming tumor, on X-
ray it present as area of radiolucency with variably
distributed punctate opacities, periosteal reaction is
absent or minimal, osteoid is absent
Osteosarcoma v/s GCT
Giant cell tumor : epiphysial
centering, occur in 20-40yr, show
bone expanding & eccenteric area of
lysis, & multinucleated giant cells on
Features distinguishing both are:
1. Location
2. Codman’s triangle
3. Intralesional bone fluffs
4. Anaplasia of stromal cells
5. Production of malignant osteoid
OSTEOSARCOMA vs GCT
OS v/s Ewing sarcoma
Ewing sarcoma : More common in men,
pelvis & ribs are mc site, onion skin
periosteal reaction seen on X-ray & on
histo composed of small round cells with
Osteosarcoma v/s
Chondroblastoma
Image showing
eosinophilic
chondroid matrix
with calcific
deposit.The
mononuclear cells
contain round to
oval nuclei
sorrounded by
pink cytoplasm.
Hyaline cartilage &
atypical mitosis
Osteosarcoma v/s
Chondrosarcoma
Chondrosarcoma
permeates around
pre-existing
trabecular bone
OSTEOSARCOMAS OF
SURFACE BONES
 PAROSTEAL OSTEOSARCOMA
 PERIOSTEAL OSTEOSARCOMA
 HIGH GRADE SURFACE OSTEOSARCOMA
PAROSTEAL
OSTEOSARCOMA
PAROSTEAL
OSTEOSARCOMA
Radiologic findings:
heavily mineralised
mass attached to
cortex, with broad
base
PAROSTEAL
OSTEOSARCOMA
 Gross findings:
1. Hard lobulated mass
attached to cortex
2. Nodules of cartilage may
be present; occ cartilage
may be incomplete cap
like
3. Soft at periphery
4. Fleshy, soft areas
PAROSTEAL
OSTEOSARCOMA
 Microscopic findings:
1. Well formed bony trabeculae
2. Hypocellular stroma
3. With or without osteoblastic rimming
4. Stromal cells show minimal atypia
5. 20% show moderate atypia
6. 50% of the tumour show cartilage differentiation
Moderately atypical spindle tumor
cells grow between irregularly
shaped bone trabeculae
Shows mature appearing bone
surrounded by a hypocellular
fibroblastic stroma with minimal
cytologic atypia
DIFFERENTIAL DIAGNOSIS
 Fibrous dysplasia
 Myositis ossificans : It does not involve cortex, show
much more cellularity than parosteal OS & atypical
mitosis not seen
 Osteochondroma : In osteochondroma radiography
shows continuity between the bone and the
osteochondroma, and the intertrabecular spaces
contain fatty or hematopoietic marrow
Parosteal OS v/s Myositis
Ossificans
MO showing osteoid production in a cellular fibrous
tissue
Parosteal OS V/S
Osteochondroma
Image showing
thickened hyaline
cartilage cap
which mature via
enchondral
calcification to
bony trabeculae
surrounded by fat
& hematopoietic
marrow
PERIOSTEAL
OSTEOSARCOMA
PERIOSTEAL
OSTEOSARCOMA
 Radiologic findings:
1. Sunburst appearance
2. Codman’s triangle is frequent
3. Cortex appears thickened
4. CT-scan & MRI : important in the evaluation of
tumor size, integrity of cortex, soft tissue
extension, & relationship to soft tissue bundle
PERIOSTEAL
OSTEOSARCOMA
 Gross findings:
1. Tumor arises from bone surface
2. May involve part or entire circumference
3. Calcified spicules can be seen, longest in center
and tapering on both sides
4. Well delineated by capsule/pseudocapsule
5. Merges imperceptibly with cortex at base
PERIOSTEAL
OSTEOSARCOMA
 Microscopic findings:
1. Tumour is arranged in lobules with moderately
atypical chondrocytes
2. There is spindling at the periphery & spindle
cells shows matrix production
3. The centre of the chondroid lobule also show
bone formation
PERIOSTEAL OSTEOSARCOMA
Shows malignant chondroid matrix merging with
osteoid
HIGH GRADE SURFACE OSTEOSARCOMA
HIGH GRADE SURFACE
OSTEOSARCOMA
Radiologic findings: surface lesion , partially
mineralized, mass extending in soft tissue
Underlying cortex is destroyed with periosteal new
bone formation
HIGH GRADE SURFACE
OSTEOSARCOMA
 Gross findings:
1. Situated on surface of bone
2. Underlying cortex is eroded
3. Surface is multilobulated
4. Colour depends on amount of chondroid matrix,
hemorrhage & necrosis
5. Soft areas separate it from parosteal
osteosarcoma
HIGH GRADE SURFACE
OSTEOSARCOMA
 Microscopic findings: same features seen in
conventional OS
1. Show osteoblastic, fibroblastic or chondroblastic
differentiation
2. High grade cytologic atypia
3. Lace like osteoid
4. Chondroblastic area rich tumor may be confused
with periosteal osteosarcoma
HIGH GRADE SURFACE OS:
DIFFERENTIAL DIAGNOSIS
1. Parosteal OS :pattern of osteoid production & high grade
cytological atypia is seen in high grade OS
2. Periosteal OS : In high grade OS, tumour shows larger
regions of spindle cell morphology & more cellular atypia
Fibrous dysplasia
Fibrous dysplasia
• Benign medullary fibro-osseous lesion
• May involve one or more bone
• Occur in children & adults with equal sex distribution
• Sites : m.c. in jaw bones,in women long bones & in
men ribs & skull are favoured sites
• Types : 1. monostotic form(more common) : skull more
commonly followed by femur, tibia, ribs
2. polystotic form : femur, pelvis, tibia in majority
of cases
Fibrous dysplasia : Radiographic
features
-Often show a non-
aggressive lesion with a
ground glass matrix
-No soft tissue extension
-periosteal reaction is not
seen unless there is a
complicating fracture
-CT-scan & MRI better
define the extent
There is a well defined lucency with
Sclerotic margins
Fibrous dysplasia : Gross
features
The bone is often
expanded
 tan grey colour
firm-to-gritty
consistency.
There my be cysts,
which may contain some
yellow-
tinged fluid
circumscribed blue
tinged translucent
material if cartilage is
Fibrous dysplasia : Microscopy
 Well cirumscribed
 Composed of fibrous & osseus component
The fibrous component is composed of bland spindle
cell with low mitotic rate
The osseus component is comprised of irregular
curvilinear trabeculae of woven bone(rarely lamellar
bone)
Secondary changes : foam cells, multinucleated giant
cells, aneurysmal bone cyst or myxoid changes
Fibrous dysplasia : Microscopy
The fibrous stroma is composed of bland spindle cells
arranged in whorled or storiform pattern
Fibrous dysplasia : Microscopy
Anastomoting trabeculae of immature woven bone without
osteoblastic rimming
Fibrous dysplasia : Microscopy
Curvilinear trabeculae often describing as resembling
‘Chinese characters’
Fibrous dysplasia : Microscopy
Characteristic
C shaped
bony spicules
with spindle
cell stroma
Fibrous dysplasia : D/D
1. Parosteal OS :
• juxtacortical position,
• show moderate stromal anaplasia
2. Low grade intramedullary OS; fibrous dysplasia like :
•radiologically more aggresive, &
• lack rim of benign host bone sclerosis
Fibrous dysplasia : D/D
3. Osteofibrous dysplasia :
exclusively seen in tibia &/or fibula of
children < 10yr,
show predilection for cortex,
characteristically shows focal to diffuse
osteoblastic rimming of the bone trabeculae
Fibrous dysplasia
Genetics :1. Mutation in GNAS1 in monostotic & polystotic
forms
2. structural rearrangements involving 12p13 & trisomy
2
Prognosis : good , rarely malignant transformation may occu
SUMMARY
Constant
fundament
al tissue
Variable
fundament
al tissue
Reactive
tissue
(possible)
Benign to
locally
aggressive
tumor
Malignant
tumor
Osteoid,
&/woven
bone
A. Cartilage
B. No
cartilage
Fibrous,
OLGCs,
osteoclasts
osteoclasts
Callus(early
to
mid),periost
itis
ossificans
OO,OB
Osteosarco
ma all types
Osteoid ,
woven
bone, &
cartilage Osteoclasts
like giant
cells
Fibrous
dysplasia
Parosteal
OS
THANK YOU

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Bone Forming Tumours & Fibrous Dysplasia

  • 1. BONE FORMING TUMOURS & FIBROUS DYSPLASIA Presented by: ARUN KUMAR Moderator: Dr. SHIKHA PRAKASH
  • 2. NORMAL BONE Parts of bones 1. Epiphysis : ends of bone 2. Diaphysis : shaft 3. Metaphysis : epiphysial end of diaphysis Macroscopically the architecture of bone may be 1. Compact 2. cancellous/spongy/trabeculae
  • 3. NORMAL BONE On longitudinal section the bone is composed of : 1. Periosteum 2. Cortex 3. endosteum 4. Medullary cavity
  • 4.  Unmineralised matrix laid down by osteoblasts  Stains homogenously pink on H&E  On polarized light demonstrates criss cross or woven pattern of collagen fibers  Can be confused with fibrin, collagen or chondroid. OSTEOID
  • 5. NORMAL BONE Woven bone : In these, collagen is deposited in a random weave manner. Lamellar bone : Collagen is deposited in a order. It replaces woven bone during growth ,deposited much slowly & stronger than woven bone
  • 6. Woven bone (top) deposited on the surface of preexisting lamellar bone(bottom)
  • 8. Histologic Type Benign Malignant Chondrogenic (22%) Osteochondroma Chondrosarcoma Chondroma Dedifferentiated chondrosarcoma Chondroblastoma Mesenchymal chondrosarcoma Chondromyxoid fibroma clear cell chondrosarcoma Osteogenic (19%) Osteoid osteoma Osteosarcoma Osteoblastoma Fibrogenic Desmoplastic fibroma
  • 9. notochordal tumors Chordoma Histiocytic origin Benign Fibrous histiocytoma Malignant fibrous histiocytoma Ewing’s sarcoma/PNET Giant cell tumor Malignant Giant cell tumor Hematopoietic (40%) Myeloma Malignant lymphoma Joint lesions Synovial chondromatosis Vascular Hemangioma Hemangioendotheliom a Hemangiopericytoma, angiosarcoma
  • 10. Lipogenic Lipoma Liposarcoma Neurogenic Neurilemmoma Smooth muscle tumors Leiomyoma Leiomyosarcoma Miscellaneous tumors Adamantinoma Metastatic malignancy Tumours of unidentified neoplastic nature Aneurysmal bone cyst Simple cyst Fibrous dysplasia Langerhan’s cell histiocytosis Chest wall hamartoma Osteofibrous dysplasia
  • 11. BONE FORMING TUMOURS 1. OSTEOID OSTEOMA 2. OSTEOBLASTOMA 3. OSTEOSARCOMA  CONVENTIONAL OS  TELANGIECTATIC OS  SMALL CELL OS  LOW GRADE CENTRAL OS  SECONDARY OS  PAROSTEAL OS  PERIOSTEAL OS  HIGH GRADE SURFACE OS
  • 13. OSTEOID OSTEOMA  Benign lesion characterized by less than 2 cm pea like mass of abnormal bone-nidus  Limited growth potential  Clinical features 1. children & adolescents, occasionally in older individuals 2. more common in males 3. any bone except sternum. 1. most common in the long bones (metaphysis or shaft). 2. The tumour tend to involve the cortex rather than medulla
  • 14. OSTEOID OSTEOMA  Signs & symptoms: 1. Pain-characteristically relieved by salicylates and NSAIDS 2. Swelling & redness 3. Joint effusion 4. Scoliosis 5. Bone growth abnormalities  Genetics : involvement of 22q13 & loss of 17q
  • 15. OSTEOID OSTEOMA: RADIOLOGIC FINDINGS  X-RAY : Dense cortical sclerosis surrounding a radiolucent nidus,  CT SCAN : best imaging study to demonstrate OO, help
  • 16. OSTEOID OSTEOMA: RADIOLOGIC FINDINGS  MRI SCAN: Useful in demonstrating medullary or periarticular lesions and peri-tumoural oedema, also help to localize the lesion BONE SCAN: associated with hot bone scan, helpful in localizing the lesion
  • 17. OSTEOID OSTEOMA : GROSS FINDINGS Small , cortically based, red, gritty or granular round lesion surrounded (well circumscribed) by ivory white sclerotic bone
  • 18. MICROSCOPIC FINDINGS  The tumour consists of a central area of vascularised connective tissue within which differentiating osteoblasts are engaged in the production of osteoid and sometime bone  if actual bone is present osteoclast may also be seen engaged in remodelling
  • 19. OSTEOID OSTEOMA Low power view showing wedge shaped nidus protruding slightly above the surface and surrounded by sclerotic bone Central nidus with dense sclerotic bone
  • 20. Osteoid osteoma shows anastomosing irregular bony trabeculae with osteoblastic rimming embedded in a hypocellular fibrovascular stroma
  • 21. PROGNOSIS  Prognosis is excellent  Recurrence is rare
  • 22. DIFFERENTIAL DIAGNOSIS  SOLITARY ENOSTOSIS  OSTEOMYELITIS  BRODIE’S ABSCESS  OSTEOBLASTOMA  OSTEOSARCOMA
  • 23. Osteoid osteoma v/s Solitary enostosis SOLITARY ENOSTOSIS : does not cause pain or host bone sclerosis. Usually consists of lamellar bone with haversian systems
  • 24. Osteoid osteoma v/s Osteomyelitis Chr osteomyelitis : showing necrotic bone,chr inflammation & fibrosis
  • 25. Osteoid osteoma v/s Brodie’s abscess Image shows fibrosis , degenerating bony spicules, & subacute inflammation
  • 27. OSTEOBLASTOMA  A rare benign tumor, usually larger than 2 cm, characterized by osteoid and woven bone production.  Age: 10-30 yrs  M:F is 2.5:1  Skeletal distribution: predilection for spine particularly posterior elements and sacrum, others- long bones-proximal and distal femur, proximal tibia  Site: majority intra-osseus but a small percentage can occur on the surface of the bone in a
  • 28. OSTEOBLASTOMA Symptoms: dull, aching, nocturnal pain not relieved by aspirin Genetics : 1. chromosomal rearrangements have been described in 4 cases, with chr no. ranging from hypodiploid to hyperdiploid. 2. MDM2 amplification 3. TP53 deletion in aggressive osteoblastoma
  • 29. OSTEOBLASTOMA: RADILOGICAL FINDINGS  SPINAL LESION 1. Main mass centered in vertebral arch 2. Round to ovoid lytic lesion with bone expansion 3. Periosteal new bone formation 4. Intra-lesional bone forming
  • 30. X-ray showing expansion of the left fourth lumbar pedicle : transeverse process and superior facet
  • 31. OSTEOBLASTOMA: GROSS FINDINGS  Lesion is well demarcated,  Round to oval with thin cortex  Mostly granular, gritty, & deep red on cutting  Whitish yellow if bone
  • 32. Osteoblastoma: excised specimen of femoral lesion showing a well demarcated fleshy tumour
  • 33. OSTEOBLASTOMA: MICROSCOPIC FINDINGS  Tumour is composed of woven bone spicules or trabeculae which are arranged haphazardly & lined by single layer of osteoblast  Rich vascularity  Osteoblast may have mitosis but not atypical  Scattered multinucleated giant cells often present
  • 34. OSTEOBLASTOMA: MICROSCOPIC FINDINGS  Tumour has a pushing border  Secondary ABC like changes may be seen  Some osteoblastomas show multiple small nidi
  • 35. Osteoblastoma shows trabeculae of woven bone lined by layer of polygonal osteoblast and the well vascularised inter-trabecular stroma
  • 36. OSTEOBLASTOMA: MICROSCOPIC FINDINGS Malignant osteoblastoma : histologically same but are more cellular, has large no. of giant cells & large amount of spiculated blue bone. locally aggressive & does not develop distant metastasis Aggressive osteoblastoma : characterised by presence of epitheloid osteoblasts, trabecular or sheet-like osteoid & osteosclerotic resorption. locally aggressive & does not develop distant metastasis Pseudomalignant osetoblastoma : tumor cells have hyperchromatic nuclei. However, the nuclei have the
  • 37. Osteoblastoma : prominent epithiloid appearance
  • 38. OSTEOBLASTOMA : PROGNOSIS Prognosis is excellent Recurrence is unusual and may occur in a bone which has difficult surgical access
  • 40. 0steoid osteoma osteoblastoma 1. involvement Every bone except sternum Predilection for spine 2. size < 2cm > 2cm 3. pain Characteristically relieved by NSAIDs Not relieved by NSAIDs 3. imaging Dense, cortical sclerosis surrounding a radiolucent nidus Lytic, well circumscribed , confined by a periosteal shell of reactive bone 4. gross Gritty or granular round lesion surrounded by ivory white sclerotic bone Round to oval, thinned cortex & periosteal reactive bone 5. microscopy Central nidus with dense sclerotic bone Composed of woven bone trabeculae arranged haphazardly
  • 41. Osteoblastoma v/s GCT GIANT CELL TUMOR: located in epiphysis, packed with giant cells and stromal cells
  • 42. OSTEOBLASTOMA: D/D ABC Osteoblastoma 1. Age First 2 decade 10-30yr 2. Sex No sex predilection M>F 3. Site Metaphysial Majority intraosseus 4. Gross Well demarcated, multiloculated mass of blood filled cystic spaces separated by tan white gritty septa Well demarcated, round to oval. Granular, gritty & deep red on cutting 5. Microscopy 6. Recurrence Well circumscribed, blood filled cystic spaces, separated by fibrous septa rich in collagen producing fibroblasts & callus like tissue Tumor composed of haphazardly arranged woven bone spicules or trabeculae Unusual
  • 43. Osteoblastoma v/s ABC ABC with blood filled cystic space surrounded by wall containing proliferating fibroblasts, reactive woven bone, & osteoclast type giant cells
  • 45. OSTEOSARCOMAS  Osteosarcomas is a mesenchymally derived malignant tumor that produces osteoid and/or bone.  They can be: 1. INTRAMEDULLARY OS 2. INTRACORTICAL OS 3. JUXTACORTICAL OS
  • 46. OSTEOSARCOMA  PRIMARY OSTEOSARCOMA 1. CONVENTIONAL OSTEOSARCOMA a) Osteobalstic b) Chondroblastic c) Fibroblastic 2. SMALL CELL OSTEOSARCOMA 3. TELANGIECTATIC OSTEOSARCOMA 4. LOW GRADE CENTRAL OSTEOSARCOMA
  • 47. OSTEOSARCOMA 5. SURFACE OSTEOSARCOMA a) Parosteal osteosarcoma b) Periosteal osteosarcoma c) High grade surface osteosarcoma  SECONDARY OSTEOSARCOMA 1. Osteosarcoma in paget’s disease 2. Postirradiation osteosarcoma 3. Osteosarcoma in other benign precursors
  • 48. age/sex site c/f prognosis Convention al OS 2nd decade M>F Metaphysis (91%) of long bones Pain,swellin g,path # If untreated fatal Telangiectat ic OS (<4%) 2nd decade M>F Metaphysis of long bones Pain,swellin g ,path # As above Small cell OS(1.5%) 2nd decade, slightly mc in female >50% metaphysis of long bones Pain,swellin g Slightly worse than convention al OS Low grade central OS(1-2%) 2nd-3rd , M=F 80% in long bones Pain,swellin g excellent
  • 49. age/sex site c/f prognosis OS sec. to paget ds 65yr M>F,2:1 Long bones, pelvis, skull Pain, swelling, path# Poor Post- radiation OS (3.4%- 5%) Children are at greatest risk Any bone, mc in pelvis & shoulder region Pain, swelling 5yr survival rate for extremity lesion is 68.2% & axial lesion 27.3% Parosteal OS Young adult, slight female predominan ce Long bones Pain, swelling, inability to flex the knee excellent
  • 50. age/sex site c/f prognosis Periosteal OS 2nd-3rd decade Slightly mc in male Diaphysial or dia-meta region of long bones Painless swelling initially, later pain excellent High grade surface OS 2nd decade, slightly mc in male Long bones Mass &/or pain Depend upon response to chemothera py
  • 52. CONVENTIONAL OS:RADIOLOGIC FINDINGS  Metaphysial location  It may be pure oseoblastic or osteolytic. In most cases it is mixed lytic/blastic lesion accompanied by cortical destruction and extension into soft tissue  Tumours tend to be eccentric. Mixed blastic/lytic lesion involving the femoral metaphys
  • 53. CONVENTIONAL OS:RADIOLOGIC FINDINGS  Periosteal reaction: 1. Codman’s triangle 2. Longitudinal laminations 3. Perpendicular spiculations  Soft tissue mass
  • 54. CONVENTIONAL OS :GROSS FINDINGS  OS often a large (over 5cm), metaphysial centered , fleshy or hard tumour which may contain cartilage  It frequently transgresses the cortex & is associated with a soft tissue mass
  • 55. CONVENTIONAL OS :MICROSCOPIC FINDINGS 1. It is highly anaplastic , pleomorphic tumor. 2. The tumour cells may be epithelioid ,plasmacytoid , fusiform, ovoid, small round cells, clear cells, giant cells or spindle cells. Most cases are mixture of 2 or more of these cell types. 3. Osteoid and/or bone production by tumor cells. dense, pink, amorphous,curvilinear with small nubs,arborisation,abortive lacunae formation, intercellular material.
  • 56. On the basis of production of predominant matrix by tumors the conventional OS is subdivided into: 1. OSTEOBLASTIC (50%) 2. CHONDROBLASTIC (25%) 3. FIBROGENIC (25%)
  • 57. OSTEOBLASTIC OSTEOSARCOMA 1. Bone &/or osteoid are the predominant matrix 2. The matrix may be thin, arborising osteoid (i.e., filigree) to dense compact osteoid & Osteoblastic OS: showing osteoid
  • 58. OSTEOBLASTIC OSTEOSARCOMA Filigree osteoid comprises thin, randomly arborizing lines of osteoid interweaving b/w neoplastic cells Flat and thick osteoid
  • 59. CHONDROBLASTIC OSTEOSARCOMA  Chondroid matrix is predominant.  It tends to be high grade hyaline cartilage.  Myxoid and other forms of cartilage are uncommon, except in the jaws & pelvis.  Osteoid is seen between spindle cells or in center of chondroid lobules.
  • 60. CHONDROBLASTIC OSTEOSARCOMA Lobules of malignant cartilage. There is spindling at the periphery and osteoid formation.
  • 61. FIBROBLASTIC OSTEOSARCOMA  25% of all conventional osteosarcoma  A high grade spindle cell malignancy  Minimal matrix production
  • 62. Unusual forms of osteosarcoma 1.Epitheloid osteosarcoma 2.Ostoblastic osteosarcoma – sclerosing type 3.Osteosarcoma resembling osteoblastoma 4.Chondromyxoid fibroma-like osteosarcoma 5.Chondroblastoma-like osteosarcoma 6.Clear cell osteosarcoma 7.Malignant fibrous histiocytoma-like osteosarcoma 8.Giant cell rich osteosarcoma Not associated with specific biologic behaviour that differs from conventional osteosarcoma.
  • 64. TELANGIECTATIC OSTEOSARCOMA Lytic lesion , poorly marginated, Cortical bone destruction without distinct surrounding bony sclerosis Periosteal reaction frequent
  • 65. TELANGIECTATIC OSTEOSARCOMA Tumor has hemorrhagic red-brown appearance resembling blood clot
  • 66. TELANGIECTATIC OSTEOSARCOMA Resmbles aneurysmal bone cyst at low power TELANGIECTATIC OSTEOSARCOMA Shows malignant osteoid
  • 67. TELANGIECTATIC OSTEOSARCOMA Hypercellular pleomorphic stromal cells within solid septa adjacent to cystic space. Mitosis seen
  • 69. Small cell OS of distal femur : X-ray image showing lytic and blastic tumour tissue at the soft tissue compartment of the lesion. SMALL CELL OSTEOSARCOMA
  • 70. SMALL CELL OSTEOSARCOMA GROSS : Indistinguishable from those of conventional OS HISTOPATHOLOGY •Small cells associated with osteoid production. •Tumours are classified according to predominant cell type : round cell type or spindle cell type. •The nuclear diameter of round cells can be very small(comparable to Ewing sarcoma) to large(comparable to large cell lymphoma).
  • 71. Small cell osteosarcoma, small cell type. Osteoid production at lower right.
  • 73. CENTRAL LOW GRADE OSTEOSARCOM A Cut surface shows a grey white tumour with a firm & gritty texture arising from the medullary cavity.
  • 74. CENTRAL LOW GRADE OSTEOSARCOMA Microscopy  Tumor is composed of hypocellular spindle cell proliferation.  The osteogenic matrix is produced as well formed trabeculae of bone.  The spindle cell show minimal cytolgical atypia  Mitotic figures are sparse.  The tumor tend to permeate marrow fat & surrounding bony trabeculae.
  • 75. CENTAL LOW GRADE OSTEOSARCOMA Bony trabeculae surrounded by a hypocellular spindle cell stroma with minimal cytologic atypia.
  • 77. OS SECONDARY TO PAGET'S DISEASE Grossly : destructive tumors along with fleshy soft tumors  Microscopically: high grade OS, mostly osteoblastic or fibroblastic. A great number of osteclast like giant cell may be found. Genetics : linked to 18q
  • 78. PAGET’S SARCOMA Proximal femur shows thickened cortical and medullary bone characteristic of underlying paget’s disease with a large hemorrhagic tan-white sarcomatous tumor that has broken through cortex
  • 79. PAGET’S SARCOMA High grade osteosarcoma permeating abnormal bone seen in Paget’s disease which is thickened and lined by osteoclasts
  • 81. POSTIRRADIATION SARCOMA Criteria for diagnosis 1. The affected bone may have been normal/benign tumour/non-bone forming malignancy. 2. History of prior radiation therapy & tumour developed in the path of radiation beam. 3. A symptom free latent period must follow. 4. Sarcoma must be biopsy proven.
  • 82. DIFFERENTIAL DIAGNOSIS OF OS  CALLUS  OSTEOBLASTOMA  ANEURYSMAL BONE CYST  CHONDROBLASTOMA  GCT  EWING’S SARCOMA  MALIGNANT LYMPHOMA  CHONDROSARCOMA  FIBROSARCOMA  FIBROUS DYSPLASIA
  • 83. HISTOLOGIC FEATURES CALLUS OSTEOBLASTOM A OSTEOSARCOM A Osteoblastic rimming +++ 0-++ 0 Trapping of host lamellar bone 0-++ 0 ++-+++ Circumscribed 0 +++ 0 Lesional fibrous tissue 0-+++ No ++-+++ Lesional hyaline cartilage 0-+++ Very rare 0-++++ Scattered atypical nuclei 0 Rare 0-++++ Atypical mitotic figures 0 0 0-++++ Extensive necrosis 0-+ 0 0-+++
  • 85. HISTOLOGIC FEATURES ANEURYSMAL BONE CYST TELANGIECTATIC OSEOSARCOMA Osteoblastic rimming 0-++ 0 Trapping host lamellar bone - 0-++++ Pure woven bone Yes or no Yes Lesional fibrous tissue 0-+ 0-++ Scattered atypical nuclei Rare +-+++ Atypical mitotic figures 0 0-+++ Large blood filled cysts +-++++ +++ Extensive necrosis 0-+ +-++++ Anaplasia - Yes
  • 86. Osteosarcoma v/s ABC ABC with blood filled cystic space surrounded by wall containing proliferating fibroblasts, reactive woven bone, & osteoclast type giant cells
  • 87. HISTOLOGIC FEATURES FIBROUS DYSPLASIA LOW GRADE OS Osteoblastic rimming 0 0-+ Trapping host lamellar bone 0 0-++ Pure woven bone Yes Usually no C &Y spicules of bone Yes No Lesional fibrous tissue ++-+++ +-++ Lesional hyaline cartilage Rare 0-++ Scattered atypical nuclei Rare +-++ Atypical mitosis 0 0-+ Extensive necrosis 0 0-+
  • 88. DIFFERENTIAL DIAGNOSIS OF OS Chondroblastoma : Epiphyseal centering , it is rarely occur in OS Fibrosarcoma : Not associated with any tumor osteoid, bone, or cartilage Chondrosarcoma : >50yr, cartilage forming tumor, on X- ray it present as area of radiolucency with variably distributed punctate opacities, periosteal reaction is absent or minimal, osteoid is absent
  • 89. Osteosarcoma v/s GCT Giant cell tumor : epiphysial centering, occur in 20-40yr, show bone expanding & eccenteric area of lysis, & multinucleated giant cells on
  • 90. Features distinguishing both are: 1. Location 2. Codman’s triangle 3. Intralesional bone fluffs 4. Anaplasia of stromal cells 5. Production of malignant osteoid OSTEOSARCOMA vs GCT
  • 91. OS v/s Ewing sarcoma Ewing sarcoma : More common in men, pelvis & ribs are mc site, onion skin periosteal reaction seen on X-ray & on histo composed of small round cells with
  • 92. Osteosarcoma v/s Chondroblastoma Image showing eosinophilic chondroid matrix with calcific deposit.The mononuclear cells contain round to oval nuclei sorrounded by pink cytoplasm. Hyaline cartilage & atypical mitosis
  • 94. OSTEOSARCOMAS OF SURFACE BONES  PAROSTEAL OSTEOSARCOMA  PERIOSTEAL OSTEOSARCOMA  HIGH GRADE SURFACE OSTEOSARCOMA
  • 97. PAROSTEAL OSTEOSARCOMA  Gross findings: 1. Hard lobulated mass attached to cortex 2. Nodules of cartilage may be present; occ cartilage may be incomplete cap like 3. Soft at periphery 4. Fleshy, soft areas
  • 98. PAROSTEAL OSTEOSARCOMA  Microscopic findings: 1. Well formed bony trabeculae 2. Hypocellular stroma 3. With or without osteoblastic rimming 4. Stromal cells show minimal atypia 5. 20% show moderate atypia 6. 50% of the tumour show cartilage differentiation
  • 99. Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae Shows mature appearing bone surrounded by a hypocellular fibroblastic stroma with minimal cytologic atypia
  • 100. DIFFERENTIAL DIAGNOSIS  Fibrous dysplasia  Myositis ossificans : It does not involve cortex, show much more cellularity than parosteal OS & atypical mitosis not seen  Osteochondroma : In osteochondroma radiography shows continuity between the bone and the osteochondroma, and the intertrabecular spaces contain fatty or hematopoietic marrow
  • 101. Parosteal OS v/s Myositis Ossificans MO showing osteoid production in a cellular fibrous tissue
  • 102. Parosteal OS V/S Osteochondroma Image showing thickened hyaline cartilage cap which mature via enchondral calcification to bony trabeculae surrounded by fat & hematopoietic marrow
  • 104. PERIOSTEAL OSTEOSARCOMA  Radiologic findings: 1. Sunburst appearance 2. Codman’s triangle is frequent 3. Cortex appears thickened 4. CT-scan & MRI : important in the evaluation of tumor size, integrity of cortex, soft tissue extension, & relationship to soft tissue bundle
  • 105.
  • 106. PERIOSTEAL OSTEOSARCOMA  Gross findings: 1. Tumor arises from bone surface 2. May involve part or entire circumference 3. Calcified spicules can be seen, longest in center and tapering on both sides 4. Well delineated by capsule/pseudocapsule 5. Merges imperceptibly with cortex at base
  • 107.
  • 108. PERIOSTEAL OSTEOSARCOMA  Microscopic findings: 1. Tumour is arranged in lobules with moderately atypical chondrocytes 2. There is spindling at the periphery & spindle cells shows matrix production 3. The centre of the chondroid lobule also show bone formation
  • 109. PERIOSTEAL OSTEOSARCOMA Shows malignant chondroid matrix merging with osteoid
  • 110. HIGH GRADE SURFACE OSTEOSARCOMA
  • 111. HIGH GRADE SURFACE OSTEOSARCOMA Radiologic findings: surface lesion , partially mineralized, mass extending in soft tissue Underlying cortex is destroyed with periosteal new bone formation
  • 112. HIGH GRADE SURFACE OSTEOSARCOMA  Gross findings: 1. Situated on surface of bone 2. Underlying cortex is eroded 3. Surface is multilobulated 4. Colour depends on amount of chondroid matrix, hemorrhage & necrosis 5. Soft areas separate it from parosteal osteosarcoma
  • 113. HIGH GRADE SURFACE OSTEOSARCOMA  Microscopic findings: same features seen in conventional OS 1. Show osteoblastic, fibroblastic or chondroblastic differentiation 2. High grade cytologic atypia 3. Lace like osteoid 4. Chondroblastic area rich tumor may be confused with periosteal osteosarcoma
  • 114. HIGH GRADE SURFACE OS: DIFFERENTIAL DIAGNOSIS 1. Parosteal OS :pattern of osteoid production & high grade cytological atypia is seen in high grade OS 2. Periosteal OS : In high grade OS, tumour shows larger regions of spindle cell morphology & more cellular atypia
  • 116. Fibrous dysplasia • Benign medullary fibro-osseous lesion • May involve one or more bone • Occur in children & adults with equal sex distribution • Sites : m.c. in jaw bones,in women long bones & in men ribs & skull are favoured sites • Types : 1. monostotic form(more common) : skull more commonly followed by femur, tibia, ribs 2. polystotic form : femur, pelvis, tibia in majority of cases
  • 117. Fibrous dysplasia : Radiographic features -Often show a non- aggressive lesion with a ground glass matrix -No soft tissue extension -periosteal reaction is not seen unless there is a complicating fracture -CT-scan & MRI better define the extent There is a well defined lucency with Sclerotic margins
  • 118. Fibrous dysplasia : Gross features The bone is often expanded  tan grey colour firm-to-gritty consistency. There my be cysts, which may contain some yellow- tinged fluid circumscribed blue tinged translucent material if cartilage is
  • 119. Fibrous dysplasia : Microscopy  Well cirumscribed  Composed of fibrous & osseus component The fibrous component is composed of bland spindle cell with low mitotic rate The osseus component is comprised of irregular curvilinear trabeculae of woven bone(rarely lamellar bone) Secondary changes : foam cells, multinucleated giant cells, aneurysmal bone cyst or myxoid changes
  • 120. Fibrous dysplasia : Microscopy The fibrous stroma is composed of bland spindle cells arranged in whorled or storiform pattern
  • 121. Fibrous dysplasia : Microscopy Anastomoting trabeculae of immature woven bone without osteoblastic rimming
  • 122. Fibrous dysplasia : Microscopy Curvilinear trabeculae often describing as resembling ‘Chinese characters’
  • 123. Fibrous dysplasia : Microscopy Characteristic C shaped bony spicules with spindle cell stroma
  • 124. Fibrous dysplasia : D/D 1. Parosteal OS : • juxtacortical position, • show moderate stromal anaplasia 2. Low grade intramedullary OS; fibrous dysplasia like : •radiologically more aggresive, & • lack rim of benign host bone sclerosis
  • 125. Fibrous dysplasia : D/D 3. Osteofibrous dysplasia : exclusively seen in tibia &/or fibula of children < 10yr, show predilection for cortex, characteristically shows focal to diffuse osteoblastic rimming of the bone trabeculae
  • 126. Fibrous dysplasia Genetics :1. Mutation in GNAS1 in monostotic & polystotic forms 2. structural rearrangements involving 12p13 & trisomy 2 Prognosis : good , rarely malignant transformation may occu
  • 127. SUMMARY Constant fundament al tissue Variable fundament al tissue Reactive tissue (possible) Benign to locally aggressive tumor Malignant tumor Osteoid, &/woven bone A. Cartilage B. No cartilage Fibrous, OLGCs, osteoclasts osteoclasts Callus(early to mid),periost itis ossificans OO,OB Osteosarco ma all types Osteoid , woven bone, & cartilage Osteoclasts like giant cells Fibrous dysplasia Parosteal OS

Editor's Notes

  1. Recognition of osteoid is important 1. mistaking chondroid of benign chondroblastoma for osteoid may result in diagnosis of osteosarcoma 2. rendering a acorrect diagnosis of os may depend on findng minute amt of osteoid in which well formed matrices r nt identified. Alkphosstin to diff frm fibrin and chondroid
  2. Size is typically &lt;1cm. Blastic center is homogenously dense but the degree may vary. Peripheral radiolucent zone represents fibrovascular zone
  3. OB has no nidus,&gt;2cm and expand affected bone.
  4. Posterior elements involved. Osteosarcomas involve body then spreads to psterior part. Os never cause bone expansion
  5. Ob does not produce lamellar bone and does not contain entrapped host lamellar bone a feature of os. Gc when present in solid masses indicate gc rich os
  6. Ob does not produce lamellar bone and does not contain entrapped host lamellar bone a feature of os. Gc when present in solid masses indicate gc rich os
  7. Anaplaticstromal cells are seen in 75% cases. 25% show subtle anaplasia. Malignant Osteoid must be distinguished from reactive osteoid. Chondroid is round homogenous masses by stromal cells resembling chondroblasts.
  8. Shows partial mineralisation and cortical thickening. Sun burst appearance