Bone: Cystic & Neoplastic Lesions

1. Cystic Lesions: Solitary bone cyst, Aneurysmal

bone cyst
2. Benign disorders: Fibrous dysplasia,
chondroma enchondroma), osteochondroma,
chondroblastoma, chondromyxoid fibroma,
osteoma osteoid osteoma, osteoblastoma
3. Malignant tumor (0.2% from all malignancy):
osteosarcoma, chondrosarcoma, giant cell
tumor (osteoclastoma), Ewings sarcoma

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BONE TUMORS & TUMOR-LIKE LESIONS

BONE-FORMING TUMORS
Osteoma
Osteoid Osteoma & Osteoblastoma
Osteosarcoma (Osteogenic sarcoma)
CARTILAGE-FORMING TUMOR
Osteochondroma
Chondroma
Chondroblastoma
Chondromyxoid fibroma
Chondrosarcoma
FIBROUS & FIBRO-OSSEOUS TUMORS
Fibrous Cortical Defect & Nonossifying Fibroma
Fibrous Dysplasia
Fibrosarcoma & Malignant Fibrous Histiocytoma (MFH)
MISCELANEOS TUMORS
Ewing Sarcoma & Primitive Neuroectodermal Tumor (PNET)
Giant Cell Tumor
Multiple myeloma, & Lymphoma
Metastatic Tumors
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Etiology Factors
Radiation
Chronic Inflammation
Chromium, nickel, cobalt, alluminium,
titanium, methylmetacrylate and
polyethylene
Implanted metallic hardware
Joint prosthesis

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Diagnosis
1. Clinical symptoms (age is
important)
2. Radiologic : location, tumor size,
periosteal reaction, form of bone
destruction (geografi, moth-eaten,
permeative)
3. Histological feature

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Clinical symptoms

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Swelling and painful

General discomfort
Limited movement
Spontaneous fracture

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Grading of bone sarcoma

Cellularity
Nuclei features
Mitosis
Necrosis
G1 = low malignancy
G2 = high malignancy

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Musculoskeletal tumour society staging

of malignant bone lesions
Stage
IA
IB
IIA
IIB
III

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Low grade, intracompartmental

Low grade, extracompartmental
High grade, intracompartmental
High grade, extracompartmental
Any grade, metastatic

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WHO CLASSIFICATION OF BONE

TUMOR
Cartilage tumors
Osteogenic tumors
Fibrogenic tumors
Fibrohistiocytic tumors
Ewing sarcoma/PNET
Haemopoeitic tumors
Giant cell tumor
Notochordal tumors
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Vascular tumors
Smooth muscle tumors
Lipogenic tumors
Neural tumors
Miscellaneous tumors
Miscellaneous lesions
Joint lesions

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WHO CLASSIFICATION OF BONE TUMOR

CARTILAGE TUMOR
Osteochondroma
9210/0
Chondroma 9220/0
Enchondroma 9220/0
Periosteal chondroma 9221/0
Multiple chondromatosis
9220/1
Chondroblastoma
9230/0
Chondromyxoid fibroma
9241/0
Chondrosarcoma
9220/3
cental, primary and secondary
9220/3
Peripheral 9221/3
Dedifferentiated
9243/3
Mesenchymal 9240/3
Clear cell
9242/3
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WHO CLASSIFICATION OF BONE TUMOR

OSTEOGENIC TUMORS
Osteoid osteoma
Osteoblastoma
Osteosarcoma
Conventional
Chondroblastic
Fibroblastic
Osteoblastic
Telangiectatic
Small cell
Low grade central
Secondary
Parosteal
Periosteal
High grade surface
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9191/0
9200/0
9180/3

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9180/3
9181/3
9182/3
9180.3
9183/3
9185/3
9187/3
9180/3
9192/3
9193/3
9194/3
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WHO CLASSIFICATION OF BONE TUMOR

Fibrogenic tumours
Desmoplastic fibroma
Fibrosarcoma

8823/0
8810/3

Fibrohistiocytic tumors
Benign fibrous histicytoma
Malignant fibrous histiocytoma

8830/0
8830/3

Ewing sarcoma/PNET
Ewing sarcoma

9260/3

Haemopoeitic tumours
Plasma cell myeloma
Malignant lymphoma, NOS

9732/3
9590/3

Giant cell tumour

Giant cell tumour
Malignancy in giant cell-tumour

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9250/1
9250/3

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WHO CLASSIFICATION OF BONE TUMOR

Notochordal tumours
Chordoma

9370/3

Vascular tumours
Hemangioma
Angiosarcoma

9120/0
9210/3

Smooth muscle tumours

Leiomyoma
Leiomyosarcoma

8890/0
8890/3

Lipogenic tumours
Lipoma
Liposarcoma
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8850/0
8850/3
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WHO CLASSIFICATION OF BONE TUMOR

Neural tumours
Neurilemmoma

9560/0

Miscellaneous tumours
Adamantimoma
Metastatic malignancy

9261/3
9261/3

Miscellaneous lesions

Aneurysmal bone cyst

Simple cyst
Fibrous dysplasia
Osteofibrous dysplasia
Langerhans cell histiocytosis
Erdheim-Chester disease
Chest wall hamartoma

9751/1

Joint lesions
Synovial chondromatosis

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9220/0

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Bone Tumors
Cystic

Chondroblastic

Osteoblastic

Other

Chondroma
Osteochondroma
Chondroblastoma
Chondromyxoidfibroma

Osteoma
Osteoid osteoma
Osteoblastoma

Fibrous dysplasia

Chondrosarcoma

Osteosarcoma

Benign

Bone cyst:
- solitary
- aneursmal

Malignant

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Giant cell tumorof bone

Plasma cell myeloma
Ewings sarcoma
Lymphoma
Metastatic tumors
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Bone Cyst
Solitary / Bone Cyst
Aneurysmal Bone Cyst

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Bone Cyst

Solitary Bone Cyst

Benign lesion of unknown etiology, predominantly
occur in the distal end of the long bones of young
males
Pain of the affected area and swelling of the soft
tissue
Multiloculated cyst filled with clear or bloody fluid
Treatment: curettage followed by insertion of bone
chips

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Solitary Bone Cyst

Imaging
Radiolucent from
metaphysis epiphysis
Bone expansion (-) or
few
marginal sclerosis (-)
or thin

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Solitary Bone
Cyst

Histopathologic
Cyst wall of connective
tissue with giant cells,
hemosiderin pigment,
some times with bone
formation

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Aneurysmal Bone Cyst

Aneurysmal Bone Cyst
Metaphysis of the long bones and vertebrae,
female>male, 3rd decade
Swelling & pain of the affected area, from few to 20 cm
Spongy appearance filled with blood
Micros: cavernous spaces with walls that lack normal
epithelial lining
The most successful treatment is removal of the entire
lesion recurrences up to 21%

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Aneurysmal Bone Cyst

Multiloculated space filled with blood

in different stage of organization
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Aneurysmal Bone Cyst

Cavernous spaces with walls lack normal endothelial linings

DD: - giant cell tumor of the bone
- teleangiectatic osteosarcoma
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Aneurysmal Bone Cyst

Large cavernous space filled with blood

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BONE-FORMING TUMORS
Osteoma
Osteoid Osteoma & Osteoblastoma
Osteosarcoma (Osteogenic sarcoma)

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OSTEOID OSTEOMA
Clinical
In children and adults
Location
Long bones, mostly close to proximal
of the femur
Close to joint joint effusion
arthritis
Gross pathology
Lesion in cortex les than 1 cm, red,
granular, surrounded by sclerotic bone

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Osteoid Osteoma

Microscopic
Central Vascularized connective tissue
Osteoblast cells, osteoid, bone
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OSTEOBLASTOMA
Clinical
Rare, 1% of all bone tumors
Mostly male, young adult and adult

Location
Vertebra sacrum
Proximal and distal femoral bone, proximal
tibia metaphysis

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OSTEOBLASTOMA

Gross pathology
Abundant blood vessels reddish brown
Concistency like sands
The tumor is round or ovoid with thin cortex
Some times with Aneurysmal Bone Cyst
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OSTEOBLASTOMA
Histopathological
Similar with osteoid osteoma
Trabeculae surrounded by a
layer of osteoblasts
Abundant blood vessels
Mitosis could be (+), nonatypical
Osteoclasts are frequently
found similar with GCT

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Osteosarcoma

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OSTEOSARCOMA
Intramedullar primary tumor
Osteoid producing high grade tumor
Other names:
Classical Osteosarcoma
Osteogenic sarcoma
Osteoblastic sarcoma
Chondroblastic osteosarcoma
Fibroblastic osteosarcoma
Medullary Osteosarcoma
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OSTEOSARCOMA
Clinical
The most frequent primary malignant
tumor
Age: 2nd decade
60% below 25 years old
30% > 40 years old
Male > female
Pathological fracture

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OSTEOSARCOMA
Location
Metaphysis (90%), diaphysis
(,9%) femur distal, tibia prox,
humerus prox
Jaws, skull, pelvis, vertebra
Lab
Alkaline phosphatase
Lactic acid dehydrogenase

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OSTEOSARCOMA
Etiology: unknown

Injury?
Pagets disease of the bone
Radiation
Other bone tumors
Non-neoplastic conditions (osteomyelitis,
prosthesis)

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OSTEOSARCOMA
Microscopic
Tumor cells
Pleomorphic
Anaplastic CD 99
Epitheloid, plasmacytoid, spindle cell, ovoid,
small round cells, clear cells, multinucleated
cells
Osteoid
Dense amorphous mass
pink color: - osteocalcin
- osteonectin

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Osteosarcoma

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Osteosarcoma

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Prognostic Factors
Without treatment fatal
Pulmonary metastasis is the most frequent bad
Response to pre-operative therapy is the most
sensitive survival indicator
Tumor necrosis > 90% (responsive) long term
survival 80-90%
Tumor necrosis <90% (non-responsive) survival <
15%

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CARTILAGE-FORMING TUMOR
Osteochondroma
Chondroma/Enchondroma
Chondroblastoma
Chondromyxoid fibroma
Chondrosarcoma

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OSTEOCHONDROMA
Definition
Growing bone layered by cartilage on the surface of the
bone and filled with bone marrow inner side

Clinical
First 3 decades
Male = female
15% multiple

Location

Femoral metaphysis of distal part

Humerus: proximal part
Tibia: proximal
Fibula: proximal

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OSTEOCHONDROMA

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OSTEOCHONDROMA
Gross pathologhy
Size less than 3 cm
Irregular grayish white mass
When calcification (+), looks and feels
like sands

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OSTEOCHONDROMA
Imaging
Pedunculated
sessile

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OSTEOCHONDROMA

Gross pathology
Cartilage cap less than 2 cm
> 2 cm malignant?
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ENCHONDROMA

Benign cartilage tumor of the medulla of bone

10-25% of all benign bone tumors
Age: 5-80 tahun
May be multiple in the same bone
Location:
Hands and feet: small tubular bone
Humerus: proximal
Femur: proximal and distal

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CHONDROMA / ENCHONDROMA

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ENCHONDROMA
Imaging
Well defined
Can be radiolucent
Often in metaphysis,
central or exentric
No destruction of the
cortex
No invasion to the soft
tissue

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ENCHONDROMA
Histopatologik
Generally hypocellular,
avasular
Hyalin matrix, myxoid or
chondroid
Some time multinodular
In small bone (hands and
feet) can be more cellular
no radiological examination
misleading to malignancy

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CHONDROBLASTOMA
Benign tumor producing cartilage in epiphysis of
immature bone
Clinical
Less than 1% of all benign bone tumor
Age: 10-25 tahun
Male > female
Location
Epiphysis and metaphysis
Femur: proximal and distal
Tibia: proximal
Humerus: proximal
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CHONDROBLASTOMA
Radiologic
Lytic lesion in central
or exentric
Size: 3-6 cm
Well circumscribed

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CHONDROBLASTOMA

Microscopical

The cells are uniform, rounded-polygonal

Cytoplasm thin and eosinophilic
The nuclei are rounded or ovoid (chondroblast)
Nuclear groove
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CHONDROMYXOID FIBROMA

Microscopical
Well circumscribed
Lobular, sel spinde or stelate cells with mixoid matrix
In the center hypocellular, bordered by hypercelular area
20-30% of cases: nuclei are pleomorphic and
hyperchromatic looks like malignant
19%: content cartilage hyalin
Mitosis is rare (not atypical)
10% with ABC
calcification and osteoclast is possible

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CHONDROMYXOID FIBROMA

Imaging
Lesions are found in the metaphysis, exentric, well
circumscribed
Ekspansion to cortex, parallel along the length of bone
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CHONDROMYXOID FIBROMA
Benign tumor with spindle or stellate cells lobules,
and matrix mixoid or chondroid
Clinical
Less than 1% of all bone tumors
More often in male
2nd 3rd decades
Location
All types of bones
Mostly in proximal tibia and distal femur

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CHONDROSARCOMA

Malignant cartilaginous tumor that may arise de novo

(primary chodrosarcoma) or originate from a pre-existing
benign cartyilaginous lesion (secondary chondrosarcoma.
May be followed by myxoid change, calcification, or
ossification
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CHONDROSARCOMA
Primary chondrosarcoma (convensional)
Originate from the center of normal bone
Clinical
25% dari tumor ganas tulang
Menduduki urutan ketiga (mieloma-osteosarkomakondrosarkoma)
90% primer
>50 tahun, sedikit lebih sering pada pria
Location
Pelvis femur proximal humerus proximal
femur distal iga
Metafisis atau diafisis
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Chondrosarcoma

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CHONDROSARCOMA

Imaging: very important

Radiolucent feature
with calcification patch
or ringlike opacities
Erotion/destruction of
the cortex

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CHONDROSARCOMA

Microscopical
Grayish blue matrix (chondroid/myxoid) with irregular cartilage
lobules separated by connective tissue or bone trabecules
More cellular than enchondroma
Atypical cells with enlargement of the nuclei and
hyperchromatic, and some time with benucleation
Infiltrating cortex and medulla different with enchondroma
Matriks kondroid/miksoid
High grade mitosis (+), necrosis (+)
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CHONDROSARCOMA

Gr I

Gr III

Grading prognosis
Celularity
Nuclear size
hyperchromatics
Gr II
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FIBROUS & FIBRO-OSSEOUS TUMORS

Fibrous Cortical Defect & Nonossifying Fibroma
Fibrous Dysplasia
Fibrosarcoma & Malignant Fibrous Histiocytoma
(MFH)

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FIBROUS DYSPLASIA
Benign medullary fibro-osseous lesion,
affected one bone or more
Location
Jaw, ribs, skull
Male: more in long bones

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FIBROUS DYSPLASIA

Microscopical
Consists of bone of connective tissue components
C letter-like trabecule without osteoblastic rimming
cementum-like bone or rounded psammomatous bodies
are some time found

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Ewing Sarcoma
PNET
ICD-O code:
- Ewing sarcoma : 9260/3
- PNET
: 9364/3
- Askin tumor
: 9365/3
Synonyms: Ewing tumor, peripheral neuroepithelioma, peripheral neuroblastoma, Askin tumor

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MISCELANEOS TUMORS
Ewing Sarcoma & Primitive Neuroectodermal
Tumor (PNET)
Giant Cell Tumor
Multiple myeloma, & Lymphoma
Metastatic Tumors

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Ewing sarcoma / PNET

Ewing described firstly as diffuse endothelioma
PNET describes a small cell malignancy which
is considered by some to be similar to, but distinct
from, Ewing tumor.
Imunohistochemistry & cytogenic studies indicate
that PNET and Ewing tumor are the same entity,
and should be considered to be neuroectodermal
derivation
The prognosis has improved dramatically since
the introduction of radiation & chemotherapy
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Ewing Sarcoma

Primitive NEuroectodermal Tumor (PNET)

Ewing sarcoma and PNET are defined as round

cell sarcomas that show varying degrees of
neuroectodermal differentiation
The term Ewing sarcoma has been used for
those tumors that lack of evidence of neuroectodermal differentiation as assesed by light
microscopy, immunohistochemistry, and EM
The term PNET has been employed for tumors
that demonstrate neuroectodermal features as
evaluated by one or more of these modalities
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Ewings Sarcoma

-A rare primary malignant tumor originates in the medullary cavities of the

long bones predominantly
-<2% of all bone malignancies
-Age: 10n 30 years old
-Clinical features: destructive lesion seen radiologically broke the cortex
reactive new bone produced onion skin appearance
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Ewings Sarcoma

Microscopically:
Undifferentiated small round cells (smaller than lymphocyte with
prominent nuclei and scanty cytoplasm) arranged in sheet or
cord
Rich of
vascular background
and fibrous tissue is visible
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Giant Cell Tumor

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GIANT CELL TUMOR

Benign bone tumor, locally aggressive, consists of
neoplastic ovoid mononuclear cell Intermingled with
giant cell osteoclast osteoclastoma
Clinical feature:
4-5%
20% from primary benign bone tumor
Ages: 20-45 years old
Male > female

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GIANT CELL TUMOR

Round, polygonal, and spindle cells,intermingled with

osteoclast-like giant cells that can be so big (50-100
nuclei)

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GIANT CELL TUMOR

Location:
Distal end of femoral tibia proximal
humerus proximal
5%: pelvis, sacrum
<5%: tubular bone of hands and feet
May be very in frequently multicentric

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IMAGING OF GIANT CELL TUMOR

Type 1 = quiescent
Well circumscribed, sclerotic arround
Cortical involvement minimal or Type 2 = active
Well circumscribed, no sclerosis arround
Cortex is thin, expansion +
Type 3 = aggressive
Poorly defined border
Often with cortex destruction and extension to soft tissue

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GIANT CELL TUMOR

No correlation between
radiological grading
and histological
features
Some time soap
bubble appearance is
found

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MALIGNANCY IN GCT

Primary malignant GCT GCT which content high grade sarcoma

part of conventional GCT tissue is still found
Secondary malignant GCT = high grade sarcoma occuring in
recurrent GCT no GCT tissue left
GCT with malignant transformation <1%, female > male

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Metastatic Tumors
The most frequent malignancy in bone
More than primary tumors of bone

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Normal Spine

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Normal Spine

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Normal Spine

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Bone Metastasis of Carcinoma

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Bone Metastasis of Carcinoma

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Bone Metastasis of Carcinoma

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Bone Metastasis of Carcinoma

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