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BONE TUMOR
MODERATOR-DR. S. S. THAKUR
• A bone tumor, is a neoplastic growth of tissue
in bone. Abnormal growths found in the bone
can be either benign or malignant.
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Bones are classified according to their shapeLong bone
Flat bone
Short bone.
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Long bone anatomy

Diaphysis: long shaft of bone
Epiphysis: ends of bone
Epiphyseal plate: growth plate
Metaphysis: b/w epiphysis and diaphysis
Articular cartilage: covers epiphysis
Periosteum: bone covering (pain sensitive)
Sharpey’s fibers: periosteum attaches to underlying
bone
• Medullary cavity: Hollow chamber in bone
- red marrow produces blood cells
- yellow marrow is adipose.
• Endosteum: thin layer lining the
medullary cavity
Diaphysis
Epiphysis
Metaphysis
• Histology of bone tissue
Cells are surrounded by matrix.
- 25% water
- 25% protein
- 50% mineral salts
4 cell types make up osseous tissue
Osteoprogenitor cells
Osteoblasts
Osteocytes
Osteoclasts
• Osteoprogenitor cells:
- derived from mesenchyme
- unspecialized stem cells
- undergo mitosis and develop into
osteoblasts
- found on inner surface of periosteum
and endosteum.
Osteoblasts:
- bone forming cells
- found on surface of bone
- no ability to mitotically
divide
- collagen secretors

Osteocytes:
- mature bone cells
- derived form osteoblasts
- do not secrete matrix
material
- cellular duties include
exchange of
nutrients and waste with
blood
• Osteoclasts
- bone resorbing cells
- bone surface
- growth, maintenance and bone repair
Abundant inorganic mineral salts:
- Tricalcium phosphate in crystalline form called
hydroxyapatite
Ca3(PO4)2(OH)2
- Calcium Carbonate: CaCO3
- Magnesium Hydroxide: Mg(OH)2
- Fluoride and Sulfate
Osteoprogeniter cells &Osteoblast
Osteoclast
Precursors of malignancy in bone
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High Risk
Ollier disease (Enchondromatosis)
and Maffucci syndrome
Familial retinoblastoma syndrome
Rothmund-Thomson syndrome (RTS)
Moderate Risk
Multiple osteochondromas
Polyostotic Paget disease
Radiation osteitis
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Low Risk
Fibrous dysplasia
Bone infarct
Chronic osteomyelitis
Metallic and polyethylene implants
Osteogenesis imperfecta
Giant cell tumour
Osteoblastoma and chondroblastoma
Classification of primary tumour involving bones

Histological
type
Hematopoietic

Chondrogenic

Benign

Malignant
Myeloma

Osteochodroma
Chondroma
Chondroblastoma
Chondromyxoid
fibroma

Malignant
lymphoma
chondrosarcoma
Histological
type

Benign

Malignant

Osteogenic

Osteoma

Osteosarcoma

Osteoid
osteoma
osteoblastoma
Unknown origin Giant cell
tumour

Ewing tumour
Giant cell tumour
admantinoma
Histological
type

Benign

Malignant

Histiocytic
origin

Fibrous
histiocytoma

MFH

Fibrogenic

Metaphyseal fibrous Dysplastic fibroma
defect
Fibrosarcoma

Notochordal
Vascular

Chordoma
Hemangioma

Hemangioendothelioma
Hemangiopericytoma

Lipogenic

Lipoma

Neurogenic

Neurilemmoma

Liposarcoma
Distribution of bone tumors in long
bones
• Epiphyseal lesions:
• Chondroblastoma
• Giant cell tumor
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Metaphyseal intramedullary lesions:
Osteosarcoma
Chondrosarcoma
Aneurysmal bone cyst
• Metaphyseal lesions centered in the cortex:
• Nonossifying fibroma (NOF)
• Osteoid osteoma

• Metaphyseal exostosis:
• Osteochondroma
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Diaphyseal intramedullary lesions:
Ewing’s sarcoma
Lymphoma
Myeloma
Fibrous dysplasia
Enchondroma

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Diaphyseal lesions centered in the cortex:
Adamantinoma
Osteoid osteoma
Sites of Tumors
Important Facts
• 0.001% of all cancers
• MC benign tumor--- Osteochondroma; Osteoid
Osteoma
• MC Skeletal malignancy– Metastasis.
• MC Bone tumor in Pediatric age group & adultsOsteosarcoma
• MC in < 10 y--- Ewing’s sarcoma
• MC Primary bone tumor – Multiple Myeloma
PRESENTING SYMPTOMS
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Patient may present with
An abnormal radiographic finding detected during evaluation of unrelated problem
PAIN:- is most frequent symptom
MASS:- rate of enlargement is important
-Fluctuating mass can be cyst,ganglion or hemangioma
-Family H/O masses near the joint may be indicator of Ollier’s disease or Maffucci
Syndrome
NEUROLOGICAL SYMPTOM:- found in few patients such as sacral tumors & with tumors
located near the nerve causing compression of nerve,especially common in sciatic notch
,inguinal canal & popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY:- found in pelvic
tumors which are painless & without a palpable mass & cause swelling due to
compression of iliac vein.
PHYSICAL EXAMINATION
• Evaluation of patient’s general health
• TUMOR MASS should be measured & its location,shape,
consistency,mobility,tenderness,local temp & change with
position should be noted.
• SKIN & SUBCUTANEOUS TISSUE :
• Small dialated superficial veins overlying the mass are produced
by large tumors
• Café-au-lait spots & subcutaneous neurofibromas indicate Von
Recklinghausen’s disease
• A venous malformation Maffucci Syndrome
• REGIONAL LYMPH NODES: sign of metastatic disease
• Atrophy of surrounding musculature should be recorded,also
neurological deficits & adequacy of circulation.
HISTORY OF THE PATIENT
• AGE:- most imp information,bcoz of their presentaion in sp age
group.
• 1st decade- usually ABC ,SBC
• 2nd decade-Chondroblastoma,osteosarcoma,Ewings
• 3rd decade- GCT
• 4th decade- chondrosarcoma
• 5th decade- Multiple myeloma
• SEX:- less imp than age
• RACE:- little imp, Ewings rare in african descent
• H/O any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma.
• Various chemical carcinogens- zinc beryllium silicate, beryllium oxide.
• Currently the most worrisome & controversial is Nickel which is used
in many orthopedic devices.
LABORATORY TEST
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Alkaline phosphatase – Higher .

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PTH test: Lower.

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Serum phosphorus: Higher

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Ionized calcium and serum calcium: Higher .
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INVESTIGATIONS

X-RAY
CT SCAN
MRI
TECHNETIUM BONE SCAN-This type of scan uses a
very low radioactive material (diphosphonate) to see
whether or not the cancer has spread to other
bones and the damage suffered by the bone.
• PET- uses radioactive glucose to locate cancer. This
glucose contains a radioactive atom that is absorbed
by the cancerous cells and then detected by a special
camera.
BIOPSY
• The biopsy is the most conclusive test because it confirms if
the tumor is malignant or benign, the bone cancer type
(primary or secondary bone cancer), and stage.
• According to the tumor size and type (malignant or benign)
and the biopsy's purpose (to remove the entire tumor or only a
small tissue sample), there are two types of biopsies used in
bone cancer diagnosis. These are: needle biopsy and incisional
biopsy.
• 1. Needle biopsy: During this procedure, a small hole is made
in the affected bone and a tissue sample from the tumor is
removed.
• There are two types of needle biopsies:
• Fine needle aspiration: During this procedure, the tissue
sample is removed with a thin needle attached to a syringe.
• Core needle aspiration: During this procedure, the surgeon
removes a small cylinder of tissue sample from the tumor with
a rotating knife like device.
• 2. Incisional biopsy: During this procedure, the surgeon cuts
into the tumor and removes a tissue sample.
BONE FORMING TUMOURS
• Benign:
Osteoma, Osteoid
Osteoma,
• Benign Aggressive:
Osteoblastoma

• Malignant: Osteogenic
Sarcoma
Osteoma
• Benign, asymptomatic, slow-growing
osteogenic lesion.
• Age/Sex: 40-50 yr, M:F = 2:1.
• Bones involved: flat bones of the skull and
face; may protrude in the paranasal sinus.
• Parosteal location.
• Gardner’s syndrome.
Ivory like bony mass
Figure 2: The lesion consists of dense and lamellar cortical
bone with a focal area of active bone modeling.
Figure 3: Photomicrograph of the more solid area of the
lesion to demonstrate the cellular woven character of the
bone.
Osteoid osteoma
• Signs/Symptoms:

• Pain, characteristically more intense at night, relieved by NSAID
and eliminated by excision
• Scoliosis

• Age:
• Sex:

• 10-30 years
• M > F (2:1)

• Anatomic Distribution:

• Nearly every location, most frequent in femur, tibia,( Over 50%)
humerus, bones of hands and feet, vertebrae and fibula
• Metaphysis / Diaphysis (cortical) of long bones
• Vertebral lesions may be associated with scoliosis.
Small central osteolytic nidus surrounded by dense
bone
The small, reddish central nidus is surrounded by a thick layer
of sclerotic bone
Wedge
shaped nidus
which is
surrounded
by dense
sclerotic
bone.
New osteoid and bone formation by plump osteoblasts.
The stroma is cellular and well vascularized
Osteoid osteoma with anastomosing trabeculae of
woven bone
Osteoblastoma
• Also called as Giant osteoid osteoma.
• Osteoblastoma is similar to osteoid osteoma with
more aggressive behavior.
• D/D from osteoid osteoma*Pain
*Absence of reactive bone
* Large size
• Location :

– In spine or major bones of lower extremity
Well differentiated radiopaque/radiolucent lesion
Osteoblastoma.-The histologic appearance is identical
to that of osteoid osteoma.
Recurrent osteoblastoma.-The appearance is similar to
that of osteoid osteoma
Osteosarcoma
• Most frequent primary malignant bone tumour.
• Age/Sex :10-25 yrs & >40 , M:F = 3:2
• Predisposing conditions:
Paget’s disease
Radiation exposure
Chemotherapy
Benign bone lesion
Foreign bodies
Trauma
Codman’s triangle:

“Sunray “ appearance
Tumor is located at the typical metaphyseal site. The tumor
shown in A is largely restricted to bone, whereas that illustrated
in B is accompanied by massive soft tissue extension.
‘skip metastasis’ located in the upper half of the femur. The
primary tumor was located in the lower metaphysis of the same
bone
The malignant bone is more basophilic and has more
irregular borders than the preexisting bone
trabeculae.
Osteosarcoma showing characteristic basophilic thin
trabeculae of neoplastic bone with an appearance that
is reminiscent of fungal hyphae
Lace-like osteoid deposition is very
characteristic of this neoplasm.
Osteoblastic osteosarcoma with finely ramifying matrix
between tumor cells
Microscopic variants
Telangiectatic :
• Blood filled cystic space and thus
radiologically appears as pure lytic lesion.
• Pathological fractures.
• Grossly the lesion simulate aneurysmal
bone cyst.
• Detection of malignant stroma in the septa
that separate the bloody cysts.
Telangiectatic osteosarcoma.
Telangiectatic osteosarcoma.
A The low-power architecture closely simulates the
appearance of an aneurysmal bone cyst
B Malignant osteoid is present in the septa
Telangiectatic osteosarcoma. Spaces containing blood are
separated by septa. The cells appear malignant even at this
level of magnification
Fibrohistiocytic
Small cell variant:
• Uniform small size tumour cells.
• Diffuse pattern of growth.
• Simulate Ewing’s sarcoma and malignant
lymphoma.
Anaplastic
Well differentiated intramedullary
.This tumor is microscopically so bland looking as to be often
underdiagnosed as a benign lesion.
In contrast to fibrous dysplasia

1- this tumor shows radiographic evidence of cortical
destruction.
2-Microscopically, atypia is minimal but still present.
3-The invasive growth pattern.
Variants defined on the basis of topographic,
clinical and radiographic features:
Juxtacortical (parosteal)
• Slightly older age group
• Juxtacortical position in the metaphysis of
long bones.
Juxtacortical osteosarcoma
of upper femur. There is only
minimal involvement of the
cortex

Juxtacortical osteosarcoma
large extracortical component
Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells
grow between irregularly shaped bone trabeculae
Parosteal osteosarcoma-The spindle cells demonstrate
minimal atypia, and the bone appears to arise directly from
the spindle cells.
Periosteal osteosaocoma
• Grows on surface of long bones.
• Upper tibial shaft or femur.
Periosteal osteosarcoma. The white shining appearance
is due to the high content of cartilage
periosteal chondrosarcoma. There is a predominance of
myxochondroid areas
Bone formation in the center of a cartilaginous lobule
in periosteal osteosarcoma
Osteosarcoma of jaw:
• Patients affected are slightly older (average age, 34
years),
• And most lesions show a prominent chondroblastic
component.
• The most common sites of involvement are the body
of the mandible and the alveolar ridge of the maxilla.
Osteosarcoma in Paget’s disease.
• Osteosarcoma are of the polyostotic type
• Pelvis, humerus, femur tibia & skull.
• Large number of osteoclasts alternating with atypical
osteoblast.
Histochemistry, IHC & molecular
genetics
• Strong alkaline phosphatase activity.
• Vimentin, S-100, keratin & EMA.
• Osteonectin, osteocalcin, osteopontin.
Prognostic factors
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Paget’s disease
Irradiation
Specific bone involvement
Multifocality
Various types
Microscopic variants
Serum elevation of alkaline phosphatase
Aneuploidy
Post chemotherapy tumour necrosis
RB gene
HER2/neu expression
P-glycoprotein.
CARTILAGE FORMING TUMOURS
• Benign:
Enchondroma, Periosteal
Chondroma, Osteocho
ndroma.
• Benign Aggressive:
Chondromyxoid
Fibroma, Chondroblast
oma.
• Malignant:
Chondroma
• Benign tumor of mature hyaline cartilage
• Age – 20-50 yrs
• Usually solitary,30% are multiple.
• Bones involved: small bones of hand & feet.
• Asymptomatic, pain & swelling.
Enchondroma is the most common tumor of
the bones of the hand
Enchondromas
• Begin in spongiosa of diaphysis, from which they expand
and thin cortex
• Unusual in ribs and long bones

Juxtacortical Chondroma
• Much less common than enchondroma
• Involve parosteal region of long bone or small bone of
hand or foot
2 syndromes characterized by multiple
chondromas:
• Ollier’s disease
• Maffucci’s syndrome
• Both disorders have 25% risk of malignant transformation to
chondrosarcoma
Maffucci syndrome-Innumerable chondromas are seen
concentrated in the distal aspect of the extremity
Juxta-cortical - The tumor produces a
semispherical expansion of the involved bone.
Enchondroma-The tumor has a typical
lobulated appearance
Osteochondroma
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•

Also known as exostosis.
Most frequent benign cartilaginous tumour.
Age/sex - <20yr, M:F=3:1
Bones involved: lower femur, upper tibia,
upper humerus and pelvis.
• Location: Metaphysis
• Probably not a true neoplasm.

• Inactivation of both copies of the EXT gene in
the growth plate chondrocytes.
• Presents as slow growing mass, painful.

• <1% cases show malignant transformation.
A- Large osteochondroma of femur with a bilobed appearance.
B Cut surface of osteochondroma of ribthick cartilaginous cup
Projection with cortex continuous with underlying bone;
may be pedunculated; cartilaginous cap with frequent
calcification
Microscopic-Mature bone is covered by a welldifferentiated cartilaginous cap.
Microscopic
Chondroblastoma
• Rare benign cartilage producing tumour.
• Age/Sex : 2nd decade M:F=2:1.
• Bones involved: Distal femur ,proximal
humerus and tibia.
• Location: Epiphysis
• Pain is constant .
Typical sharply delineated lytic appearance of
chondroblastoma of humeral head
Gross appearance of chondroblastoma of upper end of the
humerus, associated with aneurysmal bone cyst-like changes
Chondroblastoma. A- Small tumor cells of round shape are
accompanied by scattered osteoclasts.
B-Immunoreactivity for S-100 protein in the neoplastic
component.
Chondroblast: Prominent Indented Nucleus
Eosinophilic Cytoplasm Thick Cell Membrane
Uniform Appearance of Cells
Chondroblastoma with eosinophilic chondroid matrix,
giant cells, and mononuclear cells
Imagine the cells present without the nuclei: The thickened cell membranes
would give a chicken wire fence appearance
Chondromyxoid Fibroma
• Benign tumour of cartilaginous origin.
• Age/Sex: 20-30 yr/ M:F=2:1
• Bones involved: Long bones>flat bones
>vertebrae.
• Location: Metaphysis.
• Localised pain with or without tenderness
Sharply delimited chondromyxoid fibroma of lower
femoral metaphysis in a young boy.
A-Chondromyxoid fibroma of proximal femur extending into
soft tissue
B-The tumor has a lobulated appearance, in which
myxochondroid islands alternate with more cellular foci.
Chondromyxoid fibroma- (A) An irregularly shaped
hypocellular center is surrounded by a cellular spindle
cell stroma.
(B) The lobules contain tumor cells with small nuclei
and eosinophilic cytoplasmic extensions within a
myxoid background
Chondrosarcoma
• Second most common malignant tumour of bones.
• Arise de novo or from pre-existing benign
cartilagenous tumour.
• Divided into two major categories:
*Conventional chondrosarcoma
*Chondrosarcoma variants
Conventional chondrosarcoma
• 30 – 60 yr of age.
• M>F
• Divided according to location:
*Central
*Peripheral
*Juxtacortical
Typical chondrosarcoma of femurI-ill defined margins;
fusiform thickening of shaft; perforation of cortex
Typical chondrosarcoma
Peripheral Variant:
• Tumour is present on the surface of bone.
• May arise de-novo or from cartilaginous cap
of preexisting osteochondroma.
Peripheral chondrosarcoma of femur resulting in a huge
exophytic mass
Juxtacortical(periosteal) variant:
• Location:
– shaft of long bone (most often femur)

• Cartilaginous lobular pattern with areas of:
– spotty calcification
– endochondral ossification

• Closely related to periosteal osteosarcoma.
Microscopic
• Wide range of differentiation and graded into:
– well differentiated
– moderately differentiated
– poorly differentiated
Well-differentiated chondrosarcoma. The tumor has a
distinctly lobulated quality
Well differentiated- High-power appearance of grade 1
chondrosarcoma. A few doubly nucleated cells and
moderate atypia .
Moderately differentiated - High-power
appearance of grade 2 chondrosarcoma with necrosis .
The nuclei are crowded and hyperchromatic
Poorly differentiated
Grading system
• Grade I : lesions contain hyaline cartilage manifested by
sparse cellularity. The cells typically contain dark, pyknotic
nuclei. <20% of cells contain large nuclei and fine nuclear
chromatin. Mitosis is absent.
• Grade II: A) lesion are slightly more cellular and >20% nuclei
are larger than nucleus of mature lymphocyte. Binucleate
cells are easily found. Mitosis is absent.
B) cellular lesions with numerous binucleated cells and
nuclear atypia. Mitosis is present but not more than
1/ 10hpf.
• Grade III: Mitosis atleast >=2 / 10 hpf
• The main differential is of low grade (Grade 1)
chondrosarcoma and enchondroma.
• Features consistent with chondrosarcoma are:
*Pain attributable to lesion
*Age greater than 50
*Cortical destruction and a soft tissue mass
*Periosteal reaction and thickening
*Endosteal erosion>2/3 cortical thickness on a CT scan
*Size greater than 5 cm
Chondrosarcoma variants
Dedifferentiated chondrosarcoma:
• Worst prognosis.
• Age/sex: sixth decade/ M:F =1:1.
• Bones involved: pelvis, femur.
• Poorly differentiated sarcomatous component
at periphery of otherwise typical low-grade
chondrosarcoma
– usually central type
– can be peripheral
Gross appearance of dedifferentiated
chondrosarcoma of pelvic bone -
Microscopic
• Dedifferentiation:
– may be in initial lesion
– more often in specimens from recurrent tumor:
• microscopic appearance of this component may be:
–
–
–
–

rhabdomyosarcoma
fibrosarcoma
osteosarcoma
pleomorphic sarcoma with MFH-like features
The edge of an island of well-differentiated cartilage (upper left) is
surrounded by highly pleomorphic sarcoma containing tumor giant
cells
Chondrosarcoma is juxtaposed with high-grade
malignant fibrous histiocytoma
Clear cell variant:
• Behaves as low-grade malignancy
• Can undergo dedifferentiation
• Age/Sex : 30-40 yrs/M:F= 2.5:1
• Location: proximal end of femur and humerus.
Clear cell chondrosarcoma with faint lobulation, woven
bone, and clear cells
Mesenchymal variant:
• Usually second or third decade of life
• Great variability in clinical course.
• Location:
– most commonly:
• jaw
• pelvis
• femur
• ribs
• spine
Shows an island of well-differentiated cartilage in the
center
Cellular, hemangiopericytoma-like component
TUMOUR

LOCATION

AGE/M/F

SALIENT PATHOLOGIC FINDING

OSTEOMA

FACIAL
BONE

40-50/2:1

MINERALIZED COMPACT BONE

OSTEOID
OSTEOMA

CORTEX OF 10-30/2:1
LB

OSTEOBLAS VERTEBRA
TOMA
E,CORTEX
OF LB

10-30/2:1

OSTEOSARC METAPHYS
OMA
IS OF LB
ACONVENTI
ONAL .
B-LOW
GRADE
CENTRAL

“NIDUS” OF IMMATURE BONE SURROUNDED BY
SCLEROTIC BONE.

IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND
OFTEN NO SCLEROSIS

10-25/3:2
A
-OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS

-B- MILDLY ATYPICAL FIBROBLASTIC
PROLIFERATION+THICK BONE TRABECULAE
TUMOUR

LOCATION

CTELANGIEC
TATIC

METAPHYS
IS

BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY
MALIGNANT OSTEOID

DPAROSTEAL

CORTEX
30-60 YRS
OUTSIDE
PERIOSTEU
M

MILDLY ATYPICAL FIBROBLASTIC
PROLIFERATION+THICK BONE TRABECULAE

ECORTEX
PERIOSTEAL INSIDE
PERIOSTEU
M

AGE/M/F

SALIENT PATHOLOGIC FINDING

ABUNDAND CARTILAGE MATRIX +VARIABLE
MALIGNANT OSTEOID
TUMOUR

LOCATION

AGE/M/F

SALIENT PATHOLOGIC FINDING

CHONDRO
MA

HANDS
,FEET,RIBS
,FEMUR

10-40/1:1

VARIABLY CELLULAR HYLINE CARTILAGE

OSTEOCHO
NDROMA

METAPHYS
ISOF LBS

10-30/1:1

CARTILAGE CAPPED BONY PROTRUSION

CHONDROB EPIPHYSIS
LASTOMA
OF LBS

10-20/2:1

CHONDROID LIKE MATRIX, S-100 POSITIVE CELLS
WITH GROOVED NUCLEI

CHONDRO
MYXOID
FIBROMA

10-30/1:1

HYPOCELLULAR CHONDROMYXOID LOBULES
SURROUNDED BY MORE CELLULAR SPINDLE CELL
AREAS

METAPHYS
IS OF LBS
TUMOUR

LOCATION

AGE/M/F

CHONDROSAR PELIVIS.
COMA
RIBS
,FEMUR
ACONVENTION
AL

SALIENT PATHOLOGIC FINDING

/3:1

METAPHYSIS 20-80

VARIABLY CELLULAR HYLINE CARTILAGE WITH
PERMEATING BONE

BMETAPHYSIS >30
DEDIFFERENTI
ATED

CONVENTIONAL CHONDROSARCOMA
+HIGH GRADE SPINDLE CELL SARCOMA

CMESENCHYM
AL

20-50
METAPHYSIS

UNDIFFERENTIATED SMALL TUMOUR CELLS
WITH +HYLINE CARTILAGE

E- CLEAR CELL

EPIPHYSIS

20-70

CONVENTIONAL CHONDROSARCOMA
+ABUNDANT LARGE CLEAR CELLS
THANKYOU

PRESENTED BY – DR NARMADA PRASAD TIWARI

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Bone tumours by dr narmada prasad tiwari

  • 2. • A bone tumor, is a neoplastic growth of tissue in bone. Abnormal growths found in the bone can be either benign or malignant.
  • 3. • • • • Bones are classified according to their shapeLong bone Flat bone Short bone.
  • 4. • • • • • • • Long bone anatomy Diaphysis: long shaft of bone Epiphysis: ends of bone Epiphyseal plate: growth plate Metaphysis: b/w epiphysis and diaphysis Articular cartilage: covers epiphysis Periosteum: bone covering (pain sensitive) Sharpey’s fibers: periosteum attaches to underlying bone • Medullary cavity: Hollow chamber in bone - red marrow produces blood cells - yellow marrow is adipose. • Endosteum: thin layer lining the medullary cavity
  • 8. • Histology of bone tissue Cells are surrounded by matrix. - 25% water - 25% protein - 50% mineral salts 4 cell types make up osseous tissue Osteoprogenitor cells Osteoblasts Osteocytes Osteoclasts
  • 9. • Osteoprogenitor cells: - derived from mesenchyme - unspecialized stem cells - undergo mitosis and develop into osteoblasts - found on inner surface of periosteum and endosteum.
  • 10. Osteoblasts: - bone forming cells - found on surface of bone - no ability to mitotically divide - collagen secretors Osteocytes: - mature bone cells - derived form osteoblasts - do not secrete matrix material - cellular duties include exchange of nutrients and waste with blood
  • 11. • Osteoclasts - bone resorbing cells - bone surface - growth, maintenance and bone repair Abundant inorganic mineral salts: - Tricalcium phosphate in crystalline form called hydroxyapatite Ca3(PO4)2(OH)2 - Calcium Carbonate: CaCO3 - Magnesium Hydroxide: Mg(OH)2 - Fluoride and Sulfate
  • 14. Precursors of malignancy in bone • • • • • • • • • High Risk Ollier disease (Enchondromatosis) and Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis
  • 15. • • • • • • • • Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
  • 16.
  • 17. Classification of primary tumour involving bones Histological type Hematopoietic Chondrogenic Benign Malignant Myeloma Osteochodroma Chondroma Chondroblastoma Chondromyxoid fibroma Malignant lymphoma chondrosarcoma
  • 19. Histological type Benign Malignant Histiocytic origin Fibrous histiocytoma MFH Fibrogenic Metaphyseal fibrous Dysplastic fibroma defect Fibrosarcoma Notochordal Vascular Chordoma Hemangioma Hemangioendothelioma Hemangiopericytoma Lipogenic Lipoma Neurogenic Neurilemmoma Liposarcoma
  • 20. Distribution of bone tumors in long bones • Epiphyseal lesions: • Chondroblastoma • Giant cell tumor • • • • Metaphyseal intramedullary lesions: Osteosarcoma Chondrosarcoma Aneurysmal bone cyst
  • 21. • Metaphyseal lesions centered in the cortex: • Nonossifying fibroma (NOF) • Osteoid osteoma • Metaphyseal exostosis: • Osteochondroma
  • 22. • • • • • • Diaphyseal intramedullary lesions: Ewing’s sarcoma Lymphoma Myeloma Fibrous dysplasia Enchondroma • • • Diaphyseal lesions centered in the cortex: Adamantinoma Osteoid osteoma
  • 24.
  • 25. Important Facts • 0.001% of all cancers • MC benign tumor--- Osteochondroma; Osteoid Osteoma • MC Skeletal malignancy– Metastasis. • MC Bone tumor in Pediatric age group & adultsOsteosarcoma • MC in < 10 y--- Ewing’s sarcoma • MC Primary bone tumor – Multiple Myeloma
  • 26. PRESENTING SYMPTOMS • • • • • • • • Patient may present with An abnormal radiographic finding detected during evaluation of unrelated problem PAIN:- is most frequent symptom MASS:- rate of enlargement is important -Fluctuating mass can be cyst,ganglion or hemangioma -Family H/O masses near the joint may be indicator of Ollier’s disease or Maffucci Syndrome NEUROLOGICAL SYMPTOM:- found in few patients such as sacral tumors & with tumors located near the nerve causing compression of nerve,especially common in sciatic notch ,inguinal canal & popliteal fossa UNEXPLAINED SWELLING OF THE LOWER EXTREMITY:- found in pelvic tumors which are painless & without a palpable mass & cause swelling due to compression of iliac vein.
  • 27. PHYSICAL EXAMINATION • Evaluation of patient’s general health • TUMOR MASS should be measured & its location,shape, consistency,mobility,tenderness,local temp & change with position should be noted. • SKIN & SUBCUTANEOUS TISSUE : • Small dialated superficial veins overlying the mass are produced by large tumors • Café-au-lait spots & subcutaneous neurofibromas indicate Von Recklinghausen’s disease • A venous malformation Maffucci Syndrome • REGIONAL LYMPH NODES: sign of metastatic disease • Atrophy of surrounding musculature should be recorded,also neurological deficits & adequacy of circulation.
  • 28. HISTORY OF THE PATIENT • AGE:- most imp information,bcoz of their presentaion in sp age group. • 1st decade- usually ABC ,SBC • 2nd decade-Chondroblastoma,osteosarcoma,Ewings • 3rd decade- GCT • 4th decade- chondrosarcoma • 5th decade- Multiple myeloma • SEX:- less imp than age • RACE:- little imp, Ewings rare in african descent • H/O any exposure to radiation Tt or Carcinogens- bone seeking radionucleotide can cause sarcoma. • Various chemical carcinogens- zinc beryllium silicate, beryllium oxide. • Currently the most worrisome & controversial is Nickel which is used in many orthopedic devices.
  • 29. LABORATORY TEST • Alkaline phosphatase – Higher . • PTH test: Lower. • Serum phosphorus: Higher • Ionized calcium and serum calcium: Higher .
  • 30. • • • • INVESTIGATIONS X-RAY CT SCAN MRI TECHNETIUM BONE SCAN-This type of scan uses a very low radioactive material (diphosphonate) to see whether or not the cancer has spread to other bones and the damage suffered by the bone. • PET- uses radioactive glucose to locate cancer. This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special camera.
  • 31. BIOPSY • The biopsy is the most conclusive test because it confirms if the tumor is malignant or benign, the bone cancer type (primary or secondary bone cancer), and stage. • According to the tumor size and type (malignant or benign) and the biopsy's purpose (to remove the entire tumor or only a small tissue sample), there are two types of biopsies used in bone cancer diagnosis. These are: needle biopsy and incisional biopsy. • 1. Needle biopsy: During this procedure, a small hole is made in the affected bone and a tissue sample from the tumor is removed. • There are two types of needle biopsies: • Fine needle aspiration: During this procedure, the tissue sample is removed with a thin needle attached to a syringe. • Core needle aspiration: During this procedure, the surgeon removes a small cylinder of tissue sample from the tumor with a rotating knife like device. • 2. Incisional biopsy: During this procedure, the surgeon cuts into the tumor and removes a tissue sample.
  • 32. BONE FORMING TUMOURS • Benign: Osteoma, Osteoid Osteoma, • Benign Aggressive: Osteoblastoma • Malignant: Osteogenic Sarcoma
  • 33. Osteoma • Benign, asymptomatic, slow-growing osteogenic lesion. • Age/Sex: 40-50 yr, M:F = 2:1. • Bones involved: flat bones of the skull and face; may protrude in the paranasal sinus. • Parosteal location. • Gardner’s syndrome.
  • 35. Figure 2: The lesion consists of dense and lamellar cortical bone with a focal area of active bone modeling. Figure 3: Photomicrograph of the more solid area of the lesion to demonstrate the cellular woven character of the bone.
  • 36. Osteoid osteoma • Signs/Symptoms: • Pain, characteristically more intense at night, relieved by NSAID and eliminated by excision • Scoliosis • Age: • Sex: • 10-30 years • M > F (2:1) • Anatomic Distribution: • Nearly every location, most frequent in femur, tibia,( Over 50%) humerus, bones of hands and feet, vertebrae and fibula • Metaphysis / Diaphysis (cortical) of long bones • Vertebral lesions may be associated with scoliosis.
  • 37. Small central osteolytic nidus surrounded by dense bone
  • 38. The small, reddish central nidus is surrounded by a thick layer of sclerotic bone
  • 40. New osteoid and bone formation by plump osteoblasts. The stroma is cellular and well vascularized
  • 41. Osteoid osteoma with anastomosing trabeculae of woven bone
  • 42. Osteoblastoma • Also called as Giant osteoid osteoma. • Osteoblastoma is similar to osteoid osteoma with more aggressive behavior. • D/D from osteoid osteoma*Pain *Absence of reactive bone * Large size • Location : – In spine or major bones of lower extremity
  • 44. Osteoblastoma.-The histologic appearance is identical to that of osteoid osteoma.
  • 45. Recurrent osteoblastoma.-The appearance is similar to that of osteoid osteoma
  • 46. Osteosarcoma • Most frequent primary malignant bone tumour. • Age/Sex :10-25 yrs & >40 , M:F = 3:2 • Predisposing conditions: Paget’s disease Radiation exposure Chemotherapy Benign bone lesion Foreign bodies Trauma
  • 47.
  • 49. Tumor is located at the typical metaphyseal site. The tumor shown in A is largely restricted to bone, whereas that illustrated in B is accompanied by massive soft tissue extension.
  • 50. ‘skip metastasis’ located in the upper half of the femur. The primary tumor was located in the lower metaphysis of the same bone
  • 51. The malignant bone is more basophilic and has more irregular borders than the preexisting bone trabeculae.
  • 52. Osteosarcoma showing characteristic basophilic thin trabeculae of neoplastic bone with an appearance that is reminiscent of fungal hyphae
  • 53. Lace-like osteoid deposition is very characteristic of this neoplasm.
  • 54.
  • 55. Osteoblastic osteosarcoma with finely ramifying matrix between tumor cells
  • 56. Microscopic variants Telangiectatic : • Blood filled cystic space and thus radiologically appears as pure lytic lesion. • Pathological fractures. • Grossly the lesion simulate aneurysmal bone cyst. • Detection of malignant stroma in the septa that separate the bloody cysts.
  • 58. Telangiectatic osteosarcoma. A The low-power architecture closely simulates the appearance of an aneurysmal bone cyst B Malignant osteoid is present in the septa
  • 59. Telangiectatic osteosarcoma. Spaces containing blood are separated by septa. The cells appear malignant even at this level of magnification
  • 61. Small cell variant: • Uniform small size tumour cells. • Diffuse pattern of growth. • Simulate Ewing’s sarcoma and malignant lymphoma.
  • 63. Well differentiated intramedullary .This tumor is microscopically so bland looking as to be often underdiagnosed as a benign lesion. In contrast to fibrous dysplasia 1- this tumor shows radiographic evidence of cortical destruction. 2-Microscopically, atypia is minimal but still present. 3-The invasive growth pattern.
  • 64. Variants defined on the basis of topographic, clinical and radiographic features: Juxtacortical (parosteal) • Slightly older age group • Juxtacortical position in the metaphysis of long bones.
  • 65. Juxtacortical osteosarcoma of upper femur. There is only minimal involvement of the cortex Juxtacortical osteosarcoma large extracortical component
  • 66. Juxtacortical osteosarcoma--Moderately atypical spindle tumor cells grow between irregularly shaped bone trabeculae
  • 67. Parosteal osteosarcoma-The spindle cells demonstrate minimal atypia, and the bone appears to arise directly from the spindle cells.
  • 68. Periosteal osteosaocoma • Grows on surface of long bones. • Upper tibial shaft or femur.
  • 69. Periosteal osteosarcoma. The white shining appearance is due to the high content of cartilage
  • 70. periosteal chondrosarcoma. There is a predominance of myxochondroid areas
  • 71. Bone formation in the center of a cartilaginous lobule in periosteal osteosarcoma
  • 72. Osteosarcoma of jaw: • Patients affected are slightly older (average age, 34 years), • And most lesions show a prominent chondroblastic component. • The most common sites of involvement are the body of the mandible and the alveolar ridge of the maxilla. Osteosarcoma in Paget’s disease. • Osteosarcoma are of the polyostotic type • Pelvis, humerus, femur tibia & skull. • Large number of osteoclasts alternating with atypical osteoblast.
  • 73. Histochemistry, IHC & molecular genetics • Strong alkaline phosphatase activity. • Vimentin, S-100, keratin & EMA. • Osteonectin, osteocalcin, osteopontin.
  • 74. Prognostic factors • • • • • • • • • • • • Paget’s disease Irradiation Specific bone involvement Multifocality Various types Microscopic variants Serum elevation of alkaline phosphatase Aneuploidy Post chemotherapy tumour necrosis RB gene HER2/neu expression P-glycoprotein.
  • 75. CARTILAGE FORMING TUMOURS • Benign: Enchondroma, Periosteal Chondroma, Osteocho ndroma. • Benign Aggressive: Chondromyxoid Fibroma, Chondroblast oma. • Malignant:
  • 76. Chondroma • Benign tumor of mature hyaline cartilage • Age – 20-50 yrs • Usually solitary,30% are multiple. • Bones involved: small bones of hand & feet. • Asymptomatic, pain & swelling. Enchondroma is the most common tumor of the bones of the hand
  • 77. Enchondromas • Begin in spongiosa of diaphysis, from which they expand and thin cortex • Unusual in ribs and long bones Juxtacortical Chondroma • Much less common than enchondroma • Involve parosteal region of long bone or small bone of hand or foot
  • 78. 2 syndromes characterized by multiple chondromas: • Ollier’s disease • Maffucci’s syndrome • Both disorders have 25% risk of malignant transformation to chondrosarcoma
  • 79. Maffucci syndrome-Innumerable chondromas are seen concentrated in the distal aspect of the extremity
  • 80. Juxta-cortical - The tumor produces a semispherical expansion of the involved bone.
  • 81. Enchondroma-The tumor has a typical lobulated appearance
  • 82. Osteochondroma • • • • Also known as exostosis. Most frequent benign cartilaginous tumour. Age/sex - <20yr, M:F=3:1 Bones involved: lower femur, upper tibia, upper humerus and pelvis. • Location: Metaphysis
  • 83. • Probably not a true neoplasm. • Inactivation of both copies of the EXT gene in the growth plate chondrocytes. • Presents as slow growing mass, painful. • <1% cases show malignant transformation.
  • 84. A- Large osteochondroma of femur with a bilobed appearance. B Cut surface of osteochondroma of ribthick cartilaginous cup
  • 85. Projection with cortex continuous with underlying bone; may be pedunculated; cartilaginous cap with frequent calcification
  • 86. Microscopic-Mature bone is covered by a welldifferentiated cartilaginous cap.
  • 88. Chondroblastoma • Rare benign cartilage producing tumour. • Age/Sex : 2nd decade M:F=2:1. • Bones involved: Distal femur ,proximal humerus and tibia. • Location: Epiphysis • Pain is constant .
  • 89. Typical sharply delineated lytic appearance of chondroblastoma of humeral head
  • 90. Gross appearance of chondroblastoma of upper end of the humerus, associated with aneurysmal bone cyst-like changes
  • 91. Chondroblastoma. A- Small tumor cells of round shape are accompanied by scattered osteoclasts. B-Immunoreactivity for S-100 protein in the neoplastic component.
  • 92. Chondroblast: Prominent Indented Nucleus Eosinophilic Cytoplasm Thick Cell Membrane Uniform Appearance of Cells
  • 93. Chondroblastoma with eosinophilic chondroid matrix, giant cells, and mononuclear cells
  • 94. Imagine the cells present without the nuclei: The thickened cell membranes would give a chicken wire fence appearance
  • 95. Chondromyxoid Fibroma • Benign tumour of cartilaginous origin. • Age/Sex: 20-30 yr/ M:F=2:1 • Bones involved: Long bones>flat bones >vertebrae. • Location: Metaphysis. • Localised pain with or without tenderness
  • 96. Sharply delimited chondromyxoid fibroma of lower femoral metaphysis in a young boy.
  • 97. A-Chondromyxoid fibroma of proximal femur extending into soft tissue B-The tumor has a lobulated appearance, in which myxochondroid islands alternate with more cellular foci.
  • 98. Chondromyxoid fibroma- (A) An irregularly shaped hypocellular center is surrounded by a cellular spindle cell stroma. (B) The lobules contain tumor cells with small nuclei and eosinophilic cytoplasmic extensions within a myxoid background
  • 99. Chondrosarcoma • Second most common malignant tumour of bones. • Arise de novo or from pre-existing benign cartilagenous tumour. • Divided into two major categories: *Conventional chondrosarcoma *Chondrosarcoma variants
  • 100. Conventional chondrosarcoma • 30 – 60 yr of age. • M>F • Divided according to location: *Central *Peripheral *Juxtacortical
  • 101. Typical chondrosarcoma of femurI-ill defined margins; fusiform thickening of shaft; perforation of cortex
  • 103. Peripheral Variant: • Tumour is present on the surface of bone. • May arise de-novo or from cartilaginous cap of preexisting osteochondroma.
  • 104. Peripheral chondrosarcoma of femur resulting in a huge exophytic mass
  • 105. Juxtacortical(periosteal) variant: • Location: – shaft of long bone (most often femur) • Cartilaginous lobular pattern with areas of: – spotty calcification – endochondral ossification • Closely related to periosteal osteosarcoma.
  • 106. Microscopic • Wide range of differentiation and graded into: – well differentiated – moderately differentiated – poorly differentiated
  • 107. Well-differentiated chondrosarcoma. The tumor has a distinctly lobulated quality
  • 108. Well differentiated- High-power appearance of grade 1 chondrosarcoma. A few doubly nucleated cells and moderate atypia .
  • 109. Moderately differentiated - High-power appearance of grade 2 chondrosarcoma with necrosis . The nuclei are crowded and hyperchromatic
  • 111. Grading system • Grade I : lesions contain hyaline cartilage manifested by sparse cellularity. The cells typically contain dark, pyknotic nuclei. <20% of cells contain large nuclei and fine nuclear chromatin. Mitosis is absent. • Grade II: A) lesion are slightly more cellular and >20% nuclei are larger than nucleus of mature lymphocyte. Binucleate cells are easily found. Mitosis is absent. B) cellular lesions with numerous binucleated cells and nuclear atypia. Mitosis is present but not more than 1/ 10hpf. • Grade III: Mitosis atleast >=2 / 10 hpf
  • 112. • The main differential is of low grade (Grade 1) chondrosarcoma and enchondroma. • Features consistent with chondrosarcoma are: *Pain attributable to lesion *Age greater than 50 *Cortical destruction and a soft tissue mass *Periosteal reaction and thickening *Endosteal erosion>2/3 cortical thickness on a CT scan *Size greater than 5 cm
  • 113. Chondrosarcoma variants Dedifferentiated chondrosarcoma: • Worst prognosis. • Age/sex: sixth decade/ M:F =1:1. • Bones involved: pelvis, femur. • Poorly differentiated sarcomatous component at periphery of otherwise typical low-grade chondrosarcoma – usually central type – can be peripheral
  • 114. Gross appearance of dedifferentiated chondrosarcoma of pelvic bone -
  • 115. Microscopic • Dedifferentiation: – may be in initial lesion – more often in specimens from recurrent tumor: • microscopic appearance of this component may be: – – – – rhabdomyosarcoma fibrosarcoma osteosarcoma pleomorphic sarcoma with MFH-like features
  • 116. The edge of an island of well-differentiated cartilage (upper left) is surrounded by highly pleomorphic sarcoma containing tumor giant cells
  • 117. Chondrosarcoma is juxtaposed with high-grade malignant fibrous histiocytoma
  • 118. Clear cell variant: • Behaves as low-grade malignancy • Can undergo dedifferentiation • Age/Sex : 30-40 yrs/M:F= 2.5:1 • Location: proximal end of femur and humerus.
  • 119. Clear cell chondrosarcoma with faint lobulation, woven bone, and clear cells
  • 120. Mesenchymal variant: • Usually second or third decade of life • Great variability in clinical course. • Location: – most commonly: • jaw • pelvis • femur • ribs • spine
  • 121. Shows an island of well-differentiated cartilage in the center
  • 123. TUMOUR LOCATION AGE/M/F SALIENT PATHOLOGIC FINDING OSTEOMA FACIAL BONE 40-50/2:1 MINERALIZED COMPACT BONE OSTEOID OSTEOMA CORTEX OF 10-30/2:1 LB OSTEOBLAS VERTEBRA TOMA E,CORTEX OF LB 10-30/2:1 OSTEOSARC METAPHYS OMA IS OF LB ACONVENTI ONAL . B-LOW GRADE CENTRAL “NIDUS” OF IMMATURE BONE SURROUNDED BY SCLEROTIC BONE. IDENTICAL TO OSTEOID OSTEOMA BUT LARGER AND OFTEN NO SCLEROSIS 10-25/3:2 A -OSTEOID FORMED DIRECTLY BY MALIGNAT CELLS -B- MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE
  • 124. TUMOUR LOCATION CTELANGIEC TATIC METAPHYS IS BLOOD FILLED SPACE+FIBROUS SEPTAE+HIGHLY MALIGNANT OSTEOID DPAROSTEAL CORTEX 30-60 YRS OUTSIDE PERIOSTEU M MILDLY ATYPICAL FIBROBLASTIC PROLIFERATION+THICK BONE TRABECULAE ECORTEX PERIOSTEAL INSIDE PERIOSTEU M AGE/M/F SALIENT PATHOLOGIC FINDING ABUNDAND CARTILAGE MATRIX +VARIABLE MALIGNANT OSTEOID
  • 125. TUMOUR LOCATION AGE/M/F SALIENT PATHOLOGIC FINDING CHONDRO MA HANDS ,FEET,RIBS ,FEMUR 10-40/1:1 VARIABLY CELLULAR HYLINE CARTILAGE OSTEOCHO NDROMA METAPHYS ISOF LBS 10-30/1:1 CARTILAGE CAPPED BONY PROTRUSION CHONDROB EPIPHYSIS LASTOMA OF LBS 10-20/2:1 CHONDROID LIKE MATRIX, S-100 POSITIVE CELLS WITH GROOVED NUCLEI CHONDRO MYXOID FIBROMA 10-30/1:1 HYPOCELLULAR CHONDROMYXOID LOBULES SURROUNDED BY MORE CELLULAR SPINDLE CELL AREAS METAPHYS IS OF LBS
  • 126. TUMOUR LOCATION AGE/M/F CHONDROSAR PELIVIS. COMA RIBS ,FEMUR ACONVENTION AL SALIENT PATHOLOGIC FINDING /3:1 METAPHYSIS 20-80 VARIABLY CELLULAR HYLINE CARTILAGE WITH PERMEATING BONE BMETAPHYSIS >30 DEDIFFERENTI ATED CONVENTIONAL CHONDROSARCOMA +HIGH GRADE SPINDLE CELL SARCOMA CMESENCHYM AL 20-50 METAPHYSIS UNDIFFERENTIATED SMALL TUMOUR CELLS WITH +HYLINE CARTILAGE E- CLEAR CELL EPIPHYSIS 20-70 CONVENTIONAL CHONDROSARCOMA +ABUNDANT LARGE CLEAR CELLS
  • 127. THANKYOU PRESENTED BY – DR NARMADA PRASAD TIWARI