3. CHONDROSARCOMA
• Comprises a group of trs with the common
feature being the production of neoplastic
cartilage.
4. CHONDROSARCOMA(CS)
• 3rd most common malignant bone tumor
(myeloma & OS).
• Age 40 yr or older (adults with mature
skeletons). M: F ratio is 2:1.
• Arise in central portions of skeleton including
pelvis, shoulder, and ribs/proximal parts of
tubular bones of the limbs.
• Painful, progressive enlarging masses.
• Rarely involves the distal extremities in contrast
to enchondromas.
5. SUBTYPES OF CS
• ACCORDING TO SITE:
Intramedullary (Central)
Juxtacortical ( Surface)
Extraskeletal Soft Tissue Chondrosarcoma
(Mesencymal type).
• ACCORDING TO HISTOLOGY:
Conventional (or myxoid/hyaline CS)
Clear cell CS
Dedifferentiated CS
Mesenchymal CS
. PRIMARY (DE-NOVO)
.SECONDARY (EXOSTOSIS or OLLIERS DISEASE).
.
6. • A) CONVENTIONAL CS: GROSS
- Composed of malignant hyaline & myxoid
cartilage.
- Tends to involve the large flat bones (pelvic-periacetabular
region and shoulder girdle) of
adults.
- Metaphysis or proximal diaphysis of long tubular
bones. Distal parts are not involved.
- Can also involve the nasal & laryngeal bones.
- Not seen in the small bones of the hands & feet).
7. RADIOLOGICAL FEATURES
• Metaphysis or diaphysis of long bones.
• Large LYTIC lesions with radiodense
stippling, curlicues, rings due to matrix
calcifications (spotchy calcification &
ossification).
• Triad 1) Bone expansion (fusiform)
2) Cortical thickening.
3) Cortical erosion with permeation
into surrounding soft tissue.
8. GROSS:
- Large bulky trs made up of nodules of gray-white,
translucent somewhat glistening tissue.
- Foci of calcification, myxoid change, cyst
formation.
- ***Malignant cartilage infiltrates the marrow
spaces & surrounds pre-existing bony
trabeculae,
- Adjacent cortex is eroded or thickened & tr
infiltrates into the surrounding tissues,
9. MICROSCOPIC PICTURE
CONVENTIONAL CS
• Vary in degree of cellularity, cytologic
atypia, mitotic activity
• Three grades: 1-3
• Low grade or grade 1 CS : Mild
hypercellularity, scattered chondrocytes
with plump vesicular nuclei, small nucleoli,
few binucleate cells, low mitosis.
• Grade 3 : marked hypercellularity,
extreme pleomorphism, bizarre tumor
giant cells, frequent mitosis & necrosis.
10. • B) DEDIFFERENTIATED CS:(1O%).
- comprises of a “mixture” of low grade
chondrosarcoma adjacent to a poorly
differentiated high grade sarcoma such
as MFH, fibrosarcoma , or osteosarcoma.
D/D: Chondroblastic OS
11. • C) CLEAR CELL SC:
- Large malignant chondrocytes cells with
abundant clear cytoplasm, well defined cell
outlines, centrally placed nucleus with a
prominent nucleolus.
- Cells arranged in lobules.
- Osteoclast like giant cells (edge of lobule) &
woven bone trabeculae (centre of lobule).
D/D :
12. • D) MESENCHYMAL CS:
- 1/3 cases are seen in soft tissues.
- Young adults.
- Jaw bones & ribs.
- Islands of W.D hyaline cartilage surrounded by
sheets of round blue cells having
hemangiopericytoma like b.v.
- Biphasic tumour
- D/D:
30. PROGNOSIS OF CS.
• 5 yr survival is 90%, 81%, and 43% for
grade 1-3 respectively
• Size :>10cm poor prognosis
• Metastasis
31. CLINICAL FEATURES
• Painful progressive enlarging trs.
• Spread occurs to lungs & skeleton.
32. 2. OSTEOCHONDROMA
(EXOSTOSIS)
• Benign cartilage-capped outgrowth
attached to underlying bone by stalk
• Usually single
• Multiple in hereditary exostosis
33. • Solitary: in late adolescence & early
adulthood
• Multiple : in childhood
• Male : Female ratio is 3:1
• Site : arises from metaphysis of long
bones esp. about knee.
34. MORPHOLOGY
• Mushroom shaped outgrowth from surface
of a bone,
• Size : 1-20cm
• Cap of benign hyaline cartilage of variable
thickness with inner bony head & stalk
35. CLINICAL PRESENTATION
• Asymptomatic slow growing tumors
• Can be painful when impinge on nerve or
stalk is fractured
• Epiphyseal growth disturbances in multiple
exostosis
• Rarely development of chondrosarcoma
42. • ENCHONDROMA: within medullary cavity
• SUBPERIOSTEAL OR
JUXTACORTICAL CHONDROMA:
Present on surface of bone (humerus
50%)
• SOFT TISSUE CHONDROMAS.
43. ENCHONDROMA
• The most common intraosseous cartilage tumor.
• Age: 20-50 yr
• Solitary lesions
• Site : metaphyseal region of short tubular
bones of hands & feet
• OLLIER DISEASE: multiple enchondromas.
• 25% of pat with Ollier Disease dev
Chondrosarcoma
• MAFFUCCI SYNDROME: enchondromas with
soft tissue hemangiomas
• Risk of malignant trs is more in Maffucci synd.
44. MORPHOLOGY
• Size: less than 3 cm
• Gross: nodular grey blue translucent mass
• Microscopically:-
- Well circumscribed lesions.
- Hyaline matrix.
- Benign chondrocytes within lacunae.
- Ossification & calcification are frequent.
- chondromas of small bones of hands &
feet tend to hypercellular (resembling
grade 1 CS)
58. • Rare tumor.
• Seen in young adults.
• Epiphysis(50%) or apophyseal (iliac
crest/greater trochanter of femur:15%).
• Morphology : sheets of polyhedral chondroblasts
with well defined borders , pink cytoplasm,
nuclear grooves, coffee bean appearance
(D/D:LCH )hyaline matrix with chicken-wire,
FINE calcification, osteoclast type giant cells.
59. • RADIOLOGY:
-Extremely well demarcated lesion almost
always in epiphysis or may extend into
metaphysis (with an open epiphyseal
plate).
- Surrounded by dense sclerotic border.
70. EWING SARCOMA/PNET.
• ES and PNET are “Round blue cell tumors of
bone & soft tissue”.
• Other Round Blue Cell Trs are :- Lymphoma,
rhabdomyosarcoma, neuroblastoma, oat cell
carcinoma, retinoblastoma, medulloblastoma.
• Have a neural phenotype.
• Both differ only in their degree of neural
differentiation.
• Tumors that demonstrate neural differentiation
by light microsopy, immunohistochemistry ,or
E/M are labelled PNETs.
• Those that are undifferentiated are diagnosed
as ES.
71. • ES & PNETs : comprise 6-10% of primary bone
tumors.
• 2nd most common malignant bone tumor in
children after OS.
• Age: 10-15 yr.
• Boys more frequently affected.
• More in whites.
• Site: arises in medullary cavity of Diaphyses or
Metaphysis of long bones esp. femur
• 85% have : t(11; 22)translocation.
• 5-10% have : t(21; 21) translocation.
• Less than have: t(7; 22) translocation.
• Those that affect the chest wall are called
“ASKIN TUMOUR”.
72. MORPHOLOGY
• GROSS: Arises in medullary cavity
Tan white tr tissue
Vast areas of hemorrhage & necrosis
• MICROSCOPICALLY:
- Composed of sheets of small, round cells.
- High N/C ratio.
- Round nuclei having salt & pepper chromatin /frequent
mitosis.
- Scant cytoplasm.
- Clear cytoplasm( glycogen): positive for PAS.
- Homer-Wright rosettes (tr cells are arranged in a circle
about a central fibrillary space indicative of neural
differentiation).
73. • Pattern: tumor is divided by fibrous septa
into irregular lobules of closely packed
round blue tumor cells.
• Tumor cells are arranged around
capillaries.
• Areas of necrosis & acute inflammation
present .
74. X-RAYS
• Lytic,destructive tumor with permeative margins
with patchy subperiosteal, successive reactive
bone formation producing “onion-skin”
radiographic appearance.
• Invade cortex & periosteum producing soft tissue
mass.
• SUN-BURST (hair on end appearance) when
new bone is laid down perpendicular to cortex.
• CODMAN TRIANGLE :formed b/w elevated
periostium & destroyed cortex.
89. GIANT CELL TUMOR
(OSTEOCLASTOMA)
• Uncertain histogenesis
• Age: 20-40 yr with no sex predilection
• Believed to have monocyte-macrophage
lineage
• Site : EPIPHYSES of long bones , majority
around knee joint
• Most are solitary
• Multiple or multicentric in distal extremities
90. MORPHOLOGY
• GROSS: Large red brown masses
Areas of cystic degeneration
• MICROSCOPICALLY:
1) Uniform oval mononuclear cells with
indistinct cell borders which appear to
grow in syncytium (neoplastic component)
2) Osteoclast type giant cells (100 or more
nuclei): is the non-neoplastic component,
scattered REGULARLY throughout the
tumor.
91. • D/D:
1) brown tumour of hyperparathyroidism,
2) giant cell reparative granuloma,
3) chondroblastoma &
4) pigmented villonodular synovitis
92. Biologic behaviour
• Aggressive lesions
• About 40-60% recur after curretage
• Approx. 4% result in distant metastases
• Grading of giant cell tumor : not
satisfactory
• Malignant transformation following
radiotherapy
93. X-RAYS
• Large , purely lytic , & eccentric lesion at
the end of expanded long bone
• Overlying cortex is destroyed producing
soft tissue mass delineated by thin shell of
reactive bone
• Characteristic “ SOAP BUBBLE”
appearance
103. ABC
• Benign bone tr.
• Formation of of multiloculated blood filled
cystic spaces.
• Rapidly growing, EXPANSILE LESION.
• Recurring, rapidly destructive lesion.
• Less than 20yrs.
• Not a true cyst but rather a collection of
blood filled cytic spaces NOT LINED BY
ENDOTHELIUM.
104. LOCATION:
• Metaphyseal region of long bone or
• Vertebra (dorsal elements).
• Eccentric
CYTIGENETICS:
• Upregulation of USP-6 fusion gene(chr17).
105. Radiology: X-ray:-
1. Lytic, expansile, destructive,
2. Eccentric bone lesion (BLOW –OUT)
appearance.
3. Well defined margins
4. Metaphyseal location.
CT/MRI: Peculiar fluid filled levels & internal
septa
106. L/M:
1. Lesion has vascular spaces ranging from small
cap sized to large sinusoids separated by
fibrous septae (spindle shaped fibroblasts,
scattered m/n giant cells, osteoblasts
associated with reactive immature woven bone
(fibre osteoid).
2. Cartilage type matrix (BLUE BONE):1/3 cases.
107. • ABC like areas can be seen :
1. GCT.
2. CHONDROBLASTOMA.
3. Fibrous dysplasia.
• SOLID VARIANT of ABC.
113. METASTATIC TUMORS
• Most common form of skeletal malignancy
• 75% in adults come from prostate, breast,
kidney & lung malignancies.
• In children, neuroblastoma, Wilms tumor &
rhabdomyosarcoma send their metastatic
secondary deposits to bone.
• Typically multifocal deposits but can be solitary
• Site: axial skeletal , femur ,& humerus
• Radiographically: Purely lytic
Purely blastic
Mixed lytic & blastic
114. • Lytic lesions in cases of Ca kidney, lung,
GIT, malignant melanoma
• Blastic lesions in prostate
adenocarcinoma
• Mixed lytic & blastic in majority of
metastases
118. 1) FIBROUS DYSPLASIA
• Benign tumor likened to development
arrest
• Three clinical presentations:
1. Monostotic
2. Polyostotic
3. Polyostotic with café au lait skin
pigmentations & endocrine abnormalities
119. 1) MONOSTOTIC FIBROUS
DYSPLASIA
• 70% of all cases
• Equally seen in boys & girls
• Early adolescence
• Asymptomatic or cause enlargement &
distortion of bone
120. 2) POLYOSTOTIC FIBROUS
DYSPLASIA
• 27% of cases
• Younger age
• Involvement of shoulder & pelvic girdles
resulting in crippling deformities like
shepherd crook deformity & spontaneous
fractures
121. 3) McCUNE-ALBRIGHT
SYNDROME
• Polyostotic firous dysplasia with café au
lait skin pigmentation and
endocrinopathies
• Sexual precocity, hyperthyroidism,
pituitary adenomas, & adrenal hyperplasia
122. MORPHOLOGY of FIBROUS
DYSPLASIA
• Well circumscribed lesions
• Tan white & gritty
• Composed of curvilinear trabeculae of
woven bone resembling chinese letters &
shapes.
• Surrounded by moderately cellular
fibroblastic proliferation
• Bone trabeculae lack osteoblastic
rimming
138. 1) ACHONDROPLASIA
• Major cause of dwarfism
• Defect in paracrine system resulting in
reduction in proliferation of chondrocytes
in growth plates
• Enlarged head with bulging forehead,
shortened proximal extremities depression
of root of nose
• Normal intelligence, reproductive status
139. 2) OSTEOGENESIS IMPERFECTA
(BRITTLE BONE DISEASE)
• Type I collagen defect disease
• Skeletal manifestations & changes in
structure of tissue rich in type I collagen
like joints, eyes, ears, skin, & teeth
• Morphologically : Too little bone
Osteoporosis with
cortical
thinning
140. LOCAL DEFECTS:
• Failure of development of bone
(congenital absence of phalanx, rib or
clavicle)
• Formation of extra bones( supernumerary
ribs or digits)
• Fusion of two adjacent digits (syndactyly)
• Development of long ,spider like digits
141. Four major subtypes
• OI TYPE I :Postnatal fractures
blue sclerae
• OI TYPE II: Perinatal lethal
• OI TYPE III :Progressive deforming
• OI TYPE IV: Postnatal fractures
Normal sclerae
142. 3) OSTEOPETROSIS
(MARBLE BONE DISEASE)
• Rare genetic disease characterized by
reduced osteoclast bone resorption
• Diffuse skeletal sclerosis
• Stone like quality of bones (too much
bone)
• Four types
• Bones lack medullary cavity & ends of
long bones are bulbous
• Neural foramina are small
145. OSTEOPOROSIS
(OSTEOPENIA)
• Reduced bone mass & increase
fractures
• May be localized or generalized
• LOCALIZED: Disuse osteoporosis of
limb
• GENERALIZED:
1) PRIMARY: Postmenopausal
Senile
Idiopathic
146. Cont.
2) SECONDARY:
a) ENDOCRINE: Hyperparathyroidism
Hypo-hyperthyroidism
Hypogonadism
Pituitary tumors
Diabetes, type I
Addison disease
147. b) DRUGS : Anticoagulants
Chemotherapy
Corticosteroids
Anticonvulsants
Alcohol
c) NEOPLASIA: Multiple Myeloma
Carcinomatosis
149. PATHOGENESIS
• Peak bone mass is achieved in early adulthood
after cessation of modelling
• Depends on 1):Hereditary factors like type of vit.
D receptor inherited, gene for collagen 1A1,
estrogen receptors, insulin –like growth factor
2): Physical activity
3): Muscle strength
4): Diet
5): Hormonal state
6): Sex: more in females
150. Cont.
• In 4th decade, amount of bone resorbed by
BMU exceeds that what is been formed
resulting in steady decrease in skeletal
mass.
• Average 0.7% bone loss per year
• Rapid bone loss in women following
menopause
• More in whites
151. MORPHOLOGY
• Entire skeletal is involved but certain
regions are more severely involved
• More in those bones that have increase
surface areas such as vertebral bodies
• Trabeculae are thinned , lose their
connections leading to microfractures &
collapse
152. • Clinical features:
Loss of height
Lumbar lordosis & kyphoscoliosis
Fractures
• Prevention: Exercise
Calcium & Vit. D intake
Pharmacologic agents
153. PAGET DISEASE
( OSTEITIS DEFORMANS)
• Collage of matrix madness
• Three phases:
1): initial osteolytic stage
2): mixed osteoclastic- osteoblastic stage
3): osteosclerotic stage
• Age: mid- adulthood
• More in whites
• Common in Europe, America, Australia
• Rare in native populations of Scandavia, China,
Japan, & Africa
154. PATHOGENESIS
• First described by Sir James Paget
• Thought to be inflammatory
• Followed by many hypotheses
• Finally again considered as infective
process
• Slow virus infection by paramyxovirus
• Hyperresponsive to vit. D & RANKL
• Familial predisposition
155. MORPHOLOGY
• Bone resorption with many osteoclasts
• Mixed stage resulting in MOSAIC pattern
of cement lines
• Quiscent osteosclerotic stage: After many
years, excessive bone formation results so
bone becomes compact producing
osteosclerosis
• Cotton-wool appearance on X-rays
156. HYPERPARATHYROIDISM
• Primary or secondary
• Entire skeleton is affected
• Increased osteoclastic activity
• Thin cortex
• Osteoclasts tunnel into & dissect centrally along length of
trabeculae (RAIL-ROAD) producing dissecting osteitis
• Predisposes to microfractures & hemorrhages with
multinucleated giant cells creating BROWN TUMOR
• Generalized osteitis fibrosa cystica ( von Recklinghausen
disease): Severe hyperparathyroidism. Combination of
increased bone activity, peritrabecular fibrosis, & cystic
brown tumors
157. RENAL OSTEODYSTROPHY
• Seen in ch. renal disease
• Involves two events: hyperphosphataemia &
hypocalcemia
• HYPERPHOSPHATAEMIA: In CRF, impaired
PO4 excretion leads to its retention. Cause
secondary hyperparathyroidism
• HYPOCALCEMIA: Occurs due to decrease
conversation of vit. D metabolite to its active
form
• METABOLIC ACIDOSIS:
