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
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
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.
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.
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.
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
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.
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
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
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.
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
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
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.
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
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
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
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
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