BONE TUMORS

Dr Sushma Thapa
Dept of Pathology
 Classification
 Gross & microscopic, Clinical features, X-
ray findings of:
A. OSTEOSARCOMA
B. GIANT CELL TUMOR
C. EWING’S SARCOMA
D. CHONDROSARCOMA
 Classification of bone tumors
 Bone Forming Tumors
 Benign – osteoma , osteoid osteoma , osteoblastoma
 Malignant – Osteosarcoma
 Cartilage Forming Tumor
 Benign – Chondroma , osteochondroma
 Malignant – Chondrosarcoma
 Miscellaneous Tumors
 Osteoclastoma ( Giant cell tumor )
 Mostly benign
 Ewing Tumor
 Always malignant
 OSTEOSARCOMA
 bone-producing malignant mesenchymal primary
tumor of bone
 Incidence
 Gender - M>F
 Age –
 Any age may be affected – two peaks
 70% seen below 20 years
 Second peak in elderly – mostly secondary due to
other diseases
 Site
 Any bone can be involved,
 Most arise in the
metaphyseal region of the
long bones of the extremities,
 60% occurring about the
knee,
 15% around the hip,
 10% at the shoulder,
 8% in the jaw.
 Genetics
 Majority cases has mutation of Rb gene
 Patients with Retinoblastoma – has much
higher risk
 Other genes include – p53 , cyclins etc
 GROSSLY
 Usually involves the metaphysis of long bones
 Most commonly around the knee ( distal femur
and proximal tibia )
 Large firm white tan mass with hemorrhage and
necrosis
 Destruction of surrounding bone
 Lifts the overlying periosteum
 Spreads through medullary cavity but usually do
NOT penetrate epiphyseal cartilage i.e. joint is
usually not affected
 MICROSCOPICALLY
 Malignant osteoblasts
 Newly formed lacy osteoid (eosinophilic / pink)
 Mitosis , Necrosis
 Vascular invasion and distant metastasis may
be seen
 CLINICAL FEATURES
 Localized pain and swelling
 Occasionally , fracture may occur

 X-RAY FINDINGS
 Characteristic X-ray findings
 Tumor with Bone destruction
 Periosteal elevation
 Codman’s triangle – between elevated
periosteum and cortex
 Sunburst pattern – divergent spiculated bone
formation
PERIOSTEAL ELEVATION

CODMAN TRIANGLE

SUNBURST PATTERN
 TREATMENT
 Surgery ( limb salvage )
 and Chemotherapy
 PROGNOSIS
 With modern treatment –
 long survival ~~ 60%
 Hematogenous metastasis common
 – e.g. lung
 – if present – poorer prognosis
 SECONDARY OSTEOSARCOMA
 in elderly
 Assd with other primary disease –
 Most commonly Paget disease
 Radiation
 Highly aggressive
 Poor response to treatment
 Poor prognosis
 OSTEOCLASTOMA
 Also known as
 GIANT CELL TUMOR OF BONE
 Uncommon neoplasm
 Contains multinucleated giant cells (osteoclast like)
admixed with stromal cells
 Due to these giant cells – the name
 Locally aggressive tumor which rarely may become
malignant and metastasize
 Age – young adult – 20 – 40 yrs
(NOTE : in OSa – age – commonly less than 20
yrs )

 Site –
 Epiphyses of long bone
 Most commonly around knee joint (distal femur
and proximal tibia )
 GROSS
 Red-brown cut surface
 Frequent cystic degeneration
 MICRO
 Two types of cellular component
 Osteoclast like multinucleated giant cells scattered
in between spindle shaped stromal cells
 Spindle shaped stromal cells are the neoplastic
component
 Multinucleated giant cells are formed by fusion of
the stromal cells
 Multinucleated giant cells often contain more than
100 nuclei
 Necrosis , Hemorrhage , Mitosis – may be seen
 Clinically
 Enlarging swelling at the end of long bones –
often around knee joints

 X-ray
 Large lytic tumor
 “Soap bubble appearance”
 Periosteal elevation and reactive bone formation
– NOT seen
 TREATMENT
 Most of the tumors are localized and treated
by curettage
 50 % recur locally
 Rarely may metastasize
 EWING SARCOMA
 Ewing Sarcoma & PNET ( Primitive
Neuroectodermal Tumor ) come under same
group
 Same cell of origin
 Similar chromosomal translocation
 PNET has more neural differentiation
 ES is more undifferentiated tumor
 ES also come under the group of “Blue
Round cell tumors”
 It includes
 Lymphoma
 ES / PNET
 Retinoblastoma
 Rhabdomyoblastoma - embryonal type
 Neuroblastoma
 Second most common malignant bone tumor in
children
 ( In children and adolescence – commonest
malignant tumor - osteosarcoma)
 Age –
 80% are younger than age 20
 Rare after 30 yrs
 Gender
 Male slightly > girls
 Race
 Caucasians >>> blacks
 Site
 Tumor arises in medullary cavity and infiltrate
through cortex , periosteum and soft tissue
 Diaphysis
 Any bones may be affected
 Long bones more common , e.g femur
 Flat bones e.g., pelvic bones also may be
affected
 Genetics
 Chromosomal translocation involving EWS
gene on chromosome 22
 Two translocations seen
 t(11;22) - more common
 t(21;22) - less common
 GROSS
 Grey white mass involving medullary cavity
infiltrating through cortex and into soft tissue
 Necrosis and hemorrhage also seen
 MICRO
 Sheets of uniform small round cells with minimal
intervening stroma
 Cells are slightly larger than lymphocyte size
 Cells have scant cytoplasm with glycogen
 Tumor cells may show Homer-Wright rosettes
 Tumor cells circled about a central fibrillary space
 Indicates neural differentiation
 Necrosis often seen
 Homer-Wright rosettes
•Tumor cells circled about a central fibrillary
space
• Indicates neural differentiation
 Clinically
 Localized pain and swelling
 Usually in the shaft of long bones

 As often tender and swollen with fever ,

increased WBC , increased ESR , anemia
 Mimic infection / osteomyelitis
 X-ray
 Mass in diaphysis – infiltrating into soft tissue
 Periosteal elevation
 Reactive bone formation
 Onion skin Appearance - due to successive
layers of periosteal development
 Hair-on-end Appearance - when new bone is
laid down perpendicular to cortex
 Treatment
 By combination of chemotherapy and surgery
with or without radiation
 Prognosis
 75% - 5 yr survival
 50% - Long term remission
REMEMBER

EWING SARCOMA

OSTEOSARCOMA

GCT
 CHONDROSARCOMA
 Malignant tumor arising from chondroblasts
 production of neoplastic cartilage
 Classified acc. to site
intramedullary and
Juxtacortical
 Acc. to histology
conventional (hyaline and/or myxoid),
clear cell,
dedifferentiated, and
mesenchymal variants
 Age:
- > 40 yrs
- Clear cell, mesenchymal variants – younger
 Gender:
- M>F
 Site:
 central portions of the skeleton
pelvis,
shoulder, and
Ribs
 clear cell variant
epiphyses of long tubular bones
•Gross:
 large bulky tumors
 nodules of gray-white,
translucent glistening
tissue
 myxoid variants viscous
and gelatinous and the
matrix oozes from the cut
surface
 Spotty calcification,
necrosis
 Cortex thickened, eroded
MICROSCOPY:
 atypical chondroblasts
and chondrocytes
 multiple nuclei in a
lacuna
 histologically - grade I ,
II , III
 Clinically:
 painful,
 progressively enlarging masses
 X-Ray:
 prominent endosteal scalloping
 calcified matrix foci of flocculent density
 high grade more radiolucent
 Treatment
- wide surgical excision