tumors of bone
DESCRIPTION
Tumors of bone. Tumors of bone. - Cause usually unknown (Primary, idiopathic) - Genetic factors may play a role (p53 and RB mutations) - Bone infarcts, trauma, osteomyelitis, Pagets disease, radiation and metal prosthesis predispose to tumors. Tumors of bone. - Clinical presentation - PowerPoint PPT PresentationTRANSCRIPT
Tumors of bone
Tumors of bone
- Cause usually unknown (Primary, idiopathic)
- Genetic factors may play a role (p53 and RB
mutations)
- Bone infarcts, trauma, osteomyelitis, Pagets
disease, radiation and metal prosthesis
predispose to tumors
Tumors of bone- Clinical
presentation
- Incidentally
detected
- Swelling and pain
- Pathological
fracture
Interpret bone tumors with full
knowledge of
- Clinical picture
- Radiology and imaging
- Pathological findings
Tumors of bone
- Diverse in their gross and morphologic features,
and clinical behavior.
- Benign tumors outnumber malignancies and are
commoner in young
- Classification is done on the basis of tissue of
origin
Bone tumors
Histologic type Benign Malignant
Hematopoietic (40%) Myeloma, lymphoma
Chondrogenic (22%) Osteochondroma, chondroma, chondroblastoma, chondromyxoid fibroma
Chondroscarcoma, dedifferentiated and mesenchymal chondrosarcoma
Osteogenic (19%) Osteoid osteoma, osteoblastoma Osteosarcoma
Unknown (10%) Giant cell tumorEwing sarcoma, giant cell tumor, adamantinoma
Histiocytic Fibrous histiocytoma Malignant fibrous histiocytoma
Fibrogenic Fibroma Desomplastic fibroma, fibrosarcoma
Notochordal Chordoma
Vascular HemangiomaHemangioendothelioma, hemangiopericytoma, angiosarcoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma (schwannoma)
Tumors and tumor-like conditions of bone- Diverse in their gross and morphologic features, and clinical behavior.- Benign tumors outnumber malignancies and are commoner in young- Specific tumor types have preferential age and sex distribution- Specific tumor types preferentially involve specific bones and specific regions within a bone
Metaphysis
10 20 30 40 50 60 70 yrs
Osteogenic Sarcoma
10 20 30 40 50 60 70 yrs
Chondrosarcoma
Bone tumors
All bone tumors should be studied along with x
rays
- Osteochondroma is the commonest benign tumor
- Osteosarcoma is the commonest non-hematopoietic
malignancy
- Specific tumors target specific bones and sites and age
groups
- Osteosarcomas are more common in adolescence and
arise from
metaphysis around the knee.
- Chondrosarcomas and giant cell tumors arise in epiphysis
- Ewings sarcoma arises in diaphysis
Histologic grade is the most important prognostic
factor of a bone sarcoma
Bone tumors
Histologic type Benign Malignant
Hematopoietic (40%) Myeloma, lymphoma
Chondrogenic (22%) Osteochondroma, chondroma, chondroblastoma, chondromyxoid fibroma
Chondroscarcoma, dedifferentiated and mesenchymal chondrosarcoma
Osteogenic (19%) Osteoid osteoma, osteoblastoma Osteosarcoma
Unknown (10%) Giant cell tumorEwing sarcoma, giant cell tumor, adamantinoma
Histiocytic Fibrous histiocytoma Malignant fibrous histiocytoma
Fibrogenic Fibroma Desomplastic fibroma, fibrosarcoma
Notochordal Chordoma
Vascular HemangiomaHemangioendothelioma, hemangiopericytoma, angiosarcoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma (schwannoma)
Osteoid osteoma- Benign bone tumor seen often in teens and young adults
- < 2 cm diameter and have predilection for cortex of long
bones
- Painful lesions and can mimic osteomyelitis (PGE2)
- Well circumscribed mass of woven bone rimmed by
osteoblasts
- This is called the nidus and is surrounded by reactive
bone
Osteosarcoma
- Malignant mesenchymal tumor in which cancerous cells
produce bone matrix
- Most common non-hematopoietic bone tumor
- 75% occur before the age of 20 years. Second peak in elderly
- Commoner in males (1.6:1)
- Arises from metaphysis of long bones. 60% arise around the
knee
- Patients with hereditary retinoblastomas have a 1000 fold risk
- Metastasizes through hematogenous route (90% to lungs)
- Advances in treatment (chemotherapy and limb salvage
therapy) has improved long term survival to 60-70%
10 20 30 40 50 60 70 yrs
Osteogenic Sarcoma
Osteosarcoma - subcategorization
Based on Subtypes
Location Intramedullary / intracortical / periosteal etc
Differentiation Well / poorly
HistologyOsteoblastic / chondroblastic / fibroblastic, telangiectatic / giant cell / small cell
OthersSolitary / multicentricPrimary / secondary
Osteosarcoma
Gross: Bulky, gritty, hemorrhage and necrosis,bone
destructive and spreading in different directions
Microscopy: - Bizarre tumors cells with pleomorphic nuclei
- Multinucleated giant cells
- Formation of “lacy osteoid”
- Formation of other types of matrix
- Conspicuous vascular invasion - metastasize to lungs, brain
X-ray:- Large destructive lytic and osteoblastic mass with infiltrating
margins
- Lifts periosteum (Codman triangle) and produces reactive
periosteal bone formation
Tumor grading
- Low grade- High grade
- Cell
pleomorphism
- Tumor
differentiation
- Necrosis
- Mitotic activity
Hematogenous spread is very common and 10-20% have pulmonary metastases at the time of
diagnosis
Bone tumors
Histologic type Benign Malignant
Hematopoietic (40%) Myeloma, lymphoma
Chondrogenic (22%) Osteochondroma, chondroma, chondroblastoma, chondromyxoid fibroma
Chondroscarcoma, dedifferentiated and mesenchymal chondrosarcoma
Osteogenic (19%) Osteoid osteoma, osteoblastoma Osteosarcoma
Unknown (10%) Giant cell tumorEwing sarcoma, giant cell tumor, adamantinoma
Histiocytic Fibrous histiocytoma Malignant fibrous histiocytoma
Fibrogenic Fibroma Desomplastic fibroma, fibrosarcoma
Notochordal Chordoma
Vascular HemangiomaHemangioendothelioma, hemangiopericytoma, angiosarcoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma (schwannoma)
Benign cartilage tumors:Osteochondroma: Bony spur / exostosis. Mushroom shaped bone projection
covered by cartilage cap
Chondroma: - Enchondroma ( intramedullary) or juxtacortical- Usually solitary and metaphyseal in short tubular bones and < 3 cms dia- Multiple tumors form part of Olliers disease (enchondromatosis)- Develop from rests of growth plate cartilage- Contain well circumscribed nodules of cartilage and cytologically benign chondrocytes- Endochondral ossification is seen at periphery- X-ray shows typical “O ring” sign - Malignant transformation more common in Ollier disease
Chondrosarcoma- Malignant tumors that form neoplastic cartilage
- Subtypes:a) Intramedullary and juxtacortical
b) Conventional (hyaline / myxoid), clear cell,
dedifferentiated, mesenchymal
c) Low grade to high grade
- Usually seen in patients above 40, more in men (2:1)
- More often in central parts of skeleton
- Large bulky tumors with glistening cut surface with
central necrosis
- Tumor spreads by pushing margins
- Low grade tumors almost similar to enchondromas
- High grade show marked pleomorphism and bizarre
morphology
- Spread preferentially to lungs and skeleton
Bone tumors
Histologic type Benign Malignant
Hematopoietic (40%) Myeloma, lymphoma
Chondrogenic (22%) Osteochondroma, chondroma, chondroblastoma, chondromyxoid fibroma
Chondroscarcoma, dedifferentiated and mesenchymal chondrosarcoma
Osteogenic (19%) Osteoid osteoma, osteoblastoma Osteosarcoma
Unknown (10%) Giant cell tumorEwing sarcoma, giant cell tumor, adamantinoma
Histiocytic Fibrous histiocytoma Malignant fibrous histiocytoma
Fibrogenic Fibroma Desomplastic fibroma, fibrosarcoma
Notochordal Chordoma
Vascular HemangiomaHemangioendothelioma, hemangiopericytoma, angiosarcoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma (schwannoma)
Ewing Sarcoma and PNET (primitive neuro-
ectodermal tumor)
- Primary malignant small round cell tumors of bone and soft
tissue
- Share similar neural phenotype and chromosomal
translocation
- Second commonest malignant non-hematopoietic bone
tumor in children
- Children 10-15 years old, boys more affected. Diaphysis of
long bones
- 85% show t(11;22)
- Tumors arise from medullary cavity, invade cortex, spread
to soft tissue
- Comprised of small round cells slightly larger than
lymphocytes
- Cells are rich in glycogen.
- May show Homer-Wright rosettes. Necrosis is seen
- Xray shows typical layers of reactive bone (“Onion-skin”
pattern
- Combination chemotherapy has improved 5 year survival to
75%
Gross:
- Arise in medullary cavity
(diaphysis)
- Invade cortex and
periosteum
- Tan white colour with
hemorrhage and necrosis
Microscopy:
- Sheets of uniform
small cells
- Rosettes (Homer-
Wright)
- Few mitotic figures
Giant Cell Tumor:
(Osteoclastoma)
- Contains numerous osteoclast type of giant cells (monocyte
macrophage)
- Benign but locally aggressive
- Patients between 20 to 40 years
- Involve both epiphysis and metaphysis and are common in
long bones
- Xray shows typical soap bubble appearance and thin
reactive shell
- Tumor comprised of oval mononuclear cells growing in
sheets mixed with osteoclast type of giant cells with more
than 100 nuclei
- Necrosis and hemorrhage common
- Biologically unpredictable tumors
Involve both epiphysis and
metaphysis
Large purely lytic - “Soap
bubble” tumors
Gross:
- Large, red-brown
- Hemorrhage and
cystic degeneration
- Uniform mononuclear
cells in sheets with
increased mitoses
- Numerous osteoclast
type of giant cells with >
100 nuclei