2. Malignant tumors of bone
Brig.Naveed Hussain Syed
HOD Surgery Department
CMH Bahawalpur
3. Bone tumors
◾Condition of skeletal system that are
neoplastic or could be mistaken for
neoplastic condition on basis of radiological
or pathological evidence.
◾Progenitor cells- bone tumors can arise from
osteoblasts, chondroblasts, periosteal cells,
haematopoietic cells, lipocytes, schwann cells,
fibroblasts, osteoclasts, endothelial cells,
notochordal cells and epithelial cells
5. Age Tumour
<1 Neuroblastoma
1-10 Ewing sarcoma-tubular bones
10-30 Osteosarcoma, Ewing sarcoma-flat bones
30-40 Fibrosarcoma,malignant fibrous
histiocytoma,lymphoma,Malignant GCT
>40 Chondrosarcoma,chordoma,multiple myeloma
AGE OF ONSET
6. SITE OF ORIGIN
EPIPHYSIS MALIGNANT GCT
METAPHYSIS OSTEOSARCOMA
CHONDROSARCOMA
FIBROSARCOMA
DIAPHYSIS EWING’S SARCOMA
MULTIPLA MYELOMA
ADAMANTINOMA MFH
7. CHARACTERISTICS OF
MALIGNANT
TUMOR
◾ Most commonly presents as painful bony
mass
◾ Extends to soft tissue through broken cortex
◾ Rarely multifocal
◾ Radiographically-
Poorly defined borders
Wide zone of transition
Moth eaten or permeative type of bone
destruction
Interrupted type of periosteal reaction
8. APPROACH TO DIAGNOSIS OF MALIGNANT
BONE TUMOR
◾ Requires multi-phased work up
◾ T
eam work is necessary.
◾ Goal- whether lesion is benign
or malignant
◾ Steps-
History
Local examination
Laboratory test
Radiological test
Histopathological examination
9. HISTORY
◾ Age- some tumors are very age specific. Eg- Ewing
sarcoma 10-20yrs; osteosarcoma 15-25yrs and >
45yrs (bimodal); chondrosarcoma >45yrs; multiple
myeloma >50yrs.
◾ Non specific
◾ Dull aching painful lump
◾ Pathological fracture
◾ Sometimes as incidental finding
◾ H/o exposure to radiation and chemical carcinogens
◾ History of any malignancy anywhere in body or
treatment
history
for any malignancy at present or past.
10. ON EXAMINATION
◾Swelling- tenderness, location, shape,
consistency, fixity to skin and adjacent
structure, mobility, skin over swelling , dilated
or engorged veins.
◾Joint range of movement limitation
◾Sign of inflammation may be present
◾Any other skin lesion anywhere else in the
body
◾Regional lymph node
◾Systemic examination to diagnose primary
tumor in case of metastasis.
11. LABORATORY TEST
◾ Hemogram
◾ Erythrocyte
sedimentation rate
◾ C- reactive protein
◾ Serum calcium
◾ Serum phosphorus
◾ Alkaline phosphatase
◾ Lactate
dehydrogenase
◾ Parathormone
◾ Urinary Bence Jones
protein
◾ Urinary 24hrs
calcium
◾ Electrophoresis
◾ Bone marrow
examination
12. X-RAY
◾Gold standard for diagnosing any
bone tumor.
◾Lesion is assessed for-
Location,
Size,
Cortical integrity,
Margination,
Periosteal reaction and
Soft tissue involvement
Pattern of bone destruction
17. HISTOPATHOLOGICAL EXAMINATION
◾ Biopsy is most important to determine histological diagnosis and hence plan the treatment.
◾ Golden rule-
Biopsy should be done when all radiological investigation have been completed.
All suspected malignant tumor and aggressive benign tumor should be biopsied
prior to
treatment.
Biopsy should be done by the surgeon who is doing the definitive management.
Exception- where imaging guidance biopsy is needed, there it is done by interventional
radiologist.
Joint should never be violated during biopsy.
Biopsy should be done through recommended site.
Best material for biopsy from the periphery of the tumor. Lytic area provide most
representative tissue.
Biopsy site should be small as feasible with longitudinal incision avoiding
major
neurovascular structure.
18. Ewing sarcoma
Definition
– Highly malignant bone tumor arising from neuroectodermal cells
– Some sources suggest that Ewing sarcoma originates from mesenchymal stem
cells.
• Etiology: associated with various chromosomal translocations of
the EWSR1 gene (chromosome 22)
• Epidemiology
– Incidence: peak at 10–20 years
– Sex: ♂ > ♀
– Ethnicity: primarily affects white individuals
• Localization
– Primary tumor: often diaphyses of long bones (particularly femur, tibia, fibula,
and humerus) and bones of the pelvis
– Metastasis: lungs, skeletal system, bone marrow
• Clinical features
• Frequently first manifests with localized pain (progressive, worsens at
night), hyperthermia
• swelling after trauma to the bone (tissue mass that is tender to palpation and
accompanied by erythema)
• B symptoms are common.
19. Diagnostics
• Conventional X-ray
– Lytic bone lesions
– Onion skin appearance of the periosteum
• Biopsy
– Anaplastic small-blue-round-cell malignancy
• Tumor cells resemble lymphocytes .
• Differential diagnoses include lymphoma and chronic osteomyelitis.
– Chromosomal translocation t(11;22)(q24;q12) which leads to expression of fusion
protein EWS-FLI1
– Cells contain glycogen accumulations and are usually CD99-positive.
• Laboratory findings: ↑ ESR, ↑ LDH, leukocytosis
• “Ew, did you feed on 22 onions?”: Ewing sarcoma, femur region, chromosome
22, onion skin appearance.
Treatment
• Surgery (definitive resection) plus neoadjuvant and adjuvant polychemotherapy
• Additionally: radiation therapy
Prognosis
• Extremely aggressive, early metastases
• Usually responsive to chemotherapy
• Five-year survival rate of ∼ 80% for localized disease
20. (A)Ewing’s sarcoma of the
femur shows an
illmarginated, lytic-sclerotic
lesion with cortical
irregularity, laminar
periosteal reaction, with a
large soft tissue mass in the
metadiaphyseal region
(A)In another case involving
the femoral diaphysis
“saucerization” is well seen,
with peripheral Codman’s
triangles and a
large soft tissue mass
21. Osteosarcoma
• Definition: malignant, osteoid, and bone-forming tumor arising from mesenchymal stem
cells (osteoblasts) located in the periosteum
• Etiology
– Primary osteosarcoma: unknown
– Secondary osteosarcoma: Paget disease of bone, radiation injury, bone infarction
– Increased incidence in individuals with retinoblastoma and Li-Fraumeni syndrome
• Epidemiology
– Incidence: bimodal distribution
• Primary osteosarcoma: in puberty/adolescence (peak incidence age 10-30 years)
• Secondary osteosarcoma: advanced age
– Sex: ♂ > ♀
– Most common primary bone malignancy
• Localization
– Primary tumor: metaphyses of long bones (particularly distal femur and proximal tibia)
– Metastases: lungs , skeletal system, regional lymph nodes
• Clinical features
• Frequently first manifests with pain (progressive, worsens at night and with activity)
• Progressive swelling (tissue mass that is tender to palpation and accompanied by erythema)
• Pathologic fractures
• Limping, decreased range of motion
• Possible B symptoms
22. Diagnostics
• Imaging
– Conventional x-ray
• Sunburst appearance of lytic bone lesions and/or Codman triangles
• Signs of osteolysis adjacent to osteosclerosis (moth-eaten appearance)
– MRI: assesses the involvement of soft tissue, evaluation in cases of unclear radiographic findings
• Biopsy
– Pleomorphic, malignant osteoblasts that produce osteoid
– Osteosarcomas always feature woven bone matrix (compared to chondrosarcomas
and fibrosarcomas)
• Laboratory
– ↑ Alkaline phosphatase ↑ LDH ↑ ESR
Treatment
• Surgery (definitive resection) with neoadjuvant and adjuvant polychemotherapy
• Histological examination of the resected bone to evaluate the effect of neoadjuvant
chemotherapy (major prognostic factor)
• Osteosarcomas are usually resistant to radiation therapy.
Prognosis
• Aggressive course
• Primary osteosarcoma: five-year survival rate of ∼ 70% (usually responsive to treatment)
• Secondary osteosarcoma: poor prognosis (less responsive to treatment)
23.
24. Chondrosarcoma
• Definition: a malignant tumor arising from mesenchymal cells that
produce cartilage
• Etiology
– Primary chondrosarcoma: unknown
– Secondary chondrosarcoma: e.g., osteochondroma, Paget disease of
bone, radiation
• Epidemiology
– Age: usually > 50 years
– Sex: ♂ > ♀
• Localization: most common in the medullary cavity of
the pelvis, ribs, proximal femur, and proximal humerus
• Clinical features
• Deep, dull pain (worsens at night, insidious progression over
months to years)
• Local swelling
• Pathological fractures
• Neurovascular disturbances and/or limited range of motion
25. Diagnostics
• Conventional X-ray or CT
– Osteolysis with a moth-eaten appearance
– Intralesional calcifications (rings and arcs calcification, popcorn calcification)
– Endosteal scalloping and cortical breach with infiltration of soft tissue
• MRI: rim-like contrast enhancement
• Biopsy
– Malignant chondrocytes
– Lobulated appearance (hyaline cartilage nodules with peripheral calcification )
Treatment
• Surgery (definitive resection)
• Chemotherapy and radiation therapy. possibly as adjuvant therapy or as palliative
treatment
Prognosis
• Five-year survival rate of 50–85% (depending on the histological grading)
• Late recurrences are possible.
• Regular follow-ups for 10 years are required.
26.
27. RADIATION THERAPY
◾ Radiation causes cell death by inducing formation of
intracellular free radicals that
subsequently causes DNA damage.
◾ Most malignant bone tumors are radio-resistant, except
small blue cell tumors including multiple myeloma,
Ewing’s sarcoma and lymphoma.
◾ Therapy reduces the incidence of local recurrence of
malignant tumors treated with
marginal resection.
◾ Pre operative use decreases the tumor volume, facilitating the
tumor resection.
28.
29. CHEMOTHERAPY
◾ With use of modern chemotherapy protocol, 5-year survival
rate of malignant bone tumors have drastically increased.
◾ Adjuvant chemotherapy- chemotherapy administered
postoperatively to treat presumed micro metastasis.
◾ Neoadjuvant chemotherapy- chemotherapy administered
before surgical resection to regress size of the tumor , making
limb salvage operation easier, tumor resectable and also
decreases spread of tumor.
30. SURGERY
◾Decision about what type of surgery to be done
depends upon-
Diagnosis
Site of tumor
Extent of tumor
Response of tumor to neoadjuvant chemotherapy
Patient's age, background and financial status
◾Goal of limb salvage- complete eradication
of surgery, while maintaining acceptable
function, durability, and cosmesis of limb
31. SURGICAL RESECTION AND
RECONSTRUCTION
Margin Surgical procedure Result
Intralesional Piecemeal debulking or
curettage
Leave microscopic
disease
Marginal Shell out en-bloc
through
pseudocapsule
or reactive zone
May leave either
satellite or skip lesion
Wide Intracompartmental en
bloc with cuff of normal
tissue
May occasionally
leave skip lesion
Radical Extracompartmental
en bloc entire
compartment
32.
33.
34. DIFFERENT MODALITIES OF LIMB
SALVAGE
◾ Allograft
◾ Endoprosthesis
replacement
◾ Autograft
◾ Bone lengthening
◾ Rotationplasty
◾ Arthrodesis
35. MANAGEMENT OF METASTATIC
BONE TUMOR
◾ Pain management is the prime principle in
management of metastatic bone tumor.
◾ According to Mirel’s scoring system we treat
pathological fracture prophylactically to preserve
function of limb.
◾ Multidisciplinary approach to control bone pain-
Drugs therapy- analgesics and bisphosphonates.
Physical therapy- splint
Radiotherapy and use of radiopharmaceuticals.
Anaesthetia methods- blocks
Neurosurgical methods- hypophysectomy
Behavioural approaches- relaxation techniques
