Bone Tumors Premanagement

1. Bone Tumors Pre Management
(Malignant Conditions)
Dr Sasikumar Sambasivam

2. Introduction
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0.001% of all cancers
Benign and malignant tumors
Benign – latent, active and aggressive
MC benign tumor--- Osteochondroma; Osteoid
Osteoma
MC Skeletal malignancy– Mets
MC Bone tumor in Pediatric age group & adultsOsteosarcoma
MC in < 10 y--- Ewing’s sarcoma
MC Primary bone tumor – Multiple Myeloma

3. Benign
Classification and TypesMalignant
of Bone
Myeloma
Reticulum cell sarcoma
Tumor
Chondrogenic (20.9%)
Osteochondroma
Primary chondrosarcoma
Histologic Type
Hematopoietic (41.4%)
Chondromyxoid fibroma
Secondary chondrosarcoma
Dedifferentiated
chondrosarcoma
Mesenchymal chondrosarcoma
Osteogenic (19.3%)
Osteoid osteoma
Benign osteoblastoma
Osteosarcoma
Parosteal osteogenic sarcoma
Unknown origin (9.8%)
Giant cell tumor
Ewing sarcoma
Fibrogenic (3.8%)
Chondroma
Chondroblastoma
Malignant giant cell tumor
Adamantinoma
(Fibrous) histiocytoma
(Fibrous) histiocytoma
Fibroma
Desmoplastic fibroma
Fibrosarcoma
Notochordal (3.1%)
Chordoma
Vascular (1.6%)
Hemangioma
Lipogenic (<0.5%)
Lipoma
Neurogenic (<0.5%)
Neurilemoma
Hem.endothelioma&pericytoma

4. LOCATION
In the transverse plane:
a) Central – Enchondroma
b) Eccentric -GCT, osteosarcoma,
chondromyxoid fibroma
c) Cortical - Non-ossifying
fibroma,osteoid osteoma
d) Parosteal - Parosteal
osteosarcoma, osteochondroma

5. In the longitudianl plane
Epiphysis
Metaphysis
Diaphysis
Chondroblastoma
Osteoclastoma
Clear Cell Chondrosarcoma
Osteosarcoma
Chondrosarcoma
Enchondroma
Simple bone cyst
ABC
(Brodies abscess)
Chondromyxoid fibroma
Ewing sarcoma
Multiple Myeloma
Osteoid Osteoma
Lymphoma
Adamantinoma
Fibrous Dysplasia
Histiocytosis
Neoplasm
Age ( y)
Osteoid Osteoma& Ost Blastoma
10– 30
Osteosarcoma
10-25
Parosteal OS
30-60
Chondroma
10-40
Osteochondroma
10-30
Chondroblastoma
10-25
Chondrosarcoma
30-60
GCT
20-40
Ewing Sarcoma
05-20

6. Etiology
• Unknown
• Conditions Predisposing:
– Li Fraumenni
– RB
– Pagets disease
– Bone Infarct
– Radiation
– Osteomyelitis

7. Natual History
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Three mechanisms of growth and extension
– (1) compression of normal tissue,
– (2) resorption of bone by reactive osteoclasts, and
– (3) direct destruction of normal tissue
• Benign tumors by (1) and (2) and direct tissue destruction is
characteristic of malignant bone tumors.
• Sarcomas respect anatomic borders; remain in one compartment.
• Local anatomy influences tumor growth by setting the natural barriers
to extension.
• In general, bone sarcomas take the path of least resistance.
• Most benign bone tumors- unicompartmental;.
• Malignant bone tumors -bicompartmental; they destroy the overlying
cortex and go directly into the adjacent soft tissue.
– The determination of anatomic compartment involvement has become
more important with the view of of limb-preservation surgery.

8. Spread
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Exclusively Haematogenous
MC--- Lung, Bone
Early Lymphatic Spread -rare
SKIP METS:
– As a tumor nodule located within the same bone as
the main tumor but not in continuity with it
– More common in High Grade Sarcomas
– Transarticular skips are believed to occur via the
periarticular venous anastomosis.
– The clinical incidence of skip metastases is less than
1%
– Prognosticator of poor survival

9. Clinical Presentation
• Generalised body aches
• Localised Swelling; May not be obvious especially if the
tumour is deep
• Wasting of Muscles---Disproportionate to
duration of disease
• Multiple Myeloma
- Symtoms ignored for quite sometime
- Can be mistaken for many other conditions with generalised
body aches like Osteoporosis/Osteomalacia etc.
REST PAIN

10. Diagnostic Evaluation
• Basic Invetigations
• Plane Radiology of the involved part
– l/f Marginless border and Periosteal reaction
• CT for accurate determination of intra and extra osseous
extension
– Tranverse relationship to the tumor
– Should include entire bone and the adj joint
• MRI– provides most accurate contrast discrimination of
bone sarcomas
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Single best study & IOC for Intraosseous Extension
Detecting a small lesion
Radiography is negative but bone scan is positive
Skip mets and extent of infiltrative tumor

11. • Bone Scans:
– Determine Metastatic disease
– Intraosseous extension of
tumor
– Doesn’t detect Skip mets
• Angiography:
– To determine Vascularity
Specially for tumors of
Proximal Tibia where vascular
anomalies are common
• Isotope Scanning
– Thallium Scan
– PET mostly for Mets

12. Biopsy Technique and Timing
• The biopsy of a suspected bone tumor must
be performed with great care and skill.
• The consequences of a poorly executed biopsy
are often the deciding factor in the choice
between a limb-salvage procedure and
amputation.
• It is recommended that the biopsy be
performed by the surgeon who will make the
ultimate decision about the operative
procedure.

13. STAGING SYSTEMS
Musculo Skeletal Tumor
Society Classification
Stage
IA
Grade
Low (G1)
Site
Intracompart
mental (T1)
IB
Low (G1)
Extracompart
mental (T2)
IIA
High (G2)
Intracompart
mental (T1)
IIB
High (G2)
Extracompart
mental (T2)
III
Any G
Any (T)
Regional or
distant
metastasis (M1)
Enneking et al
Stage IA (G1, T1, M0): Low-grade
intracompartmental lesion, without
metastasis
Stage IB (G1, T2, M0): Low-grade
extracompartmental lesion, without
metastasis
Stage IIA (G2, T1, M0): High-grade
intracompartmental lesion, without
metastasis
Stage IIB (G2, T2, M0): High-grade
extracompartmental lesion, without
metastasis
Stage IIIA (G1 or G2, T1, M1):
Intracompartmental lesion, any grade, with
metastasis
Stage IIIB (G1 or G2, T2, M1):
Extracompartmental lesion, any grade, with
metastasis

14. AJCC
Ewing’s sarcoma ----Grade 4

15. Benign Vs Malignant
• Age of Skeleton –
- Mature or Immature
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Location
Transitional zone
Reactive zone
Matrix
Cortex
Periosteal reaction
Soft tissue swelling
Courtesy : slideshare

16. Radiographic differences between
Benign& Malignant IGNANTR
Tumours
BENIGN
MALIGNANT

17. Periosteal Reaction
SOLID
Benign
Spiculated
V Aggressive
Lamellated
Aggressive
Codman's

18. Periosteal Reaction
Sun Burst appearance
Codman’s Triangle

19. Mankin – Biological behavior
Criteria
Score
0
1
Size
Small
Big
Score
0-1 Benign
Margination
Present
Absent
2
Aggressive
Cortex
Intact
Destroyed
3-4
Malignant
Soft Tissue
Mass
Absent
Present

20. Osteosarcoma
Symptoms: Progressive pain/night pain or painless mass.
Mostly metaphyseal, 10% diaphyseal, 1% epiphyseal
Site– Lower end of femur and upper end of tibia> proximal
humerus
MC secondary malignancy in Heriditary RB
Characteristic Production of tumor osteoid / immature bone
Bimodal; Childhod and adolescence ; peak between 10 and 19 y
Pathological Classification :
 Osteoblastic ,chondroblastic ,telengiectatic, giant cell type and small cell
type
 Variants:
 OS of the jaw bone MC variant ---- low met potential
 Periosteal OS – MC variants of Classical OS arising in extremities
 Parosteal OS
 Paget’s OS
 Secondary Osteosarcoma

21. • Incidence of pathological
fracture <1%
• Radiologically,
– Increased intramedullary
radiodensity(calcified
cartilage),
– an area of
Radiolucency(Non Ossified
tumor),
– Permeative destrn with
poorly defined borders,
– Cortical destruction,
periosteal elevation,
– extraosseous extn,
Classically,
Codmann triangle---subperiosteal new
bone formn
Sunray appearance---due to growth of
tumor into the overlying soft tissue
with subsequent ossification

22. Parosteal
Osteosarcoma
• Low grade malignancy
• Rare , elderly , Juxtacortical,
• Better Prognosis than
Classical OS.
• MC --lobulated mass on the
posterior aspect of DISTAL
FEMUR
Periosteal
Osteosarcoma
• Intemediate grade
• Arises from surface of
bone- Juxtacortical
• MC site—TIBIAL SHAFT.

23. Intramedullary
Osteosarcoma
• Rare
• Low grade
Small cell
Osteosarcoma
• Rare
• High grade
• Resemble Ewing sarcoma
or Lymphoma

24. Secondary Osteosarcoma
• Older patients
• These occur at the site of another disease process
• E.g.
– Paget’s disease– Paget’s Sarcoma—1%; avg age 64 yrs, MC
site– pelvis>femur>humerus
– Previous RT,
– Fibrous dysplasia,
– Bone infarcts,
– Osteochondromas,
– Chronic osteomyelitis,
– Dedifferentiated chondrosarcomas,
– melorrhostosis and
– osteogenesis imperfecta.

25. Chondrosarcoma
• 9% of primary malignancies of bone
• Age: broad, primary
chondrosarcoma peak around 30 –
60yrs, secondary chondrosarcoma
25 – 45 yrs.
• Any location but common around
pelvis(31%), proximal femur(21%),
proximal humerus(13%); 76% are
Central.
• MC type in hand, sternum and
Scapula.
• Classical tissue: Cartilage without
evidence of direct osteoid formn.
• Key Sign: Endosteal Scalloping
Radiology:
Lesion arising in medullary
cavity with irregular matrix
calcification.
Pattern is described as
“punctate,” “popcorn,” or
“comma-shaped

26. Clinically: Increasing pain and palpable mass. Pain in absence of
pathological fracture is helpful to differentiate between enchondroma
and low grade chondrosarcoma
Secondary Chondrosarcoma
• Olliers disease (multiple enchondromatosis)
• Maffuci syndrome (multiple enchondromatosis + soft tissue
haemangioma)
• Multiple hereditary exostoses
• Solitary osteochondroma
• Synovial chondromatosis
• Chondromyxoid fibroma
• Periosteal Chondroma
• Chondroblastoma
• Previous radiation treatment
• Firbours dysplasia

27. Giant Cell Tumour
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Rarely Malignant
Locally aggressive , recurrent tumor, low
metastatic potential
Has Multinucleated Giant cells
Mononuclear cells are the Proliferating
component of the tumor not MNGs
8.6% - 22% of known GCT become
malignant after local recurrence; increases
risk of transformation
MC in 20-40 yrs; F>M
Epiphyseal;Usually solitary;long bones;
about 75% around knee jt.
Pain , mass and decreased mobility of adj.
joint
Radilogically, Eccentric lytic lesions;
Classical soap bubble appearance; No
Sclerosis or calcification

28. Ewing sarcoma
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Second MC primary bone tumor in paediatric groups
MF chest wall tumor of children
9% of primary malignancy of bone
Age: most occur in 5 – 25 yrs
Males > Females
Diaphysis; MC in Femur>pelvis,tibia,fibula,bones of feet
MC chromosomal abnormality t(11;22)
Clinically: Pain(insidious onset, mild and intermittent initially),
fever, erythema, swelling
• Pathology: Small round blue cell tumors, grouped with PNETs;
Homer Wright Pseudorosettes
• METS to lungs and other bones

29. Radiologically
• Destructive lesion in the
diaphyses of longbone
with “onion skin”-Lamellated periosteal
reaction.
• No new bone formation
• Bone Scan--- both to
define extent and and
bone mets
• Others: PAS positve and
Diastase sensitive

30. Small Round Cell tumors of Bone
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Ewing’s sarcoma
Primary Lymphoma of bone
Metastatic Neuroblastoma
Embryonal
Rhabdomyosarcoma
• Small cell Osteosarcoma
• Mesenchymal cell
Chondrosarcoma
• Metastatic small cell
carcinoma

31. Chordoma
• Locally malignant neoplasm from notochordal remnants in the
midline of the neural axis
• AGE: > 40 y
• Site: Ends of the spines > Sacrococcygeal region and BOS>
Spheno Occipital region
• HP: Physaliferous cells are pathognomonic
• Highly fatal – high rate of local recurrence and local
complications
• Clinically:
– Spheno-occipital: headaches, symptoms of cranial nerve compression,
retropharyngeal abscess.
– Sacrococcygeal: lower back pain, sciatic pain, bowel and bladder
problems. Palpable mass on PR.
– Spinal: presents with nerve root or cord compression.
• Radiology : Destructive lesions, virtually arising from midline.

32. Adamantinoma
Arises from aberrant epithelial cells
Prediliction to Mandible and Diaphysis of
tibia
10-35 y; Mostly Cystic and slow growing
Clinically , pain and palpable mass
Pathologically, islands of epithelial cells in
fibrous stroma
Radiologically, Multiple sharp demarcated
lesion. Radiolucent lesions seperated by
areas of dense Sclerotic bone--Honey comb
appearance

33. Summary
• ‘ALTRMCPS’
• PeriostealReactionRadiological features
• D/D s
• Biopsy
• Enneking’s Staging

34. Dr William F Enneking
Thank you.