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Bone Tumors Pre Management
(Malignant Conditions)
Dr Sasikumar Sambasivam
Introduction
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0.001% of all cancers
Benign and malignant tumors
Benign – latent, active and aggressive
MC ...
Benign
Classification and TypesMalignant
of Bone
Myeloma
Reticulum cell sarcoma
Tumor
Chondrogenic (20.9%)
Osteochondroma
...
LOCATION
In the transverse plane:
a) Central – Enchondroma
b) Eccentric -GCT, osteosarcoma,
chondromyxoid fibroma
c) Corti...
In the longitudianl plane

Epiphysis

Metaphysis

Diaphysis

Chondroblastoma
Osteoclastoma
Clear Cell Chondrosarcoma

Oste...
Etiology
• Unknown
• Conditions Predisposing:
– Li Fraumenni
– RB
– Pagets disease
– Bone Infarct
– Radiation
– Osteomyeli...
Natual History
•

Three mechanisms of growth and extension

– (1) compression of normal tissue,
– (2) resorption of bone b...
Spread
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•
•
•

Exclusively Haematogenous
MC--- Lung, Bone
Early Lymphatic Spread -rare
SKIP METS:
– As a tumor nodule loc...
Clinical Presentation
• Generalised body aches
• Localised Swelling; May not be obvious especially if the
tumour is deep
•...
Diagnostic Evaluation

• Basic Invetigations
• Plane Radiology of the involved part

– l/f Marginless border and Periostea...
• Bone Scans:
– Determine Metastatic disease
– Intraosseous extension of
tumor
– Doesn’t detect Skip mets

• Angiography:
...
Biopsy Technique and Timing
• The biopsy of a suspected bone tumor must
be performed with great care and skill.
• The cons...
STAGING SYSTEMS
Musculo Skeletal Tumor
Society Classification
Stage
IA

Grade
Low (G1)

Site
Intracompart
mental (T1)

IB
...
AJCC

Ewing’s sarcoma ----Grade 4
Benign Vs Malignant
• Age of Skeleton –
- Mature or Immature

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•
•
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Location
Transitional zone
Reactive zone
Ma...
Radiographic differences between
Benign& Malignant IGNANTR
Tumours
BENIGN

MALIGNANT
Periosteal Reaction

SOLID
Benign

Spiculated
V Aggressive

Lamellated
Aggressive

Codman's
Periosteal Reaction
Sun Burst appearance

Codman’s Triangle
Mankin – Biological behavior
Criteria
Score
0

1

Size

Small

Big

Score
0-1 Benign

Margination

Present

Absent

2

Agg...
Osteosarcoma
Symptoms: Progressive pain/night pain or painless mass.
Mostly metaphyseal, 10% diaphyseal, 1% epiphyseal
...
• Incidence of pathological
fracture <1%
• Radiologically,
– Increased intramedullary
radiodensity(calcified
cartilage),
–...
Parosteal
Osteosarcoma
• Low grade malignancy
• Rare , elderly , Juxtacortical,
• Better Prognosis than
Classical OS.
• MC...
Intramedullary
Osteosarcoma
• Rare
• Low grade

Small cell
Osteosarcoma
• Rare
• High grade
• Resemble Ewing sarcoma
or Ly...
Secondary Osteosarcoma
• Older patients
• These occur at the site of another disease process
• E.g.
– Paget’s disease– Pag...
Chondrosarcoma
• 9% of primary malignancies of bone
• Age: broad, primary
chondrosarcoma peak around 30 –
60yrs, secondary...
Clinically: Increasing pain and palpable mass. Pain in absence of
pathological fracture is helpful to differentiate betwee...
Giant Cell Tumour
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•
•
•
•

•
•
•
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Rarely Malignant
Locally aggressive , recurrent tumor, low
metastatic potential
Has ...
Ewing sarcoma
•
•
•
•
•
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Second MC primary bone tumor in paediatric groups
MF chest wall tumor of children
9% of pri...
Radiologically
• Destructive lesion in the
diaphyses of longbone
with “onion skin”-Lamellated periosteal
reaction.
• No ne...
Small Round Cell tumors of Bone
•
•
•
•

Ewing’s sarcoma
Primary Lymphoma of bone
Metastatic Neuroblastoma
Embryonal
Rhabd...
Chordoma
• Locally malignant neoplasm from notochordal remnants in the
midline of the neural axis
• AGE: > 40 y
• Site: En...
Adamantinoma

Arises from aberrant epithelial cells
Prediliction to Mandible and Diaphysis of
tibia
10-35 y; Mostly Cys...
Summary
• ‘ALTRMCPS’
• PeriostealReactionRadiological features
• D/D s
• Biopsy
• Enneking’s Staging
Dr William F Enneking

Thank you.
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Bone tumors pre management

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Bone tumors pre management

  1. 1. Bone Tumors Pre Management (Malignant Conditions) Dr Sasikumar Sambasivam
  2. 2. Introduction • • • • • • • • 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. 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. 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. 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. 6. Etiology • Unknown • Conditions Predisposing: – Li Fraumenni – RB – Pagets disease – Bone Infarct – Radiation – Osteomyelitis
  7. 7. Natual History • 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. 8. Spread • • • • 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. 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. 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 – – – – 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. 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. 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. 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. 14. AJCC Ewing’s sarcoma ----Grade 4
  15. 15. Benign Vs Malignant • Age of Skeleton – - Mature or Immature • • • • • • • • Location Transitional zone Reactive zone Matrix Cortex Periosteal reaction Soft tissue swelling Courtesy : slideshare
  16. 16. Radiographic differences between Benign& Malignant IGNANTR Tumours BENIGN MALIGNANT
  17. 17. Periosteal Reaction SOLID Benign Spiculated V Aggressive Lamellated Aggressive Codman's
  18. 18. Periosteal Reaction Sun Burst appearance Codman’s Triangle
  19. 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. 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. 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. 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. 23. Intramedullary Osteosarcoma • Rare • Low grade Small cell Osteosarcoma • Rare • High grade • Resemble Ewing sarcoma or Lymphoma
  24. 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. 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. 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. 27. Giant Cell Tumour • • • • • • • • • 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. 28. Ewing sarcoma • • • • • • • • 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. 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. 30. Small Round Cell tumors of Bone • • • • Ewing’s sarcoma Primary Lymphoma of bone Metastatic Neuroblastoma Embryonal Rhabdomyosarcoma • Small cell Osteosarcoma • Mesenchymal cell Chondrosarcoma • Metastatic small cell carcinoma
  31. 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. 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. 33. Summary • ‘ALTRMCPS’ • PeriostealReactionRadiological features • D/D s • Biopsy • Enneking’s Staging
  34. 34. Dr William F Enneking Thank you.
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