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By : Dr. Utsav Agrawal
PAIN :- is most frequent symptom
    -deep constant pain,poorly localised,worse at night
    -initially controlled by analgesics,later requires narcotics

MASS:- may be painful/painless
    - rate of enlargement is important
    -Fluctuating mass can be cyst,ganglion or hemangioma
    -Family H/O masses near the joint may be indicator of Ollier’s disease or
Maffucci Syndrome

NEUROLOGICAL SYMPTOM:- found in few patients such as sacral tumors & with
tumors located near the nerve causing compression of nerve,especially common in
sciatic notch ,inguinal canal & popliteal fossa
UNEXPLAINED SWELLING OF THE LOWER EXTREMITY:- found in pelvic tumors
which are painless & without a palpable mass & cause swelling due to compression of
iliac vein.

Patient may present with an abnormal radiographic finding detected during evaluation
of unrelated problem

Patient may also come with complaint of weight loss, respiratory , gastro or genito-
urinary disturbances.
Carried out in 4 phases –
1st phase – involves
       High index of suspicion for tumors
       Meticulous history
       Thorough physical examination
       Routine X-rays
       Routine lab facilities
2nd phase – is prebiopsy regional evaluation to determine size,
location and type of tissue in involved in the tumor. This includes CT
and MRI
3rd phase – is the actual biopsy.
4th phase – is undertaken if presumptive clinical & path evidence is
suggestive of malignancy. Here search for metastasis is done using
CT/HRCT of lung & Tc-99 bone scan.
AGE:- most important as most tumors present in specific age group.
        1st decade- usually ABC ,SBC
        2nd decade-Chondroblastoma,osteosarcoma,Ewings
        3rd decade- GCT
        4th decade- chondrosarcoma
        5th decade- Multiple myeloma
SEX:- less imp than age
        Some tumors like GCT are more in females
FAMILY HISTORY
RACE:- little imp, Ewings rare in african descent
H/O any exposure to radiation Tt or Carcinogens- bone seeking
radionucleotide can cause sarcoma.
Various chemlcal carcinogens- methylcholanthrene,zinc beryllium
silicate, beryllium oxide.
Currently the most worrisome & controversial is Nickel which is used
in many orthopedic devices.
Risk
 Ollier disease (Enchondromatosis)
 Maffucci syndrome
 Familial retinoblastoma syndrome
 Rothmund-Thomson syndrome (RTS)
Moderate Risk
 Multiple osteochondromas
 Polyostotic Paget disease
 Radiation osteitis
Low Risk
 Fibrous dysplasia
 Bone infarct
 Chronic osteomyelitis
 Metallic and polyethylene implants
 Osteogenesis imperfecta
 Giant cell tumour
 Osteoblastoma and chondroblastoma
Evaluation of patient’s general health
TUMOR MASS should be measured & its location, shape, consistency, mobility,
tenderness, local temp & change with position should be noted.
SKIN & SUBCUTANEOUS TISSUE :
Small dilated superficial veins overlying the mass are produced by large tumors
Café-au-lait spots & subcutaneous neurofibromas indicate Von Recklinghausen’s
disease
A venous malformation on the same of body as the cartilagenous tumor is an
indicator of Maffucci Syndrome
REGIONAL LYMPH NODES:should be carefully palpated for signs of metastatic
disease
Atrophy of surrounding musculature/ fasciculations should be recorded
Neurological deficits
Circulation should be assessed and compared with opposite site.
Joints, specially proximal and distal to the mass should be carefully examined for
range of motion, discontinuity or intra-articular masses.
Location of tumors
Hemoglobin
CBC
ESR
CRP
Prastrate specific antigen, PAP
Electrophoresis & urinary Bence Jones protein
Alkaline phosphatase test: Normally, this enzyme is present in
     high levels when bone-forming cells are very active.High levels
     of alkaline phosphatase can also be an indicator of bone tumors
PTH test

Serum phosphorus

Ionized calcium and serum calcium
It is the most conclusive test
Types : Open Incisional Biopsy 1.Incisional
                                2. Excisional
          Closed percutaneous core Biopsy
The Enneking system for the surgical staging of bone and soft-tissue
tumors is based on grade (G), site (T), and metastasis (M) and uses
histologic, radiologic, and clinical criteria.

Grade
G0 - Benign lesion
G1 - Low-grade malignant lesion
G2 - High-grade malignant lesion
Site
T0 - A benign tumor that is confined within a true capsule and the lesion's anatomic
compartment of origin (ie, a benign intracapsular, intracompartmental lesion)
T1- An aggressive benign or malignant tumor that is still confined within its anatomic
compartment (ie, an intracompartmental lesion)
T2 - A lesion that has spread beyond its anatomic compartment of origin (ie, an
extracompartmental lesion)
Metastasis
M0 - No regional or distant metastasis
M1 - Regional or distant metastasis
Stage   Grade      Site                 Metastasis

IA      Low G-1    Intracompartmental   -
                   T1
IB      Low G-1    Extracompartmental -
                   T2
IIA     High G-2   Intracompartmental   -
                   T1

IIB     High G-2   Extracompartmental -
                   T2

III     Any G      Any T                Regional/ distant
                                        metastasis
Stage                   Definition              Behaviour                  Example

1                       Latent                  Remains static or   Non-ossifying
                                                heals spontaneously fibroma

2                       Active                  Progressive but            Aneurysmal bone
                                                limited by natural         bone cyst
                                                barriers

3                       Locally Aggressive      Progressive growth,        GCT
                                                not limited by
                                                natural barriers


    Barriers- Cortical bone, articular caritlage, joint capsule, fascia,
    tendinous origin and inertion sites of muscles
Primary tumour (T) TX: primary tumour cannot be assessed
       T0: no evidence of primary tumour
       T1: tumour 􀀩 8 cm in greatest dimension
       T2: tumour > 8 cm in greatest dimension
       T3: discontinuous tumours in the primary bone site
Regional lymph nodes (N) NX: regional lymph nodes cannot be assessed
       N0: no regional lymph node metastasis
       N1: regional lymph node metastasis
Distant metastasis (M) MX: distant metastasis cannot be assessed
       M0: no distant metastasis
       M1: distant metastasis
       M1a: lung
       M1b: other distant sites
G Histopathological Grading
Low Grade
High Grade
Stage   T       N       M       G
IA      T1      No/Nx   Mo      G1/2    Low Grade
IB      T2      No/Nx   Mo      G1/2    Low Grade
IIA     T1      No/Nx   Mo      G3/4    High
IIB     T2      No/Nx   Mo      G3/4    High
III     T3      No/Nx   Mo      Any G
IVA     Any T   No/Nx   Any M   Any G
IVB     Any T   N1      Any M   Any G
        Any T   Any N   M1b     Any G
Classification (W.H.O.)
 Bone-forming tumours
 Cartilage forming tumours
 Giant-cell tumour
 Marrow tumours
 Vascular tumours
 Other connective tissue tumours
 Other tumours
 Secondary malignant tumours of bone
Bone forming tumours
 Benign                       Malignant
  Osteoma                     Osteosarcoma
  Osteoid osteoma or          Central (Medullary)
   osteoblastoma               Peripheral (Surface)
 Intermediate                  Parosteal
  Aggressive osteoblastoma    Periosteal
                               High grade surface
Cartilage forming tumours
Benign                    Malignant
 Chondroma                Chondrosarcoma
 Enchondroma               Differentiated
 Osteochondroma            chondrosarcoma
 Chondroblastoma          Juxtacortical
 Chondromyxoid fibroma
                            chondrosarcoma
                           Mesenchymal
                            chondrosarcoma
                           Clear cell chondrosarcoma
Giant cell tumour
 Osteoclastoma
Marrow tumours
 Ewing’s sarcoma
 Neuroectodermal tumour
 Malignant lymphoma of bone (Primary/secondary)
 Myeloma
Vascular tumours
Benign
 Haemangioma
 Lymphangioma
 Glomus tumour
Intermediate
 Haemangio endothelioma
 Haemangio pericytoma
Malignant
 Angiosarcoma
 Malignant haemangio pericytoma
Other connective tissue tumours
  Benign                   Malignant
   Benign fibrous          Fibrosarcoma
    histiocytoma            Malignant fibrous
   Lipoma                   histiocytoma
  Intermediate              Liposarcoma
   Desmoplastic fibroma    Malignant mesenchymoma
                            Leiomyosarcoma
                            Undifferentiated sarcoma
Other tumours
Benign
 Neurilemmoma
 Neurofibroma


Malignant
 Chordoma
 Adamantinoma
Secondary malignant tumours of bone
From primary in:
 Thyroid
 Breast
 Bronchus
 Kidney
 Prostate

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Bone tumours_UTSAV

  • 1. By : Dr. Utsav Agrawal
  • 2.
  • 3. PAIN :- is most frequent symptom -deep constant pain,poorly localised,worse at night -initially controlled by analgesics,later requires narcotics MASS:- may be painful/painless - rate of enlargement is important -Fluctuating mass can be cyst,ganglion or hemangioma -Family H/O masses near the joint may be indicator of Ollier’s disease or Maffucci Syndrome NEUROLOGICAL SYMPTOM:- found in few patients such as sacral tumors & with tumors located near the nerve causing compression of nerve,especially common in sciatic notch ,inguinal canal & popliteal fossa UNEXPLAINED SWELLING OF THE LOWER EXTREMITY:- found in pelvic tumors which are painless & without a palpable mass & cause swelling due to compression of iliac vein. Patient may present with an abnormal radiographic finding detected during evaluation of unrelated problem Patient may also come with complaint of weight loss, respiratory , gastro or genito- urinary disturbances.
  • 4. Carried out in 4 phases – 1st phase – involves High index of suspicion for tumors Meticulous history Thorough physical examination Routine X-rays Routine lab facilities 2nd phase – is prebiopsy regional evaluation to determine size, location and type of tissue in involved in the tumor. This includes CT and MRI 3rd phase – is the actual biopsy. 4th phase – is undertaken if presumptive clinical & path evidence is suggestive of malignancy. Here search for metastasis is done using CT/HRCT of lung & Tc-99 bone scan.
  • 5. AGE:- most important as most tumors present in specific age group. 1st decade- usually ABC ,SBC 2nd decade-Chondroblastoma,osteosarcoma,Ewings 3rd decade- GCT 4th decade- chondrosarcoma 5th decade- Multiple myeloma SEX:- less imp than age Some tumors like GCT are more in females FAMILY HISTORY RACE:- little imp, Ewings rare in african descent H/O any exposure to radiation Tt or Carcinogens- bone seeking radionucleotide can cause sarcoma. Various chemlcal carcinogens- methylcholanthrene,zinc beryllium silicate, beryllium oxide. Currently the most worrisome & controversial is Nickel which is used in many orthopedic devices.
  • 6. Risk Ollier disease (Enchondromatosis) Maffucci syndrome Familial retinoblastoma syndrome Rothmund-Thomson syndrome (RTS) Moderate Risk Multiple osteochondromas Polyostotic Paget disease Radiation osteitis Low Risk Fibrous dysplasia Bone infarct Chronic osteomyelitis Metallic and polyethylene implants Osteogenesis imperfecta Giant cell tumour Osteoblastoma and chondroblastoma
  • 7. Evaluation of patient’s general health TUMOR MASS should be measured & its location, shape, consistency, mobility, tenderness, local temp & change with position should be noted. SKIN & SUBCUTANEOUS TISSUE : Small dilated superficial veins overlying the mass are produced by large tumors Café-au-lait spots & subcutaneous neurofibromas indicate Von Recklinghausen’s disease A venous malformation on the same of body as the cartilagenous tumor is an indicator of Maffucci Syndrome REGIONAL LYMPH NODES:should be carefully palpated for signs of metastatic disease Atrophy of surrounding musculature/ fasciculations should be recorded Neurological deficits Circulation should be assessed and compared with opposite site. Joints, specially proximal and distal to the mass should be carefully examined for range of motion, discontinuity or intra-articular masses.
  • 9. Hemoglobin CBC ESR CRP Prastrate specific antigen, PAP Electrophoresis & urinary Bence Jones protein Alkaline phosphatase test: Normally, this enzyme is present in high levels when bone-forming cells are very active.High levels of alkaline phosphatase can also be an indicator of bone tumors PTH test Serum phosphorus Ionized calcium and serum calcium
  • 10. It is the most conclusive test Types : Open Incisional Biopsy 1.Incisional 2. Excisional Closed percutaneous core Biopsy
  • 11. The Enneking system for the surgical staging of bone and soft-tissue tumors is based on grade (G), site (T), and metastasis (M) and uses histologic, radiologic, and clinical criteria. Grade G0 - Benign lesion G1 - Low-grade malignant lesion G2 - High-grade malignant lesion Site T0 - A benign tumor that is confined within a true capsule and the lesion's anatomic compartment of origin (ie, a benign intracapsular, intracompartmental lesion) T1- An aggressive benign or malignant tumor that is still confined within its anatomic compartment (ie, an intracompartmental lesion) T2 - A lesion that has spread beyond its anatomic compartment of origin (ie, an extracompartmental lesion) Metastasis M0 - No regional or distant metastasis M1 - Regional or distant metastasis
  • 12. Stage Grade Site Metastasis IA Low G-1 Intracompartmental - T1 IB Low G-1 Extracompartmental - T2 IIA High G-2 Intracompartmental - T1 IIB High G-2 Extracompartmental - T2 III Any G Any T Regional/ distant metastasis
  • 13. Stage Definition Behaviour Example 1 Latent Remains static or Non-ossifying heals spontaneously fibroma 2 Active Progressive but Aneurysmal bone limited by natural bone cyst barriers 3 Locally Aggressive Progressive growth, GCT not limited by natural barriers Barriers- Cortical bone, articular caritlage, joint capsule, fascia, tendinous origin and inertion sites of muscles
  • 14. Primary tumour (T) TX: primary tumour cannot be assessed T0: no evidence of primary tumour T1: tumour 􀀩 8 cm in greatest dimension T2: tumour > 8 cm in greatest dimension T3: discontinuous tumours in the primary bone site Regional lymph nodes (N) NX: regional lymph nodes cannot be assessed N0: no regional lymph node metastasis N1: regional lymph node metastasis Distant metastasis (M) MX: distant metastasis cannot be assessed M0: no distant metastasis M1: distant metastasis M1a: lung M1b: other distant sites G Histopathological Grading Low Grade High Grade
  • 15. Stage T N M G IA T1 No/Nx Mo G1/2 Low Grade IB T2 No/Nx Mo G1/2 Low Grade IIA T1 No/Nx Mo G3/4 High IIB T2 No/Nx Mo G3/4 High III T3 No/Nx Mo Any G IVA Any T No/Nx Any M Any G IVB Any T N1 Any M Any G Any T Any N M1b Any G
  • 16.
  • 17. Classification (W.H.O.)  Bone-forming tumours  Cartilage forming tumours  Giant-cell tumour  Marrow tumours  Vascular tumours  Other connective tissue tumours  Other tumours  Secondary malignant tumours of bone
  • 18. Bone forming tumours Benign Malignant  Osteoma  Osteosarcoma  Osteoid osteoma or Central (Medullary) osteoblastoma Peripheral (Surface) Intermediate  Parosteal  Aggressive osteoblastoma  Periosteal  High grade surface
  • 19. Cartilage forming tumours Benign Malignant  Chondroma  Chondrosarcoma Enchondroma  Differentiated  Osteochondroma chondrosarcoma  Chondroblastoma  Juxtacortical  Chondromyxoid fibroma chondrosarcoma  Mesenchymal chondrosarcoma  Clear cell chondrosarcoma
  • 20. Giant cell tumour  Osteoclastoma
  • 21. Marrow tumours  Ewing’s sarcoma  Neuroectodermal tumour  Malignant lymphoma of bone (Primary/secondary)  Myeloma
  • 22. Vascular tumours Benign  Haemangioma  Lymphangioma  Glomus tumour Intermediate  Haemangio endothelioma  Haemangio pericytoma Malignant  Angiosarcoma  Malignant haemangio pericytoma
  • 23. Other connective tissue tumours Benign Malignant  Benign fibrous  Fibrosarcoma histiocytoma  Malignant fibrous  Lipoma histiocytoma Intermediate  Liposarcoma  Desmoplastic fibroma  Malignant mesenchymoma  Leiomyosarcoma  Undifferentiated sarcoma
  • 24. Other tumours Benign  Neurilemmoma  Neurofibroma Malignant  Chordoma  Adamantinoma
  • 25. Secondary malignant tumours of bone From primary in:  Thyroid  Breast  Bronchus  Kidney  Prostate