bone tumors and their management Dr naveen kumar kushwaha, DNB surgical oncology,AHRR

1. BONE TUMORS

6. EVALUATION OF BONE NEOPLASMS
1. History
2. Physical examination
3. Imaging
4. Differential diagnosis
5. Biopsy
6. Staging
7. Tumor board discussion
8. treatment

8. HISTORY
• Clues for primary bone neoplasms
1. Insidious onset pain. Pain before swelling. Pain worse at night or at rest. Pain
increasing on weight bearing
2. Tenderness
3. Swelling
4. Limitation of movement
5. Weakness,fatigue,weight loss
6. Fracture of involved bone- pathological fracture-rare –more for benign and Mets
7. Any previous history of treatment for cancer and history of needle biopsy done
elsewhere
8. No history of bone pains or similar swelling elsewhere- to rule out haemophilias or
other metabolic disorders
9. Reduction in size of swelling with antibiotics

9. • Symptoms of mets will include symptoms of that of primary- most
common- thyroid, breast , prostate, kidney, lungs(goitre,lump,luts-
frequent micturition, hematuria, cough and hemoptysis)
• Family history- le Fraumeni syndrome- cns tumors , bone tumors,
sarcomas, breast tumors
• Past history- h/o childhood malignancy- retinoblastoma

10. PHYSICAL EXAMINATION
Performance status is of not much role in extremity lesions because of pain due to tumor.
• SWELLING
1. Site
2. Size
3. Shape- distance of the swelling from a bony landmark
4. Temperature and tenderness
5. Margins
6. consistency
7. Skin, dilated veins and scars-BIOPSY SCARS- DECIDES SURGERY
8. Pulsatile or not
9. Relation to muscle and the skin
10. Circumference of the swelling needs to be assessed to evaluate post neoadjuvant response
• Distal neurovascular status
• Adjacent joint movements
• Limb length discrepancy- in cases of pathological fracture
• Regional lymphadenopathy(lymph node Mets are more common in Ewing’s(2nd decade) as compared to osteosarcoma)
• gait

12. IMAGING
• X ray -2 views- involves both joint ends in two perpendicular views- extent of lesion with skip mets
• MRI- of the entire length of limb- with contrast
• Evaluation of the chest
• On x-ray see following points- AP and Lateral view of joint in skeletally mature patient
1. Location
2. Type of lesion- lytic or sclerotic or mixed
3. Zone of transition- well defined or not
4. Matrix- in GCT and Ewing's matrix is not appreciable
5. Periosteal reaction- benign or malignant
6. Extraosseous Soft tissue component
7. Pathological fracture
8. Skip Mets
9. Joint involvement

13. ON BASIS OF HISTORY, EXAMINATION AND
IMAGING
1. LOCATION OF THE LESION
2. EXTENT OF THE LESION
3. WHAT IS THE LESION DOING TO THE BONE
4. WHAT IS THE BONE DOING TO THE LESION
5. HINT AS TO ITS TISSUE TYPE AND MATRIX

14. LODWICK CLASSIFICATION
• This classification basically depicts the margin between the tumor and
the viable bone.
• Slowly progressive disease is walled off by native bone giving rise to
distinct margins
• Rapidly progressive disease destroys bone giving rise to indistinct
margins

16. Osteosarcoma,chondrosarcoma, myeloma, mets,
lymphoma

17. Ewing, osteosarcoma, myeloma,mets,infection,
lymphoma

18. Type Ia and Type Ib
• Simple cyst
• Enchondroma
• Fibrous dysplasia
• Chondroblastoma
• GCT
• Chondrosarcoma –rare
• Same as type Ia
• More common in GCT
• Myeloma,mets

19. Reaction of bone to tumor
• Limited responses of the bone
1. Destruction- lysis
2. Sclerosis
3. Remodelling- periosteal reaction
• Lysis and sclerosis depend on the rate of growth of tumor. As far as
periosteal reaction is concerned interrupted is more in favor of malignant
and Mets as compared to continuous
• Complex is in between the two

20. CODMAN’S TRIANGLE-

22. Tumor matrix
• Matrix is the internal tissue of the tumor.
• Most tumor matrix is soft tissue so lytic on x ray
• Cartilage matrix- calcified rings, arcs, dots- enchondroma
,chondroblastoma,chondrosarcoma
• Ossific matrix- osteosarcoma

25. MRI
• Extent of marrow involvement, skip lesion, physis
and joint involvement
• Characterize the lesion
• Relation of neurovascular bundle to the mass
• Also helps to plan biopsy- from solid and not
necrotic areas
• Plan definitive surgery
• Dynamic contrast enhancement MRI- also tells us
about the activity of the lesion
• T1W images are better to assess the bone marrow
involvement. And whether the image is T1W or
T2W can be identified on the basis of joint fluid
colour
T1W image is better because it is very difficult to
differentiate between the edema and the
hyperintense tumor and the marrow on T2W. Also in
T1W fat appears brighter unlike T2W.
• While planning definitive surgery(LSS) – we
cannot contaminate the rectus femoris
compartment.

27. • For pulmonary Mets we do NCCT(most common site of Mets- lungs
followed by bone)
• Important blood parameters-
1. Routine
2. LDH
3. Serum alkaline phosphatase
4. Coagulation profile and viral markers
• LDH and ALP are also used as poor prognostic markers and for
deciding the aggressiveness of the tumor(osteosarcoma)

28. BIOPSY AND STAGING
• BIOPSY is the gold standard
• FNAC is not done for primary diagnosis . It is done in cases of metastasis
and recurrence
• IN CASES OF METSTATIC OR MALIGNANT DISEASE WE TRY TO STAGE THE
DISEASE.
• CT THORAX FOLLOWED BY TECHNETIUM BONE SCAN OR WB FDG PET
• In case of Ewing's sarcoma , bone marrow biopsy has role in metastatic
work up.

29. BIOPSY- MOST IMPORTANT FACTOR
• Biopsy should never be done through a vital structure because that
structure needs to be sacrificed in the definitive procedure either the
knee joint or the patellar tendon
• Biopsy is to be done after history, clinical examination and imaging.----
if done prior to imaging it will change the character of the disease and
it will show the disease extent more and contamination adds up
• Biopsy not indicated in all cases- asymptomatic enchondroma, NOF,
bone cyst, osteochondromas

30. INDICATIONS OF BIOPSY
• Aggressive benign lesions
• Malignant bone tumors
• Infection- can mimic malignancy
• Biopsy also help decide adjuvant treatment.
• Improper biopsy can change the outcome in 12 % of patients.

31. Biopsy is of two types
1. Needle biopsy- no role of FNAC
2. Open biopsy- incisional biopsy
• Sensitivity and specificity of core needle biopsy in musculoskeletal
tumors is 90-100%

32. Principles of biopsy
• Short route
• In line with surgical incision
• Avoid neurovascular structures
• Avoid intercompartmental planes, go via muscles(after biopsy
muscles contract so less chances of bleeding, same is not true for the
intercompartmental plane)
• Avoids joints
• Avoid transverse incision
• Avoid necrotic material

36. GUIDELINES FOR BIOPSY OF BONE TUMORS

38. Always through anterior 1/3 rd
of deltoid never through the
deltopectoral groove

40. JAMSHIDI NEEDLE
• For cystic lesions we scratch the
wall of the cavity with the
needle- then with the help of
syringe we aspirate- do from all
the walls – put it on a gauze and
then send the solid material for
testing.
• For infections send culture and
sensitivity

41. • During open biopsy
1. Avoid exsanguination by compression
2. Deep incision should go through single muscle compartment
3. Don’t raise flaps
4. Minimal retraction
5. Avoid hematoma formation- put bone wax or cement in the hole.
Make a circular hole not a rectangular one because of high chances
of pathological fracture
6. Drain if placed should be in line with incision and not far away

42. Staging systems- bone tumors
1. Enneking’s staging system (MSTS)
2. AJCC staging system

45. TNM for appendicular skeleton, facial bones, skull, trunk

52. • Order of approach in case of bone tumors
1. History and clinical examination
2. Imaging
3. Differential diagnosis
4. Surgical plan
5. biopsy

53. SURGICAL PLANNING
• In high grade osteosarcomas , Ewing's sarcomas and unresectable
osteoclastomas , neoadjuvant chemotherapy is preferred option
1. It treats micro metastasis at the outset of treatment.
2. It tells us about the response of tumor to chemotherapy which is a
surrogate marker for prognosis
3. Allows time for healing and organization of any pathological
fracture if present.
4. Plan surgical treatment and procure any implants or prosthesis if
needed.
5. Enhancement of LSS

54. • NACT does not improve survival(POG-8651 study)
• Parosteal osteosarcoma- low grade malignancy- wide surgical excision
alone without chemotherapy---- on biopsy if areas of
dedifferentiation present then chemotherapy can be given.
• Periosteal osteosarcoma- low grade to intermediate grade
osteosarcoma-----same treatment as the conventional high grade
osteosarcoma

58. • In Ewing's sarcoma, chemotherapy is definitely required and surgery
is generally indicated for extremities where tumor can be completely
excised. If the tumor cannot be excised then definitive RT is the main
modality of treatment.
• However disease control rate with radiotherapy is inferior to surgery.

61. RESPONSE ASSESSMENT POST
CHEMOTHERAPY
• Subjectively patients symptoms improvement
• Clinical (girth of the tumor) and radiological assessment

62. SURGICAL OPTIONS
1. LIMB SALVAGE SURGERY
2. AMPUTATION

63. PRINCIPLES OF LIMB SALVAGE SURGERY AND
RECONSTRUCTION TECHNIQUES
• Two basic components
1. Resection- achieve adequate margin
2. Reconstruction-options are non biological(endoprosthesis and temporary
spacers) and biological(ECRT, bone grafts, rotationplasty)
Both are needed for optimal function
• In limb salvage cases generally the patient had received neoadjuvant
therapy and there is adequate chemotherapy gap prior to LSS
• Our aim is wide margins and function similar to or above the level of
amputation.
• ONCOLOGICAL ASPECT IS MORE IMPORTANT THAN THE FUNCTIONAL
ASPECT

64. • Enneking concept of margins (MSTS)- for any tumor there are 4 kinds
of margins
1. Intralesional margin- seen in case of benign tumors in which we go
and scoop out the tumor
2. Marginal-we go through the pseudo capsule of the tumor
3. Wide- in this we go through the normal tissue around the tumor
4. Radical- it is an extra compartmental resection in which an entire
compartment involving the tumor is excised.

65. • 2-3 cm longitudinal margins are taken.
• Circumferential margins- in this comes the concepts of natural
barriers- tissues that has any resistance against tumor invasion and
includes fascia, joint capsule, tendon, tendon sheath, periosteum,
vascular sheath, cartilage, pleura peritoneum and
epineurium.(KAWAGUCHI CONCEPT OF QUALITATIVE MARGIN)
• Based on this the barriers are divided into two types- thick and thin
barriers
• Thick barriers- physically strong membranous tissue with white luster
through which underlying tissue cannot be seen. Like- iliotibial band,
joint capsule and peritoneum of infant or young child
• Thin barriers- weaker membranous tissue through which underlying
tissue can be seen. Like the healthy fascia of individual muscle,
peritoneum in adults and vascular sheath and epineurium

66. • Each barrier basically quantitates to a margin.
• Thick barrier- 3 cm margin
• Thin barrier- 2 cm margin
• If a tumor is reaching one surface of the barrier then we have to
reduce by a cm.(decided on the basis of imaging)

67. CHALLENGES TO LSS
• Poorly placed biopsy incisions- skin involved also needs to be resected and then
needs coverage with a flap/ reconstructive surgeries. Also if one biopsy is
negative then we tend to do it from a different site. If we want to change the site
then the next biopsy should be in the line of incision
• Major vascular involvement-
• Encasement of major motor nerve- this is a bigger problem in lower limb as
compared to the upper limb in which we have three major nerves. So one can be
sacrificed
• Pre operative infection- conventional prosthesis cannot be used
• Inadequate motors after resection- generally seen in large tumors involving the
muscles, also when the tumor causes pathological fracture extending into the
joint- we can do a extra- articular resection and then reconstruct with a
arthrodesis.
• Inability to obtain oncologically acceptable margins

68. Reconstruction option
• Only place where reconstruction cannot be used is when there is loss of
motors. Like in cases where there is loss of rectus femoris is there
conventional mega prosthesis cannot be used, so we have to resort to
arthrodesis or amputation
• How to decide best reconstructive option for the patient-
1. Type of resection and age of patient
2. Available motor muscle groups
3. Cost
4. Local skin condition
5. Need for adjuvant treatment
6. Availability of supportive departments – tissue bank, reconstructive
surgery unit and radiation unit

69. • Tumor excision surgeries are of two types
1. Articular resections- in this tumor is removed with a part of the
joint
2. Intercalary resection –in which bone is removed sparing the joint
above and the joint below- we simply have to replace that segment
of the bone

70. UPPER LIMB –ARTICULAR RESECTIONS
• Scapula and Proximal humerus - they behave in a functionally similar
manner- good elbow and hand function and we aim to give a stable
shoulder with minimal active movements
In case of proximal humerus following muscles will be lost- deltoid, rotator
cuff, long head of biceps, part of brachialis , part of triceps origin, insertion of
p major, t major, l dorsi. Axillary nerve is also sacrificed
Axillary nerve sparing resections are also done. But prosthesis loosen over
time.
• Clavicle- as in Ewing's clavicle. partial or complete resection with no
significant restriction of function . Usually difficulty in lifting or throwing
kind of function.

71. • Elbow resections- prosthesis replacements provides a near normal
movements but does not allow them to lift heavy weights
• Distal radius resections- as in case of GCT distal radius- wrist
arthrodesis is performed and good elbow/forearm and hand function
but no wrist movements- ULNAR TRANSLOCATION
• Total humerus resections- it is functionally similar to proximal
humerus resection . Elbow ROM is limited if there is loss of all
muscles attached to humerus.

72. LOWER LIMB- ARTICULAR RESECTIONS
• Proximal femur-Reconstructed with endoprosthesis. Capsule is
reconstructed with a proline mesh. Muscles reattached to the
prosthesis- iliopsoas/ abductors
• Knee- most common site for bone tumors. Mobile joint with
endoprosthesis replacement is the treatment of choice. Arthrodesis is
also compatible with good function(fibular strut or primary cement
spacer).In distal femur cases we usually ambulate the patients from
the next day and in cases of proximal tibia we usually wait for 6 weeks
for the soft tissues to heal(in this we attach the patellar tendon to the
prosthesis or we put a gastrocnemius flap cover).
• Total femur resections-

73. • Ankle- when we do articular resection of lower end of tibia, centralize
fibula and do ankle arthrodesis

74. INTERCALARY RESECTIONS- LOWER LIMBS
• Preserve the joint functions
• Various modalities are used
a. Extracorporeal radiation therapy- tumor bone is excised and we give high
dose of radiation – 50 Gy in single fraction and then the same bone is
utilized. Advantages-
• Contoured graft for the defect
• No need of vascular surgeon
• No need of tissue bank
• Good function
Sterilization of the bone segment can also be done by cryo (liquid nitrogen)
or pasteurization.

75. b. Reconstructive surgery team-
1. Flaps
2. Vascularized bone transfers(> 8 cm defect) or composite
grafts(allograft and autograft)
c. Intercalary resections - nail cement spacers, diaphyseal prosthesis

76. • Tibia- tibialisation of fibula
• Pelvic resections- types
1. iliac
2. Peri acetabular
3. Pubic
4. sacral

77. • In pelvic tumors- resections not involving the acetabulum , no
reconstruction is required. We keep the patient in bed for 4 weeks followed
by progressive weight bearing.
• Resections involving the acetabulum – reconstruction is done
1. ECRT
2. Arthrodesis- remaining femur with the pelvis
3. Mesh pseudo arthrodesis- proline mesh is used to secondarily stabilize
the head of femur.
• Pathological fracture is not a contraindication for limb salvage surgery but
it increases the incidence of local recurrence. But it depends on the
response to chemotherapy and the degree of contamination.

78. AMPUTATION
• INCISION
• NEUROVASCULAR STRUCTURES
• MYOPLASTY OR MYODESIS

79. NAVIGATION AND PSI
• Computer assisted tumor surgery
• Helps in complex resection
• Reduces morbidity

80. POST OP ROUNDS
• We can start weight bearing immediately the next day along with the
knee range of movements.
• Palpate for distal pulses
• Check for limb length discrepancy
• Drain output

81. FEATURES TO LOOK ON PATHOLOGY REPORT
• Primary diagnosis
• Grade of tumor
• Percentage of necrosis
• Resection margins- along the bone as well as circumferential- if
margins are positive on HPE after LSS then better to go for
amputation due to high chances of local recurrence in case of
osteosarcoma

82. PROGNOSTIC FACTORS
• Metastasis – single most important factor- isolated pulmonary lesion
better
• Size of tumor
• Site- pelvic and axial tumors- poor
• Response to induction therapy
• Complete surgical removal- wide/radical margins

83. FOLLOW UP
• On biopsy report , response assessment of neoadjuvant
chemotherapy is checked on the basis of huvos grading
• For osteosarcoma we give chemotherapy in the form of either
methotrexate based or non methotrexate based regimens. Commonly
used are-
1. MAP
2. IAP
3. AP
4. AIM
• 3 cycles before followed by 3 cycles after surgery.

85. Picci’s grading – Ewing’s sarcoma based on
percentage of viable tumor
• Grade I- evidence of macroscopic foci of viable tumor cells
• Grade II- microscopic foci
• Grade III- no foci of tumor cells present

86. • CHANGING CHEMOTHERAPY IN POOR RESPONDERS DOES NOT HELP
IN IMPROVING SURVIVAL IN CASE OF OSTEOSARCOMA.(Scandinavian
study group trial)

89. SUMMARY

93. THANK YOU