benign aggressive bone tumors

1. Benign aggressive bone
tumors

2. INTRODUCTION
● aggressiveness of these lesions is between
purely benign and frankly malignant.
● Today in this seminar brief dicussion of such benign
aggressive tumors would be done
● GIANT CELL TUMOR
● CHONDROBLASTOMA
● OSTEOBLASTOMA
● CHONDROMYXOID FIBROMA
● LANGERHANS CYST HISTOCYTOSIS

3. Giant Cell Tumor
● Sir Astley Cooper first describe in 1818 emphasizing its
benign nature.
● Dr Paget called it “brown or Myeloid tumour” in 1853.
● WHO- ‘’An aggressive , potentially malignant lesion’’
Its an aggressive lesion characterised by well- vascularised
tissue composed of spindle cells and multinucleated giant cells
uniformly dispersed throughout the tumour.

4. Incidence
● acc to mayo clinic series 5% of neoplasms
of bone.
● common in some parts of andhra pradesh in
india.

5. Age groups involved.
● Usually after epiphyseal closure.
● Hence age group involved is 20 to 40
years(70%) with slight female predominance
(3:2 ).
● Gradual Decrease in after fifth decade.

6. Site of Involvement
Situated mostly at epiphyseo-metaphyseal region of long bones.
AROUND KNEE JOINT 55 %
DISTAL END OF RADIUS 10-12 %
UPPER END OF HUMERUS 7%
LOWER END OF TIBIA 3-4 %
SMALL BONES OF HAND AND FEET 2%
SPINE ,SKULL AND PELVIS 15-17%

7. CLINICAL PRESENTATION
● complains of vague persistent pain over the
swelling at end of long bones…swelling
present from few weeks to several months.
● Note- This pain could have increased after
pathological fracture which may bring the
patient to surgeon for first time.

8. Clinical Picture
Overlying skin is stretched , shiny
with no engorged veins.
● PALPATION- The swelling is
warm , smooth with variable consistency, predominantly
bony and tenderness is present on firm palpation.
● EGG SHELL CRACKLING-ELICITABLE WHEN THERE
IS PATHOLOGICAL FRACTURE OR TOO MUCH
THINNING OF CORTEX.

9. ROM at adjacent joint can be restricted due to
mechanical block.

10. Radiological featuresPlain Radiograph-
● Tumour will show large , sharply
circumscribed area of reduced density
asymmetrically located in the epiphysis,
begining subcortically and extending
towards metaphysis.
● SOAP BUBBLE APPEARANCE-
multilocular and trabeculated appearance of
tumor.
● Geographical destruction type of osteolysis.
● cortical thinning.
● no sclerotic rim and new bone formation.

11. Computed Tomography
● Helps in confirming the integrity
of cortex and outlining the
tumour extent.
● Subcortical destruction can
be well appreciated with a
ct scan.
● Limitation- soft tissue
extension of tumour and its
relationship with adjacent
structures cannot be seen .

12. M.R.I
● With mri the morphologic analysis
and extent of disease into
surrounding soft tissue can be
assessed .
● As in CT SCAN, sub cortical
destruction can be well appreciated
by multiplanar mri.
● Intramedullary tumors best
appreciated in T1 weighted images.
● Extraosseous Tumors best
appreciated in T2 weighted images.

13. ANGIOGRAPHY
● to check Relationship of major
vessles to large tumors.
● Does not correlate with
grading of tumor.

14. RADIONUCLIDE SCINTIGRAPHY
● GCT takes up increased uptake of technetium
99.
● Again does not have any correlation with
grading of tumor.

15. Biopsy
This is the final diagnostic tool for diagnosis of gct.
Sample can be taken by
● OPEN INCISIONAL BIOPSY
● FINE NEEDLE ASPIRATION
● CORE NEEDLE BIOPSY

16. GROSS APPEARANCE
● Epiphyseal end of long bone will be expanded with
thining of periosteum and cortex, being easily broken
by handling.
● Composed of ragged , very friable, readily bleeding
tissue containing variously sized cavitations and
small cysts.
● colour -varies from reddish brown to chocolate color
in which vascular tissue predominates , to greyish or
mottled where connective tissue is major component.
● no evidence of periosteal new bone formation.
● the inner wall of tumor is lined by a fibrous capsule
from which the septae extend inwards to partition the
tumor.

17. MICROSCOPIC APPEARANCE
● Multinucleated giant cells and spindle
cells are the main component .
● GIANT CELLS in this tumor are
characteristic and specific having size
of 10 to 100 microns with centrally
placed uniform sized nuclei
numbering 15 to 150 max.
● SPINDLE CELLS- They are oval
,elongated and contain relatively
large chromatinized nucleus and
small acidophilic cytoplasm

18. ● The appearance of spindle cells indicates the malignant
potential of tumor.
POINT TO BE NOTED-
● Appearance of giant cells is not diagnostic. Giant cells are
also seen in lesions like aneurysmal, unicameral cyst, non
ossifying fibroma, chondroblastoma and brown tumor of
hyperparathyroidism
● appearance of spindle cells is important.(main neoplastic
component)

19. GRADING OF GCT

20. CAMPANACCI RADIOGRAPHIC GRADING
● Grade 1- tumor has well
marinated border of a thin rim
of mature bone, and the
cortex is intact or slightly
thinned but not deformed.

21. ● Grade 2- tumor has relatively
well defined margins, but no
radio opaque rim, cortex is thin
and expanded but is present.

22. Grade 3- cortex is perforated
with extension of tumor into soft
tissue.

23. Enneking staging of Giant cell tumor
Based on clinical radiological and histopathological features
Stage 1- LATENT(10 - 15%)
● Patient is asymptomatic, discovered incidentally.
● May be associated with pathological fractures.
● Radiologically-tumor is intracapsular, with well defined margins and sclerotic rim.
no cortical destruction.
Stage 2- ACTIVE ( 70-75 %)
● Patient is symptomatic.
● Often associated with pathological fracture.
● Radiologically- intracapsular, has expanded or thinned out cortex ,but there is no
breakthrough the cortex.

24. Stage 3 (aggressive) : 10 - 15 %
● Symptomatic
● Rapidly growing mass
● Radiologically- Extracapsular, has cortical
perforation
● Will show intense vascularity on angioram

25. Note-
TNM Classification system is not applicable to
GCT because anatomically it remains
intracompartmental for a long time within well
formed capsule of the periosteum and soft
tissue.

26. Giant Cell Variants
1. Aneurysmal bone cyst
2. Brown tumor of hyperparathyroidism
3. non ossifying fibroma
4. Unicameral bone cyst
5. Fibrous dysplasia
6. Chondroblastoma
7. Giant cell reparative granuloma
8. ossifying fibroma
9. osteogenic sarcoma

27. MANAGEMENT AND COMPLICATIONS OF
GCT

28. INTRODUCTION
● Majority of tumors are benign, have 30 to 40
% recurrence and has tendency to
metastasize.

29. AIM OF TREATMENT
● To eradicate the growth completely at initial operation
● to avoid ablation of limb
● to maintain possible function

30. ● Traditionally
: intralesional curettage/resection with bone graft
:recurrence 35-42 %
● En Bloc resection
: recurrence 10%
: multiple complications

31. ● HISTORICALLY TREATMENT CONSISTED OF SIMPLE CURETTAGE
● BUT RECURRENCE RATES > 50%
● FOR DEFECTS AFTER RESECTION OR CURETTAGE,EITHER
ALLOGRAFT OR BONE CEMENT USED AS FILLING AGENTS
EXTENDED CURETTAGE –USE OF A POWER BURR TO ENLARGE THE
CAVITY 1-2 CM IN ALL DIRECTIONS IS NOW CONSIDERED STANDARD

32. ADJUNCT Tx
●PMMA, Liquid N2, Phenol, l,electrocautery.
–Local extension of margin
–Kill residual foci and remaining tumour cell
● ASSOCIATED WITH PATHOLOGIC
FRACTURES & WOUND HEALING PROBLEMS

33. Bone graft
ADVANTAGE:
• RESTORING NORMAL BIOMECHANICS TO JOINT
SURFACE
•PREVENT FUTURE DEGENERATIVE JOINT DISEASES
•RESTORING BONE STOCK

34. •DISADVANTAGES
: JOINT MUST BE PROTECTED FOR AN EXTENDED
PERIOD OF TIME TO PREVENT A PATHOLOGICAL
FRACTURES.
● TUMOUR RECURRENCE IS DIFFICULT TO
DISTINGUISH FROM GRAFT RESORPTION.

35. Bone cement
•THE ABOVE DISADVANTAGES OVERCOME BY USE OF BONE
CEMENT
•PROVIDES IMMEDIATE STABILITY-HENCE QUICKER
REHABILITATION
•EASIER DETECTION OF RECURRENCE SEEN AS EXPANDING
RADIOLUCENCY ADJ TO CEMENT
•KILLS RESIDUAL TMR CELLS THROUGH POLYMERISATION
HEAT.
● CYTOTOXIC AGENTS- METHOTREXATE AND ADRIAMYCIN
CAN BE INCORPORATED IN BONE CEMENT.

36. Curettage with bone
cementing

37. En bloc excision
•INITIAL PROCEDURE OF CHOICE AND HERE 2 CM OF NORMAL
BONE IS ALSO EXCISED
•
•DEFECTS ARE FILLED WITH CANCELLOUS BONE
GRAFTS,FREEZE DRIED ALLOGRAFT OR PROSTHESIS

38. resection of tumor in lesions of
sacrificable part
● lower end ulna
● upper end of fibula
● phalanges
● metatarsal rays
RECURRENCE IS ALMOST NILL

39. Resection and
reconstruction with
Autograft

40. •AROUND THE KNEE,A HEMI CONDYLAR OSTEOARTICULAR
ALLOGRAFT RECONSTRUCTION OR A ROTATING HINGE
ENDOPROSTHESIS MAY BE NECESSARY
•FOR AGGRESSIVE LESION OF DISTAL RADIUS,PRIMARY
RESECTION AND RECONSTRUCTION WITH A PROXIMAL
FIBULAR AUTOGRAFT INDICATED

41. EXCISION AND RECONSTRUCTION
•FOR GCT AFFECTING LOWER END OF FEMUR OR UPPER END
OF TIBIA
• AFTER EN BLOCK EXCISION RECONSTRUCTION CAN BE
DONE BY
1.TURN-O-PLASTY TECHNIQUE
2.ARTHRODESIS
3.ARTHROPLASTY

42. RADIOTHERAPY- Disappointing recurrence rate of 50-70%.
● malignant sarcomatous transformation ocuured in 7-10 %
cases
CHEMOTHERAPY- NO EFFECTIVE CHEMOTHERAPEUTIC
AGENTS AVAILAIBLE FOR MANAGEMENT OF GCT.

43. RECURRENCE OF LESIONS
● Most local recurrences and pulmonary metastases occur within 3
years or even upto 20 years.
● Patient should have radiograph of primary tumor site and of the
chest at
>> 3 MONTHS INTERVAL FOR 1YR
>> 6 MONTHS INTERVAL FOR NEXT 2 YRS
>>AND ANNUALLY THEREAFTER

44. •treatment is same as for primary lesions.
•after biopsy shows that tumor is still
benign,repeat curretage or resection is
performed.

45. CHONDROBLASTOMA

46. INTRODUCTION
● Codman in 1931 first described..”Codmans
tumor”
● It is a rare , benign bone tumor of immature
cartilage cells derivation arising in epiphysis
consisting of polygonal chondroblast, foci of
chondroid, osteoclast like giant cells and
small foci of calcifications.

47. ● INCIDENCE- less than 1 % of primary bone
tumors.
● AGE
Ranges - 3 to 73 years.
Usually teenagers, before obliteration of epiphyseal
plate.
90% Reported in 5 to 25 years.
● SEX
Male:female 2:1

48. ● SITE
Epiphyseal region of long bone, occasionally extends to adjacent
metaphysis.
● COMMON OCCURRENCE
lower extremity(72%)...in which 50 % cases have been reported
knee
Proximal humerus 18%
Proximal Tibia 17 %
Distal Femur 16 %
Proximal femur 16%
Ankle bones 9%

49. SIZE - 1 to 6 cm
CLINICAL FEATURES-.
>Pain & swelling – several months.
>May be referred to nearest joint
>Some loss of joint function & muscle wasting.
>Joint effusion esp. around knee.
>Pathological fractures – rare.

50. RADIOLOGICAL FEATURES
X – RAYS:
>Lytic area –
>Oval or round
< ½ of epiphyseal area.
>Thin rim of sclerosis
>Punctate or streaky calcification.

51. ● In metaphyseal extension,
i.e tumor crossing growth
plate results in –
Eccentric location &
bulging expansion of
cortex

52. PATHOLOGY
Gross:
● Usually well demarcated
lesions.
● Capsule – thin, easily
disruptable
● Cut section- Soft, reddish –
purple, friable, focally fritty
tissue.
● Cystic spaces and
haemorrhages may be seen.

53. MICROSCOPIC:-
● Islands of chondroblasts
with uniform polyhedral
closely packed cells.
● Background of fibrous
stroma cells within the
islands – PAVING
STONE APPEARANCE.

54. ● Cells – round, plump and active little
immature matrix is present.
● Pericellular lattice-like fine
calcification – “CHICKEN WIRE” or
“PICKET FENCE” pattern are seen.
● Small granular purplish areas of
microcalcification.
● Multinucleated Giant cells –
scattered in stroma.
● Often prominent, dilated blood
vessels at centre & periphery are
present.

55. DIFFERENTIAL DIAGNOSIS
● Enchondroma
Hand, diaphysis.
● G.C.T.
Eccentrical, soap bubble or trabecular pattern
No calcifications
Often after closure of growth plate.
● Central chondrosarcoma
Slow growth, severe pain & margins not demarcated on xrays.

56. ● Chondromyxoid fibroma
Site- Metadiaphysis
Septae are present.

57. TREATMENT AND PROGNOSIS
● Curettage and autologous bone grafting – high recurrence rate (10 –
35%).
● Close follow up & observation for all till skeletal maturity – in
patients , whose potential growth is remaining and lesions abuts the
epiphyseal plate, lesions should be followed up and observed, instead
of premature or aggressive treatment
● Marginal extra capsular excision – when growth plate not at all at
risk, curettage and excision is done.
Defect can be obliterated with Autogenous bone graft.

58. ● Methacrylate adjunct –
when excision impractical & intracapsular curettage
is of high risk of recurrence due to surgical
inaccessibility.
● Curettage followed by cryosurgery:
>In case of recurrence or when associated with ABC.
>Yields consistent good results with a high cure rate when
entire tumor is adequately frozen by liquid nitrogen.

59. Radiosensitivity:
● Radiosensitive tumor
● Not used for uncomplicated cases – potential hazards
of irradiation induced malignant transformation.

60. OSTEOBLASTOMA

61. INTRODUCTION
● Jaffe and lichtenstein first described it in 1956.
● Rare bone tumor,
● 1 % of all primary bone tumors
● 3 % of benign bone tumors.
It is a Solitary benign progressively growing bone tumor
histologically similar to osteoid osteoma , size, clinical and
radiological features differentiate it.

62. AGE
● Young person 10 to 30 years (80%)
SEX
● Males: female -3:1.

63. SITE
Vertebral Column 34 %
Extremities 30%
Hands and feet 14%
Skull and facial bones 15 %

64. ● In long bones it occurs in medullary portion of metaphysis.
Rarely juxtacortical or periosteal osteoblastomas are
reported.
● In Spine- spinous and transverse process are frequently
involved. lesions in body are rare.

65. Clinical features
● Pain- Dull , aching, persistent and localized.
● Slight local tenderness and palpable swelling of increasing
size.
● spinal location
> radiculopathy(50%).
>rarely produce neurological symptoms .
>scoliosis and muscle spasm may appear.
● lesions of extremities may present with wasting and limp.

66. RADIOLOGICAL FEATURES
● lesion is well circumscribed, radiolucent,
eccentric and with an intact surrounding shell
of bone .
● centrally there is lytic area >2cm with
surrounding sclerosis.

67. ● In vertebrae it is seen as
definitely expansile radiolucent
growth containing granular
radiopacities
● ct scans are better appreciated
for vertebral lesions

68. CT SCAN
.
● it can show cotton wool
appearance when
calcification of tumor tissue
has occured...if cotton wool
appearance is seen in
lesion...it is diagnostic.

69. PATHOLOGY
GROSS
● well circumscribed, 2-10 cm.
● surrounded by shell of cortical bone or thickened
periosteum.
● Haemorrhagic, granular , friable and calcified
lesions.

70. HISTOPATHOLOGY
•Vascular spindle-cell
stroma with abundant
irregular spicules of
mineralized bone and
Osteoid

71. DIFFERENTIAL DIAGNOSIS
OSTEOBLASTOMA OSTEOID OSTEOMA
1. INCIDENCE one fifth as common as osteoid
osteoma
10% of benign bone tumor
2. COMMONEST LOCATION Vertebral column, often posterior
element
Proximal femur
3.CLINICAL PRESENTATION Pain inconsistent pain persistent , nocturnal
4. RADIOGRAPHY Size > 2 cm
no or minimal perifocal osseous
reaction
size < 2cm, perifocal osseous
reaction is marked.
5.HISTOLOGY
>Osteoid trabeculae with
discontinuous and irregular bone
formation,
> abundant fibrous stromal
reaction,
>many multinucleated osteoblastic
giant cells
>osteoid trabaculae with
continuous and regular bone
formation.
>Scanty stromal reaction
>multinucleated osteoblastic giant
cells are rare.

72. ● OSTEOSARCOMA
No ALP rise in osteoblastoma, stromal cells are not large, but
are relatively uniform.
● GCT
>>more than half are located near knee, or distal radius.
>>soap bubble appearance.

73. TREATMENT
● Curettage and resection.
● bone grafting of defect.
● In spine- if resection causes instability,
instrumented fusion can be necessary.
● Radiation- some authors have recommended,
adjuvant radiation therapy for spinal lesions, as
revision surgery is difficult.

74. CHONDROMYXOID
FIBROMA

75. INTRODUCTION
● Rare tumour, least common benign cartilaginous tumors.
● Can be misdiagnosed as chondrosarcoma...as it is
composed of similar cytological features, however
radiological and clinical findings are of benign tumor .

76. AGE
● 2nd and 3rd decade, rare in children.
SEX
● No predominance.
LOCATION
● metaphyseal region of large tubular
bones.(eccentric)
● can also involve thin tubular bones- fibula,
phalanx and calcaneus.(usually entire width)

77. CLINICAL PICTURE
● In young adults tumor causes mild or no pain,
slowly increasing local swelling and palpable
tender tumor.
● Rarely metastasizes
● In less than 10 years age group- symptoms
are more pronounced.

78. RADIOLOGICAL FINDINGS
XRAY
● in long bones
>>Lesion -translucent mass of variable
sizes , located eccentrically in metaphysis
>> on medullary aspect of lesion- margin
is scalloped and sclerosed.
>>Cortex - Expanded and thinned out,
may appear interrupted.

79. ● in case narrow tubular bone
(fibula) or small tubular bone
(phalanx)- generally entire
width of bone is involved
● fusiform expansion and
thinning of both cortices.

80. PATHOLOGY GROSS
● Outer surface- covered by thin shell of newly
formed periosteal bone.
● Cut Surface-solid tumor mass of greyish white
or bluish grey color,resembles cartilage and
sometimes contain cavities of mucoid tissue
● consistency - usually firm.
● Calcified areas are unusual.
● no bony septa traverse the tumor.

81. MICROSCOPIC
•Tumor is composed of lobulated
and pseudo lobulated areas of
stellate cells with indistinct
cytoplasmic borders,lying within
the central portion of lobule and
widely separated by mucin like
material.

82. TREATMENT
● Curettage is not sufficient, as tumor may recur.
● En Bloc excision and filling cavity with
autogenous bone results in high rate of cure.
● Despite Wide excision , if tumor recurs then
additional studies should be done to rule out
malignant transformation.

83. Curettage and Bone cementing

84. LANGERHANS CELL
HISTIOCYTOSIS

85. ● Previously called HISTIOCYTOSIS X
● Characterised by widespread dissemination of
reticuloendothelial system.
● Syndrome constitutes following clinical conditions
1. Eosinophilic Granuloma
2. Hand -Schuller -Christian disease
3. Letterer -Siwe disease.

86. Orthopaedic surgeon is primarily concerned
with
EOSINOPHILIC GRANULOMA OF BONE

87. EOSINOPHILIC GRANULOMA
● Self limited benign bone destructive lesion characterised by histocytic
and eosinophilic leucocyte infiltration.
Incidence
● Usually in first two decades with peak incidence in 5 to 10 years group.
● solitary lesion usually seen in ribs vertebrae, skull, flat bones and long
tubular bones.
● multiple lesions are rare - ocurrance in skull and femur
● diaphysis and metaphysis are commonly involved.

88. Clinical Picture
● Acute onset
● complains of dull aching pain in a limited local area of bone.
● local tenderness , warmth and swelling over the affected bone.
● pathological fracture of long bone or vertebral collabs can be
seen.
● Disease usually progress for few weeks to few months , and
recovery is usually spontaneous

89. RADIOGRAPHY
● Rapidly destructive lytic process
producing punched out
appearance

90. ● In diaphysis of long bones lesions may
have aggressive permeative appearance
with periosteal reactive bone formation.

91. ● Eosinophilic granuloma of
vertebral body
● showing marked flattening of
vertebral body or vertebral
plana

92. Histopathology
•Diagnosis is made by identification of
langerhans cell.
•Mixture of eosinophils, plasma cells,
histiocytes along with peculiar large
mononuclear giant cells (Langerhans cells)
with abundant pale cytoplasm and cleaved
nuclei.
•Necrosis, fibrosis and reactive cells are
evident

93. TREATMENT
● Microscopically diagnosis is made by identification of
langerhans cell.
● Recommended treatments incd-
>> corticosteroid infiltrations
>> radiation therapy
>> Curettage with or without bone grafting.

94. Hand schuller - christian disease

95. REFRENCES
1. ORTHOPAEDICS PRINCIPLES AND THEI APPLICATION: SAMUEL L TURES
2. CAMPBELLS OPERATIVE ORTHOPAEDICS
3. TEXTBOOK OF ORTHOPAEDICS AND TRAUMA - GS KULKARNI
4. J.B.JS.