2. ā¢ Benign tumours
ā¢ Tumour-like conditions of bone
ā¢ Osteoclastoma (GCT)
ā¢ Osteochondroma
ā¢ Primary malignant tumours
ā¢ Aneurysmal bone cyst
ā¢ Some uncommon malignant tumours
ā¢ Fibrous dysplasia
ā¢ Metastasis in bone
3. ā¢ The term ābone tumourā is a broad term used
for benign and malignant neoplasms, as well
as ātumour-like conditionsā of the bone (e.g.,
osteochondroma).
ā¢ Metastatic deposits in the bone are commoner than
primary bone tumours of the primary bone
malignancies, multiple myeloma is the commonest.
4. ā¢ Osteochondroma is the commonest benign
tumour of the bone.
ā¢ Most primary malignant bone tumours occur
in children and young adults; in whom these
constitute one of the common malignant
tumours.
ā¢ The nature of a bone tumour can be
suspected based on the type of destruction
seen on an X-ray ( Tableā28.1).
5.
6. BENIGN TUMOURS
OSTEOMA
ā¢ This is a benign tumour composed of sclerotic, well
formed bone protruding from the cortical surface of a
bone.
ā¢ The bones involved most often are the skull and facial
bones.
ā¢ Generally, the tumour is of no clinical significance
except that it may produce visible swelling.
Treatment:
No treatment is generally required except for cosmetic
reasons, where a simple excision is sufficient.
7. OSTEOID OSTEOMA
ā¢ It is the commonest true benign tumour of the bone.
Clinical presentation:
ā¢ 5-25 years.
ā¢ Tibia being the commonest.
ā¢ The tumour is generally located in the diaphysis of long
bones.
ā¢ Posterior elements of the vertebrae are a common site.
ā¢ The presenting complaint is a nagging pain, worst at
night, and is relieved by salicylates.
8. Diagnosis:
It is generally confirmed on X-ray.
ā¢ The tumour is visible as a zone of sclerosis
surrounding a radiolucent nidus, usually less
than 1 cm in size (Fig-28.1a).
ā¢ In some cases, the nidus may not be seen on a
plain X-ray because of extensive surrounding
sclerosis, and may be detected on a CT scan.
9.
10.
11. Treatment:
ā¢ Complete excision of the nidus along with the
sclerotic bone is done.
ā¢ Prognosis is good.
ā¢ It is not a pre-malignant condition.
12. UNCOMMON BENIGN TUMOURS OF THE
BONE
OSTEOBLASTOMA:
ā¢ This is a benign tumour consisting of vascular osteoid
and new bone.
ā¢ It occurs in the jaw and the spine.
ā¢ It occurs in 2nd decade of life.
ā¢ The patient presents with an aching pain.
Radiologically,
ā¢ It is a well-defined radiolucent expansile bone lesion 2-
12 cm in size.
ā¢ There is minimal reactive new bone formation.
Treatment is by curettage.
13. CHONDROBLASTOMA:
ā¢ This is a cartilaginous tumour containing characteristic
multiple calcium deposits.
ā¢ It occurs in young adults, and is located around the
epiphyseal plate.
ā¢ Bones around the knee are commonly affected.
Radiologically,
ā¢ Well-defined lytic lesion surrounded by a zone of sclerosis.
ā¢ Areas of calcification within the tumour substance give rise
to a mottled appearance (Fig-28.1b).
ā¢ Treatment is by curettage and bone grafting.
14.
15. Haemangioma of the bone
ā¢ This is a benign tumour of angiomatous origin,
commonly affecting the vertebrae and the skull.
ā¢ It occurs in young adults.
ā¢ Common presenting symptoms are persistent pain
and features of cord compression.
ā¢ Typically, one of the lumbar vertebrae is affected.
16. ā¢ Radiologically, it appears as loss of horizontal
striations and prominence of vertical striations
of the affected vertebral body.
ā¢ The importance of this tumour lies in
differentiating it from commoner diseases like
TB spine or metastatic bone disease of the
spine.
ā¢ Treatment is by radiotherapy.
17. ADAMANTINOMA
ā¢ It is a common tumour of the jaw bone; also
sometimes occurring in the tibia in its lower-half.
ā¢ 10-35 years of age.
ā¢ Minimal pain, and it increases in size gradually.
ā¢ Typically, it appears as a honeycomb-like, loculated
lesion on X-ray.
ā¢ Local recurrence is common.
ā¢ Treatment is by resection.
18. CHORDOMA
ā¢ This is a locally malignant, sometimes actually
malignant tumour supposedly originating from the
remnants of notochord.
ā¢ Sacrum and cervical spine are common sites.
ā¢ Persistent pain and swelling,
ā¢ Bone destruction is the only hallmark feature of this
tumour.
ā¢ Treatment, wherever possible, is complete excision.
ā¢ If complete excision is not possible, radiotherapy is
done.
19. OSTEOCLASTOMA (GIANT CELL TUMOUR)
ā¢ Giant cell tumour (GCT) is a common bone tumour
with variable growth potential.
PATHOLOGY
ā¢ The cell of origin is uncertain.
ā¢ Microscopically, the tumour consists of
undifferentiated spindle cells,profusely interspersed
with multi-nucleate giant cells.
20. ā¢ The tumour stroma is highly vascular.
ā¢ These giant cells were mistaken as osteoclasts in the
past, hence the name osteoclastoma.
CLINICAL FEATURES
ā¢ Age group of 20-40 years i.e., after epiphyseal fusion.
ā¢ Around the knee i.e.lower-end of the femur and upper-
end of the tibia.
ā¢ Lower-end of the radius is another common site.
ā¢ Presenting complaints are swelling and vague pain.
21. EXAMINATION
ā¢ Bony swelling eccentrically located at the end of the
bone.
ā¢ Surface of the swelling is smooth.
ā¢ There may be tenderness on firm palpation.
ā¢ A characteristic āegg-shell cracklingā is often not
elicited..
22. DIAGNOSIS
ā¢ GCT is one of the common cause of a solitary lytic
lesion of the bone, and must be differentiated from
other such lesions (Tableā28.3).
RADIOLOGICAL FEATURES
ā¢ A solitary, may be loculated, lytic lesion.Eccentric
location, often subchondral.
ā¢ Expansion of the overlying cortex (expansile lesion).
23.
24.
25.
26. ā¢ āSoap-bubbleā appearance ā the tumour is
homogeneously lytic with trabeculae of the
remnants of bone traversing it, giving rise to a
loculated appearance.
ā¢ No calcification within the tumour.
ā¢ None or minimal reactive sclerosis around the
tumour.
27. TREATMENT
ā¢ Wherever possible, excision of the tumour is the best
treatment.
ā¢ For sites like the spine, where excision is sometimes
technically not possible, radiotherapy is done.
Following treatment
methods are commonly used:
a) Excision: This is the treatment of choice when
the tumour affects a bone whose removal does not
hamper with functions e.g., the fibula, lower-end of
the ulna etc.
28. b) Excision with reconstruction:
ā¢ For example, in tumours affecting the lower-end of
femur, the affected part is excised en bloc, and the
defect thus created made up by one of the following
methods:
ā¢ Arthrodesis by the Turn-o-Plasty procedure (Fig-
28.3a):
29.
30. ā¢ Arthrodesis by bridging the gap by double fibulae
(Fig-28.3b), one taken from same extremity and the
other from the opposite leg (Yadav, 1990).
ā¢ Arthroplasty: In this procedure, the tumour
is excised, and an attempt is made to reconstruct the
joint in some way.
31. c) Curettage with or without supplementary
procedures:
ā¢ Curettage performed alone has the disadvantage of a
high recurrence rate.
ā¢ Cryotherapy, where liquid nitrogen is used to
produce a freezing effect and thus kill the residual
cells, and thermal burning of the residual cells using
cauterization of the walls of the tumour are popular.
ā¢ ābone cementā, which by the heat it produces while
setting, ākillsā the residual cells.
32. d) Amputation: For more aggressive tumours, or
following recurrence, amputation may be necessary.
e) Radiotherapy: It is the preferred treatment method
for GCT affecting the vertebrae.
33. PROGNOSIS
ā¢ Recurrence following treatment is a serious
problem.
ā¢ With every subsequent recurrence, the
tumour becomes more aggressive.
34. PRIMARY MALIGNANT TUMOURS
OSTEOSARCOMA (OSTEOGENIC SARCOMA)
ā¢ Osteosarcoma is the second most common, and a
highly malignant primary bone tumour.
Pathology:
ā¢ An osteosarcoma can be defined as a malignant
tumour of the mesenchymal cells, characterised by
formation of osteoid or bone by the tumour cells.
35. Clinical features
ā¢ Pain is usually the first symptom, soon
followed by swelling.
ā¢ Patient presents with a pathological fracture.
36.
37. Examination:
ā¢ The swelling is in the region of the metaphysis.
Skin over the swelling is shiny with prominent
veins.
ā¢ The swelling is warm and tender.
ā¢ Margins of the swelling are not well defined.
ā¢ Movement at the adjacent joint may be limited
ā¢ Regional lymph nodes may be enlarged.
38. Radiological examination:
X-ray shows the following features (Fig-28.4):
ā¢ An area of irregular destruction in the
metaphysis, sometimes overshadowed by the
new bone formation.
ā¢ The cortex overlying the lesion is eroded.
ā¢ There is new bone formation in the matrix of
the tumour.
39.
40. Codmanās triangle:
ā¢ A triangular area of subperiosteal new bone is
seen at the tumour-host cortex junction at the
ends of the tumour.
ā¢ Sun-ray appearance: New bone is laid down
along the blood vessels within the tumour
growing centrifugally, giving rise to a āsun-ray
appearanceā on the X-ray.
41. Serum alkaline phosphatase (SAP):
ā¢ It is generally elevated, follow up of a case of
osteosarcoma.
ā¢ A rise of SAP after an initial fall after tumour
removal is taken as an indicator of recurrence or
metastasis.
Biopsy:
ā¢ An open biopsy is performed to confirm
the diagnosis.
42. Treatment
a) Confirmation of the diagnosis
ā¢ Histologically, tumour new bone formation is
pathognomonic of osteosarcoma.
b) Evaluation of spread of tumour.
ā¢ Lung is the earliest site for metastasis.
43. ā¢ To plan amputation surgery: Complete removal
of local tumour
ā¢ To plan a limb saving operation: In cases presenting
early, a radical excision of the tumour is being
performed these days (limb saving surgery), thus
avoiding amputation.
Methods used for precise evaluation of spread of the
tumour locally are:
ā¢ Bone scan for finding the intra-medullary spread
(āskipā lesions),
ā¢ CT and MRI scans for finding the soft tissue spread.
44. c) Treatment of the tumour
Local control:
ā¢ This is achieved by surgical ablation.
ā¢ Amputation remains thTableā28.5 gives the
recommended levels of amputation in osteosarcoma
at common sites.
45.
46. Role Of Radiotherapy:
ā¢ Radiotherapy is used for local control of the
disease for tumours occurring at surgically
inaccessible sites, or in patients refusing
surgery.
Control of distant macro or micro-metastasis.
ā¢ These are effectively controlled by adjuvant
chemotherapy, immunotherapy etc.
ā¢ A solitary lung metastasis -excision.
47. Role of Chemotherapy:
ā¢ Chemotherapy has revolutionised the
treatment of osteosarcoma.
ā¢ The drugs used are high dose Methotrexate,
Citrovorum factor, Endoxan, and sometimes
Cisplatinum.
ā¢ These drugs are highly toxic and should be
given in centres where their side effects can
be effectively managed.
48. Role of Immunotherapy:
ā¢ This is a new concept not yet practiced widely.
ā¢ In this technique, a portion of the tumour is
implanted into a sarcoma survivor and is removed
after 14 days.
ā¢ The sensitised lymphocytes from the survivor are
infused into the patient.
ā¢ These cells then selectively kill the cancer cells.
d) Follow up: The patient is checked up every 6-8
weeks. Flow chart-28.1.
49.
50. Prognosis:
ā¢ Without treatment, death occurs within 2 years,
usually within 6 months of detection ofmetastasis.
ā¢ 5-year survival with surgery alone is 20 per cent.
ā¢ With surgery and adjuvant chemotherapy a 5-year
disease free period is reported to be as high as 70
per cent.
ā¢ A primarily lytic type (telangiectatic) osteosarcoma
has the worst prognosis.
51. SECONDARY OSTEOSARCOMA
ā¢ This is an osteosarcoma developing in a bone
affected by a pre-malignant disease.
ā¢ Treatment is along the lines of the
conventional osteosarcoma.
52. PAROSTEAL OSTEOSARCOMA
ā¢ This is a type of osteosarcoma, arising in the region
of the periosteum.
ā¢ It is a slower growing tumour, seen in adults.
ā¢ The common site is lower-end of the femur.
ā¢ Treatment is on the lines of osteosarcoma.
ā¢ Prognosis is better.
53. EWINGāS SARCOMA
ā¢ This is highly malignant tumour occurring between
the age of 10-20 years, sometimes up to 30 years.
Bones affected:
ā¢ It commonly occurs in long bones (in two-third
cases), mainly in the femur and tibia.
ā¢ About one-third of cases occur in flat bones, usually
in the pelvis and calcaneum.
54. Site: The tumour may begin anywhere, but
diaphysis of the long bone is the most common
site.
Clinical features:
ā¢ The tumour occurs between 10-20 years of age.
ā¢ The patient presents with painand swelling.
ā¢ There may be a history of trauma
ā¢ Fever
55. Examination:
Swelling is usually located in the diaphysis and
has features suggesting a malignant swelling.
Radiological features:
ā¢ In a typical case, there is a lytic lesion in the
medullary zone of the midshaft of a long
bone, with cortical destruction and new bone
formation in layers ā onion-peel appearance
(Fig- 28.5).
56.
57. Differential diagnosis:
ā¢ Ewingās sarcoma can be differentiated from other
bone tumours.
ā¢ From chronic osteomyelitis, it can be differentiated
by the following features in the former:
ā¢ Sequestrum
ā¢ Well-defined cloacae and a rather smooth periosteal
reaction located at metaphysis.
58.
59. Treatment:
ā¢ This is a highly radio-sensitive tumour,melts
quickly but recurs.
ā¢ Treatment consists of control of local tumour by
radiotherapy (6000 rads), and control of
metastasis by chemotherapy.
ā¢ Chemotherapy consists of Vincristine,
Cyclophosphamide, and Adriamycin in cycles,
repeated every 3-4 weeks for about 12- 18 cycles.
.
60. Prognosis:
ā¢ It is very poor.
ā¢ Bone to bone secondaries are very common.
ā¢ 5-year survival (which was only 10%), has
now improved to 30-40%
61. MULTIPLE MYELOMA
ā¢ It is a malignant neoplasm derived from plasma cells.
Clinical features:
ā¢ 40 years of age.
ā¢ Men >women.
ā¢ Common presenting complaint is increasingly severe
pain in the lumbar and thoracic spine.
ā¢ Pathological fractures, especially of the vertebrae
and ribs may result in acute symptoms.
62. ā¢ Neurological symptoms may result if the tumour
presses on the spinal cord or the nerves in the spinal
canal.
ā¢ There is local tenderness over the affected bones.
Radiological examination
ā¢ Characteristic radiological features are as follows (Fig-
28.6):
ā¢ Multiple punched out lesions in the skull and other flat
bones.
ā¢ Pathological wedge collapse of the vertebra, usually
more than one, commonly in the thoracic spine.
63.
64. Other investigations
ā¢ Blood: Low haemoglobin, high ESR (usually very
high), increased total protein, A/G ratio reversed,
increased serum calcium, normal alkaline
phosphatase.
ā¢ Urine: Bence Jones proteins are found in 30 per
cent of cases.
ā¢ Serum electrophoresis: Abnormal spike in the
region of gamma globulin (myeloma spike is
present in 90 per cent of cases.
ā¢ Sternal puncture: Myeloma cells may be seen.
65. ā¢ Bone biopsy from the iliac crest, or a CT guided
needle biopsy from the vertebral lesion may
show features suggestive of multiple
myeloma.
ā¢ Bone scan:
ā¢ Open biopsy
66. TREATMENT
It consists of control of the tumour by
chemotherapy and splintage to the diseased
part by PoP, brace etc.
ā¢ Radiotherapy plays a useful role in cases with
neurological compression, localised painful
lesions, fractures and soft tissue masses.
ā¢ Complications like pathological fractures must
be prevented by splinting the affected part.
67. Chemotherapy:
ā¢ Melphalan is the drug of choice.
ā¢ It is given in combination with Vincristine,
Prednisolone, and Cyclophosphamide.
ā¢ The cycles are repeated every 3-4 weeks for 6-
12 cycles.
68. SOME UNCOMMON MALIGNANT
TUMOURS
CHONDROSARCOMA
ā¢ This is a malignant bone tumour arising from
cartilage cells.
ā¢ It may arise in any bone but is common in flat bones
such as scapula, pelvis and ribs.
69. Diagnosis
ā¢ It occurs commonly in adults between 30-60 years of
age, and is rare in children.
ā¢ Metastasis occurs through the blood vessels,
commonly to the lungs.
ā¢ Presenting symptoms are pain and swelling
ā¢ X-ray shows erosion of the cortex and bone
destruction.
ā¢ The tumour matrix may have mottled calcification,
typical of a cartilaginous tumour (Fig-28.7).
ā¢ Diagnosis is confirmed by a biopsy.
70.
71. Treatment
ā¢ Depends upon the behaviour of the tumour.
ā¢ Amputation is necessary for most tumours.
ā¢ In some low grade tumours, after proper
assessment, wide resection of the tumour is
done (limb saving surgery).
72. SYNOVIAL SARCOMA
ā¢ This is a malignant tumour, histologically a
combination of synovial cells and fibroblasts.
ā¢ It occurs most commonly around the knee.
ā¢ The tumour spreads via the blood vessels,
lymphatics, and along the soft tissue planes.
ā¢ Treatment is by amputation
ā¢ Prognosis is poor.
73. METASTASIS IN BONE
ā¢ Metastatic tumours in the bone are commoner than
the primary bone tumours.
ā¢ The tumours most commonly metastasising to bone
are carcinoma of the lung in the male and carcinoma of
the breast in the female.
ā¢ Other malignancies metastasising to the bone are
carcinoma of prostrate, carcinoma of thyroid etc.
CLINICAL FEATURES
ā¢ A patient with secondaries in the bone may present in
the following ways:
74. ā¢ a(i) bone pain ā in the spine (commonest site), ribs or
extremities; and
(ii) pathological fracture ā commonly in the spine.
ā¢ b) It may be a patient, not a known case of primary
malignancy, who presents with:
(i) bone pain
(ii) a pathological fracture through an area of bone
weakened by such a lesion.
ā¢ Malignancies which are known to present first time
with secondaries (with silent primary) are carcinoma of
thyroid, renal cell carcinoma, carcinoma of the bladder
etc.
75. INVESTIGATIONS
ā¢ A case of secondaries in the bone can be
investigated as follows:
a) In a case with known primary, a complaint of
bone pain may be due to metastatic lesion of
the bone.
b) On plain X-rays, 20-25 % or more of
metastatic deposits are missed. (Fig-28.8).
c) PET scan is the most recent imaging modality
for early detection of metastasis.
76.
77. ā¢ b) In a case presenting first time with bony
secondaries, a systematic investigation
programme is required to detect the primary.
78. Radiological examination
ā¢ Majority of bone secondaries are osteolytic,
but a few are osteoblastic.
ā¢ Carcinoma of the prostate in males and
carcinoma of the breast in females are the
commonest tumours to give rise to sclerotic
secondaries in the bone.
79. Blood:
ā¢ A high ESR, and an elevated serum calcium are
indications of bony secondaries in a suspected
case.
Other investigations:
ā¢ These depend upon the site suspected on clinical
examination.
ā¢ In a secondary without a known primary, useful
investigations are an abdominal ultrasound, Ba
studies, IVP, thyroid scan etc.
80. Treatment:
ā¢ It consists of symptomatic relief of pain,
prevention of any pathological fracture, and
control of secondaries by chemotherapy or
radiotherapy, depending upon the nature of
the primary tumour.
ā¢ Role of surgery is limited, mostly to the
management of pathological fractures.
81. TUMOUR-LIKE CONDITIONS OF THE BONE
OSTEOCHONDROMA
ā¢ This is the commonest benign ātumourā of the
bone.
ā¢ It is a result of an aberration at the growth
plate, where a few cells from the plate grow
centrifugally as a separate lump of bone.
82.
83. Clinical presentation:
ā¢ The patient, usually aroundadolescence,
presents with a painless swelling around a
joint, usually around the knee.
ā¢ There may be similar swellings in other parts
of the body in case of multiple exostosis .
84. Treatment:
ā¢ When necessary, the tumour should be
excised.
ā¢ The excision includes the periosteum over the
exostosis; since leaving it may result in leaving
a few cartilage cells, which will grow again and
cause recurrence of the swelling.
85. MAFFUCCI SYNDROME
ā¢ This is a hereditary disorder where multiple
enchondromas and cavernous haemangiomas
occur together.
86. SIMPLE BONE CYST
ā¢ This is the only true cyst of the bone, different
from other lesions, which though appear clear
ācyst-likeā on X-ray, are actually osteolytic,
some-times solid lesions.
87.
88. Treatment:
ā¢ The cyst is known to undergo spontaneous
healing, particularly after a fracture.
ā¢ One or two injections of methylprednisolone
into the cyst results in healing.
ā¢ Some cases need curettage and bone
grafting.
89. FIBROUS DYSPLASIA
ā¢ This is a disorder in which the normal bone is
replaced by fibrous tissue ā hence its name.
ā¢ The mass of fibrous tissue thus formed grows
inside the bone and erodes the cortices of the
bone from within.
ā¢ A thin layer of sub-periosteal bone forms
around the mass, so that the bone appears
expanded.