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BONE TUMOURS
DR.SUNIL KUMAR
ASST.PROFESSOR
DEPT.OF GEN.SURGERY
MNR MEDICAL COLLEGE
18-12-2018
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
ā€¢ The term ā€˜bone tumourā€™ is a broad term used
for benign and malignant neoplasms, as well
as ā€˜tumour-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.
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 ( Tableā€“28.1).
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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 ā€˜egg-shell cracklingā€™ is often not
elicited..
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 (Tableā€“28.3).
RADIOLOGICAL FEATURES
ā€¢ A solitary, may be loculated, lytic lesion.Eccentric
location, often subchondral.
ā€¢ Expansion of the overlying cortex (expansile lesion).
ā€¢ ā€˜Soap-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.
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.
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):
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.
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€¢ ā€˜bone cementā€™, which by the heat it produces while
setting, ā€˜killsā€™ the residual cells.
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.
PROGNOSIS
ā€¢ Recurrence following treatment is a serious
problem.
ā€¢ With every subsequent recurrence, the
tumour becomes more aggressive.
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.
Clinical features
ā€¢ Pain is usually the first symptom, soon
followed by swelling.
ā€¢ Patient presents with a pathological fracture.
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.
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.
Codmanā€™s 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 ā€˜sun-ray
appearanceā€™ on the X-ray.
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.
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.
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
(ā€˜skipā€™ lesions),
ā€¢ CT and MRI scans for finding the soft tissue spread.
c) Treatment of the tumour
Local control:
ā€¢ This is achieved by surgical ablation.
ā€¢ Amputation remains thTableā€“28.5 gives the
recommended levels of amputation in osteosarcoma
at common sites.
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.
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.
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.
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.
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.
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.
EWINGā€™S 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.
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
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).
Differential diagnosis:
ā€¢ Ewingā€™s 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.
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.
.
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%
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.
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.
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.
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
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.
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.
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.
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.
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).
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.
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:
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.
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.
ā€¢ b) In a case presenting first time with bony
secondaries, a systematic investigation
programme is required to detect the primary.
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.
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.
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.
TUMOUR-LIKE CONDITIONS OF THE BONE
OSTEOCHONDROMA
ā€¢ This is the commonest benign ā€œtumourā€ 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.
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 .
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.
MAFFUCCI SYNDROME
ā€¢ This is a hereditary disorder where multiple
enchondromas and cavernous haemangiomas
occur together.
SIMPLE BONE CYST
ā€¢ This is the only true cyst of the bone, different
from other lesions, which though appear clear
ā€˜cyst-likeā€™ on X-ray, are actually osteolytic,
some-times solid lesions.
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.
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.
ā€¢ Treatment is curettage and bone grafting.
THANK YOU

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Tumours of the bones &joints

  • 1. BONE TUMOURS DR.SUNIL KUMAR ASST.PROFESSOR DEPT.OF GEN.SURGERY MNR MEDICAL COLLEGE 18-12-2018
  • 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 ā€˜bone tumourā€™ is a broad term used for benign and malignant neoplasms, as well as ā€˜tumour-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 ( Tableā€“28.1).
  • 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 ā€˜egg-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 (Tableā€“28.3). 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€¢ ā€˜Soap-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€¢ ā€˜bone cementā€™, which by the heat it produces while setting, ā€˜killsā€™ 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. Codmanā€™s 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 ā€˜sun-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 (ā€˜skipā€™ 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 thTableā€“28.5 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. EWINGā€™S 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€¢ Ewingā€™s 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 ā€œtumourā€ 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 ā€˜cyst-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.
  • 90.
  • 91. ā€¢ Treatment is curettage and bone grafting.
  • 92.
  • 93.
  • 94.
  • 95.