BONE TUMORS

 Benign tumors  Secondary  Tumor-like conditions of bone

 Malignant tumors
Bone is a mesenchymal
tissue. Thus tumors of bone
may arise from different
tissue components-osseous,
e.g bone, cartilage,
periosteum and nonosseous,
e.g fat,fibrous, tissue, nerve
tissue, vasculaar tissue, etc.
Indigenous to the bone.
carcinoma of
bone
Tumors of bone are BENIGN TUMORS
commonly benign and Osteoma
metastatic deposits in - this is a benign tumor composed of
bone are commoner than sclerotic, well-formed bone protruding
primary bone tumors. Of from the cortical surface of a bone.
the primary bone
malignancies, multi[ple
myeloma is the
commonest.
Most primary malignant
bone tumors occur in
child and young adults.

Classification of bone tumors

Benign Malignant
1. Bone forming tumor - osteoma ( from Osteosarcoma
osteoblast)
- osteoblastoma
2. Cartilage forming tumors  Chondroma Chondrosarcoma
 Chondroblastoma
(from cartilage
cells)
 osteochondroma
3. Giant cell tumor ( GCT) - Benign GCT Malignant gct
FROM OSTEOCLASTS
4. Marrow Tumors  Ewing’s tumor (from
reticuloendothelial cells of marrow)
 Multiple myeloma (from plasma
cells)
 Malignant lymphoma(NHL-Non
Hodgkin lymphoma)
5. Vasular tumors Hemangioma Angiosarcoma
6. Others ( other connective - lipoma - liposarcoma
tissue and nerve tissue - fibroma - fibrosarcoma
tumors) - neurilemmoma( from - malignant fibrous histiocytoma
nerve sheath) - undifferentiated sarcoma
- neurofibrosarcoma
7. Tumor-like lesions - bone cysts-simple or
aneurysmal
- fibrous dysplasia
- reparative giant cell
granuloma(e.g. epulis
Types
 1. Ivory osteoma - arises Chondroma
from the membrane bone of This tumor arises from
skull. precartilaginous cells of the bone,
2. Osteoid osteoma- it can which fails to become ossified. It
arise in any bone except the usually involves the short long
skull bone, the commonest bones viz. Metacarpals, metatarsals
bones affected are the femur and proximal phalanges of hands
and tibia. and feet.
X-ray will confirm the Pathology
diagnosis. X-ray shows a It is usually solitary.
small round or oral
radiolucent area with Types
sclerosis at the margin. They are two types:-
These tumors do not a. enchondroma-growing within
undergo malignant the bone
transformation. b. Ecchondroma-fracture is
Treatment common due to thinning of the
Excision of the osteoma cortex.Occasionally chondroma
malignant change becoming a
chondrosarcoma
Clinical features
- the tumor usually presents with
painless expanded swelling of the
affected bone.
- an ecchondroma may interface
with joint and tendon movement.
- the patient may present with a
fracture after a trivial injury.
- X-ray shows a rarefied area on the
cortex with a clear outline.
Treatment
Excision of the tumor
MALIGNANT TUMORS
OSTEOSARCOMA Primary osteosarcoma
Excluding multiple There are no known premalignant
myelomas these are the most conditions related to it. It is
Osteochondroma
( syn-exostosis)
This tumor consists of normal
bone covered by a cap of
cartilage. It is one of the most
common benign tumors.
It is truly a hamartoma,
arising from the growth plate
of the developing bone. As
the bone grows in length, the
tumor gets left behind and
thus appears to migrate along
the shaft towards its center.
It grows outwards from the
bone like a mushroom. The
lesion stops growing when
the growth of the skeleton
ceases.
Clinical features
- Age- usually present in
teenage and adult life.
The most common site of
osteochondroma is the
growing ends of bone
includeing the diastal femur,
proximal tibia and proximal
humerus.
Examination
The tumor is bony hard in
consistency and fixed to the
bone but not the muscle or
skin. X-ray shows mushroom
like bony tumor but not the
cartilaginous cap.
Treatment
Excision of the tumor if it
causes pain or pressure
symptoms on the adjacent
structures, but one should
wait untill the cessation of
skeletal growth, I.e after
epiphyseal union.
 common primary malignant commoner and occurs in the age
tumors of bone, derived from group of 10-20 years. It is much
the pluripotent mesenchymal more malignant than the secondary
cell with bony propensity. one.

Secondary osteosarcoma
Classification This occurs in the older age On examination
Osteosarcoma has been group(45 years onwards). it arises The swelling is usually
subclassified as follows. from preexisting lesion or in bone located in the region of
that has been irradiated. metaphysis, firm to soft in
1. Depending on the feel and highly tender.
Pre-existing lesions are paget’s
presence of preexisting Local temperature is raised
disease, multiple
lesion- (i) primary due to high vascularity and
enchondromatosis, fibrous
osteosarcoma- no pre- the skin over the swelling is
dyspiasia, irradiation, multiple
existing lesion present. red, tense and glossy with
osteochondroma, etc.
(ii)secondary osteosarcoma - prominent veins on it.
developing in presence of a Spread
pre-exsting lesiom. All osteosarcomams are aggressive
2. depending on the dominant lesion and metastasize widely
histomorphology the through the bloodstream, usually to
following are the subtypes.(a) the lungs. Lymph node involvement
Osteoblastic osteosarcoma- is unusual. Osteoblastic type.
with alot of new bone
formation. Clinical feature
(b) osteolytic type or - pain is usually the first symptom.
telangiectatic osteosarcoma- Soon followed by swelling. The
which is predominantly a pain is constant boring and
lytic tumor. Hence becomes worse, as the swelling
pathological fracture is increases in size
common. - the bones commonly involved in
© fibroblastic osteosarcoma, order of frequency are distal femur.
the basic cell being the Proximal tibia, proximal humerus,
fibroblast. pelvis and fibula. Over 70percent of Origin of osteosarcoma from
(d) chondroblastic all osteosarcoma as occur in the the metaphysis of a long
osteosarcoma, the basic cell lower limbs bone. It is to be noted that
being a cartilage cell. It is osteoclastoma is epiphyscal
common in the pelvis. -
and Ewing’s sarcoma is
there
displayed in origin
may
be a

history of trauma but more often it

is incidental and draws attention of
the patient to the swelling.

X-ray
- local X-ray shows the
following features ( fig.61.4)
a. The growth is at the
metaphysis.
b. Usually but not always
there is evidence of new bone
formation. Tumor bone is
laid below the periosteum
especially along the stretched
blood vessels and at the
junction of bone and lifted
periosteum. These are
prominent in X-ray plates as
sunray spicules and codman’s
trioangle respectively.
c. There is a big soft tissue
shadow.
- X-ray chest- metastasis may
be present in the form of
multiple deposits or as a
solitary cannon ball deposit.
CT SCAN AND MRI
these are important
investigation modalities to
know the extent of tumor
spread within the medullary
cavity. The soft tissue
involvment is best delineated
with the MRI.
BIOPSY
Either a core biopsy or a
biopsy is done to confirm the
dianosis. Sometimes, the fine
needle aspiration cytology
( FNAC), a relatively quicker
and easier method may
establish the diagnosis.
TREATMENT
The aim of treatment is to
Sometimes the patients presents
with a pathological fracture.
EWING’S SARCOMA Osteoclastoma(syn-Giant
It is one of the most lethal primary
cell Tumor, Gct)
malignant bone tumors in the These neoplasms probably
pediatric age group.
arise from the mesenchmal
The tumor usually involes the stromal cell and may be
diaphysis of long bones as well as
benign , locally malignant or
flat bones such as scapula and malignant. Malignancy may
pelvis.
be a transformation of benign
It arises from the stromal cells of tumor or it may arise de novo.
the bone marrow be round cells and
this tumor is called
the spindle cells ( fig. 61.5) osteoclasoma becouse of he
presence of multinucleate
Pathology giant cells in the tumor which
- femur is the most common bone resemble osteoclasts.
involved followed by tibia, fibula,
humerus, pelvis, and scapula. Pathology
- the tumor is soft and may Giant cell tumor is a
resemble brain tissue cut surface is neoplasm found maily in the
grayish white. epiphysis of long bones most
commonly at the lower end of
femur. Other flat bones like
ribs, scapula, mandible, etc.
And fibula, lower end of
radious may also be involved.
Grossly, the lesion is soft
gray to red and hemorrhagic
in appearance and produces
thinning and expansion of the
cortical bone. These are bony
trabeculae, passing through
Histologic features are;- the soft tumor mass. On the
i. Undifferentiated round cells in X-ray these give a ‘soap
sheets bubble appearance’ which
ii. There are hyperchromatic cells though diagnostic of
with scanty cytoplasm. So this osteoclastoma is not
tumor is called round cell tumor. necessarily a constant feature.
There is alternate deposition of Microscopiccally two types of
tumors appearance, best cells are found viz.
appreciated in X-ray. a. Spindle cells- these are
- spread- hematogenous spread to basic mononuclear stromal
lungs and other bones is very quick cells, the exact nature of
and more common than in which is still unknown.
osteosarcoma. b. Giant cells of foreign body
confirm the diagnosis from - prognosis is worse- if untreated, type- these are either products
the clinical features, X-ray death is usually within 2 years. of basic mononuclear cells, or
findings and biopsy, to derived from osteoclasts or
evaluate the spread of tumor Clinical features modified megakaryocytes.
from CT scan and MRI of the Age - 5-15 years.
affected bone and chest X- Sex - it is more common in males. Clinical features
ray and to execute adequate This is pain and swelling in relation Age- 20 to 40 years.
treatment. to the affected bone, associated Sex-it is more common in
The different modalities of with local heat and tenderness. females. ( fig 61.1)
treatment include:- This may be accompanied by fever - swelling is usually located at
1. Surgery and malaise so that osteomyelitis is the end of long bone
2. Chemotherapy suspected. Moreover, incision on gradually increasing in size
3. Radiotherapy the swelling , based on this and duration may be more
diagnosis often brings out semisolid than a year.
gray material looking like pus and
a. SURGERY this further confuses the diagnosis.
A limb ablation or a limb Thus, as for all bone tumors, the
salvage surgery may be done importance of jointly considering
depending on the spread of the clinical , radiological and
the tumor. pathological evidence cannot be
1. Early presentation of overemphasized.
tumor: when the tumor is
diagnosed in the early stage Investigations
neoadjuvant chemotherapy is 1. Local X-ray shows:
given to down size the tumor a. Onion peels appearance due to
and its vasculariry. Limb alternate layers of reactive new
salvage surgery is performed bone formation and the tumor
subsequently. The bone tissue.
defect of the excised tumor is b. Extensive destruction of the bone - pain at the site of lesion is
filled with bone grafts. as shown by widening of the not unbearable and is much
Custom made prosthesis or medulla as well as gross rarefaction less than that of
intramedullary nail can also of the cortex. osteosarcoma.
be used depending on the 2. Tissue diagnosis is obtained by
situation. needle or open biopsy. On examination
2. Locally advanced tumor: 3. MRI scanning is very useful to 1. Surface is smooth, skin
in patients with locally determine the extent of temperature not raised and
advanced disease, amputation intramedullary and soft tissue usually there is ni venous
has to be performed with involvement and aids in surgical prominence over the swelling.
complete removal of the planning. 2. Tenderness-mild
tumor. Pain relief is also 4. X-ray chest and CT scan of 3. Pathological fracture of the
obtained with amputation and abdomen and chest are additional affected bone is common as
is an important indication for imaging studies to determine the cortex gets thinned out.
palliative amputation metastasis. 4. Joint involvement is mor as
the tumor arises from the
b. CHEMOTHERAPY Treatment epiphysis.
Preoperative neodjuvant There are three modalities of Features of the malignant
chemotherapy decreases the treatment: variety are akin to those of
size of the tumor and also a. Radiotherapy osteosarcoma.
ablates the micrometastases b. Chemotherapy
that have already occurred.it c. Surgery Investigations
has made possible the
concept of limb salvage Ewing’s sarcoma is a highly
surgery. radiosensitive tumor. In most
Drugs that yield best cases, distant metastasis has
response include occurred by the time diagnosis is
methotrexate, endoxan and made.
cisplatin. Thus treatment consists of:-
i. Control of the local tumor by
c. RADIOTHERAPY radiotherapy (6000rads) and control
This may be indicated in of the metastasis by chemotherapy.
cases where the tumor is ii. The chemotherapy consists of
surgically inaccessible or VAC or vincristine , adramycin and
patient refuses surgery. cyclophosphamide in cycles
repeated every 2 to 4 weeks for
about 12 to 18 cycles.
After radiotherapy, the tumor is
excised and the gap is filled with
bone and the segments are
supported with plate and screws or
nail.
Amputation is done in selected
cases only.
1. Local - X-rays shows the
following features:(fig.61.70
i. It occures at the end of a
long bone and is usually
eccentric in situation.
ii. The long axis of the tumor
is long the transverse axis of
the bone.
iii. There is destruction of the
bone substance so that the
cortex is expanded and
thinned out over the tumor.
iv. There is often a ‘soap
bubble appearance’ due to the
presence of trabeculae of the
remnants of bone transversing
the tumor.
2. Biopsy- this must be done
in all cases to confirm the
dianosis. Fine needle
aspiration cytology (FNAC)
may show the multinucleated
giant cells.
3. X-ray chest - to detect any
metastasis.

Treatment Clinical features

- treatment is essentially
surgical. The ideal surgical
treatment is total excision of
the tumor which is readily
applicable to dispensable
bones like the fibula or ribs.
- for lesions at juxtaarticular
sits,e.g. knee joint, the choice
-age - 50years or more.
-sex- it is more common in men
than in women.
- the common presentation is
increasingly severe pain in the
lumbar and thoracic spine.
-there may be general weakness,
anemia and infection.
rests between through
curettage and filling of the
cavity with bone chips or the
more major procedure of
prosthetic replacement.
- amputation is occasionally
required for a frankly
malignant GCT or a recurrent
GCT of the limbs.
- radiotherapy has been tried
for lesions either nonoperable
or at inaccessible sites such
as spinal GCT.
MULTIPLE MYELOMA
multiple myeloma is the most
common primary maliganant
neoplasm of bone in the older
age group > 50 years.
The tumor arises from the
plasma cells present in the
bone marrow. Hence it is also
known as plasmacytoma
when it occurs as a solitary
lesion, it is known as
solitary plasma cytoma,
and when multiple, it is
known as multiple
myeloma(fig. 61.8).
Pathology
Grossly, the tumor is soft,
gray and friable.
The bone is simply replaced
by tumor and there is no
reactive new bone formation.
Microscopically, there is dull
monotony of plasma cells
and intercellular matrix is
little or nil.
Spread
There is hematogenous
spread to lungs, liver, spleen
and other bones.
Pathologic physiology
- there is hyperpoteninemia,
with an increase in the
globular fraction, kown as M-
protein or Bence jines protein
- Occasionally patological fracture
occurs and presents with a
deformity.
Renal failure.
Investigation
1. Local X-ray - multiple punched
out areas of destruction in the skull
and other flat bones(fig.61.8)
2. Other tests to support the
diagnosis of multiple myeloma
are:-
a. Urine- bence jones proteins are
found in 30percent cases.
b. Blood- low hemoglobin with
very high ESR,increased total
protein and Albumin/Globin ratio is
reversed. Increased serum calcium
and serum alkaline phosphatase is
characteristically normal.
c. Open biopsy- and open biopsy
from the leison may sometimes be
required to confirm the diagnosis.
Treatment
- the main modality of treatment is
chemotherapy and the drug of
choice is melphalan. Other drugs
used are vincristine,prednisolone
and cyclophosphamide. The
treatment cycles are repeated every
4weeks for 6 to 12 cycles.
- radiotherapy in addition to
chemotherapy is given to relieve
pain.
-complication like pathological
fracture is prevented by splinting
the affected part. In case of
pathological fracture, surgical
fixation may be advised.
-antibiotics are given to control
infection.
-blood transfusion and hematinics
are given to correct anemia.
in the serum or urine. In
60percent cases, the M-
protein is 1gG type.
- abnormal proteins are
excreted through the kidney
and may cause renal failure
due to tubular block.
- bone is decalcified, so that
there is rise of serum Ca++and
fall in serum phosphate.
- due to bone marrow
depression, there is anemia
and intercurrent infection.
-there may be collapse of the
vertebral bodies to cause
neurological
manifestations,e.g.
paraplegia.

Sites Of Affection TUMOR-LIKE

SECONDARY 1. Bones rich in red marrow are CONDITIONS OF BONE
CARCINOMA OF commonly affected, e.g. vertebrae,
BONE skull, pelvis, sternum, ribs, upper SIMPLE BONE CYST
Secondary metastasis account end of femur and humerus. it occurs in children and
for the majoriry of malignant 2. Unusual sites are below elbow adolescents. The ends of the
bone tumors and far more and knees. If there is such lesion, long bones are the favourable
common than primary possibility of multiple myeloma sites, the commonest site
malignant tumors of bone. should be excluded. beign the upper end of the
The sources are from primary humerus.
malignant tumors with Clinical features The cyst itself may not
affinity to metastasize to - pain, swelling and often a produce symptoms and the
bone, eg. Carcinoma of pathological fracture are the usual patint often presents with a
breast, prostate, lung, kidney presenting features. pathological fracture through
and thyroid. - a vertebral metastasis may present the cyst.
with back pain, compression X-ray shows a well-defined
In some cases no primary site fracture, root pain or paraplegia. radiolucent zone in the
can be found at the time metaphysis or diaphysis plate
when the secondary lesion is considere ‘active as against
presents.
Investigations the one away from it e.g. in
1. Local X-ray. the diaphysis.
2. Biopsy in doubtful cases.
Types of bone lesion 3. Bone scan with radioactive Treatment
The majority is osteolytic isotope. It is the optimal it is treated with currettage
but a few, mostly arising investigation for bone pain and and bone grafting in selected
from the prostate cases.
detection of early lesions. Asymptomatic bone cysts
stimulate new bone 4. All investigations are done to need no treatment.
formation and are then detect the primary tumor. The
called osteosclerotic. breast, prostate, kidney, Aneurysmall bone cyst.
Routes of spread bronchus and thyroid should be It is an expansile lytic
especially investigated. lesion usually occurring
 Most commonly
metastasis occurs through
before the age of 20 years.
hematogenous spread. Treatment It consists of a blood
 There is a direct a. Curative: this is out of question filled space enclosed, in a
communication between excepting when the primary growth shell, ballooning up the
the pelvic venous plexus is suitable for radical surgery and overlying cortex - hence
and the vertebral veins. there is a solitary bone metastasis, its name.
So carcinoma from pelvic e.g. hypernephroma, thyroid A gradually increasing
organs may directly reach carcinoma, etc.
swelling is the usual
the pelvic bones and b. Palliative presentation. There may
vertebrae.
 Tumors of the oral cavity
1. Drugs be mild pain often it
may involve the jaw a. Analgesics- are given for relief presents with a
bones and those of the of pain. Habit forming drugs are pathological fractures.
rectum may involve the avoided as far as possible. X-ray shows the
sacrum by direct b. Chemotherapy- combination following features.
contiguity. chemotherapy is preffered. 1. eccentric well-defined
 In thyroid cancer, Hypercalcemia of malignancy
whatever is the requirs rehydration and IV bi
radiolucent area at the
histological type of the phosphonate therapy. ends of long bones and
primary tumor, the c. Endocrine treatment, e.g. in case dorsolumbar spines.
metastatic lesion is of orostate cancer. 2. Expansion of the
always of the follicular
variety. 2. Radiation: it is one of the best overlying cortex.
ways of palliation. External beam 3. Trabeculation within
radiation is the treatment of choice the substance of the
for localized bone pain. tumor.
3. Surgery: the role of surgery is Treatment is by currettage
limited. A fungating growth from a and bone grafting. In
bone may require amputation. For some cases surgical
pathological fractures, internal intervation is needed for
fixation provides good results. the treatment of
pathological fracture.
FIBROUS DYSPLASIA
This is a disorder in which
normal bone is replaced by
fibrous tissue-hence the
name.
The mass of fibrous tissue
thus formed grows inside the
bone and erodes the cortices
of the bone from within
TYPES
1. MONOSTOTIC- only
single bone involvement is
see. This form affects the
femur,tibia,ribs or the
craniofacial bones. Children
in 5 to 15 years of age are
commonly affected. Pain,
deformity or fractures are the
usual presenting features.
2. POLYSTOTIC- multiple bone Craniofacial bones are
involvement is seen in this variety. almost always involved in
This type of presentation is seen this form.
with with precocious
puberty(Albright’s syndrome) and
X-ray will show sharply
other endocrine disorders such as defined, centrally placed
acromegaly, Thyrotoxicosis or lylic areas with
cushing’s syndrome. homogenous ground glass
appearance. Diagnosis is
confirmed by biopsy.
Treatment
The fibrous defect is
thoroughly curetted out and
the gap is filled with bone
grafts.