67

CS Yadav, HL Nag, KS Malhotra, S Rastogi

Tumors of Bones and Joints

BONE TUMOR However, as the needle biopsy yields only a small
sample of tissues, it is not as reliable as open biopsy.
A bone tumor may be defined as a new growth arising
in the bone or from cells, which are the components
of skeletal tissue. Bone tumors may be classified into APPROACH TO A PATIENT
benign tumors, malignant tumors, and tumor like WITH BONE TUMOR
lesions, or may be classified on the basis of the tissue
In general, while making a diagnosis of a bone tumor,
of their origin (Table 67.1).
certain clinical and radiological criteria should be
Bone tumors generally present with pain, as a
lump, tender area or a pathological fracture. The noted:
diagnosis of a bony tumor depends upon the combi- 1. Age of the patient: Certain tumors have predilection
nation of clinical features, the X-ray appearance and for specific age groups, e.g. Ewing’s sarcoma and
the histopathological findings. Before treatment, a osteosarcoma occur in adolescent age group
biopsy is essential, and an open biopsy is preferable. whereas Giant cell tumors (GCT) are found only
Needle biopsy can be used in tumors of the spine. after closure of the growth plate.

Table 67.1: Classification of bone tumors

Tissue of origin Benign lesion Malignant lesion

Bone forming Osteoma Osteosarcoma

(Osteogenic) Osteoid osteoma
Osteoblastoma
Cartilage forming Enchondroma Chondrosarcoma
(Chondrogenic) Osteochrondroma
Chondroblastoma
Chondromyxoidfibroma
Fibrous,osteofibrous Fibrous dysplasia Fibrosarcoma
(Fibrogenic) Nonossifying fibroma Malignant fibrous
Fibrous histiocytoma
Vascular Hemangioma Angiosarcoma
Glomus tumor Hemangioendothelioma
Cystic angiomatosis Hemangiopericytoma
Reticuloendothelial Giant cell tumor Malignant giant cell tumor
Langerhans' cell histiocytosis Hodgkin’s lymphoma
Lymphangioma Leukemia
Myeloma
Neural Neurofibroma Malignant schwannoma
Neurilemmoma Neuroblastoma
Ewing's sarcoma
Notochordal Chordoma
Fat Lipoma Liposarcoma
Unknown Simple bone cyst Adamantinoma
Aneurysmal bone cyst
1214 Essentials of Surgery

Table 67.2: Bone tumors and their predilection sites Table 67.3: Bone tumors and their
common sites of occurrence
Epiphysis
• Giant cell tumors Bone tumors Common sites
• Chondroblastoma • Giant cell tumor, Around knee joint
Metaphysis osteosarcoma,
• Osteosacroma • Parosteal osteosarcoma Posterior cortex of distal
• Osteochondroma femur
• Chondrosarcoma • Periosteal osteosarcoma Distal tibia
• Osteoblastoma • Chordoma Sacrum
• Osteoid osteoma • Adamantinoma Tibia, fibula
• Bone cysts • Enchondroma Short tubular bones of
• Enchondroma hand and feet
Diaphysis • Chondroblastoma Proximal tibial epiphysis
• Ewing’s sarcoma • Chondromyxoid fibroma Tibial metaphysis
• Fibrous dysplasia • Simple bone cyst Proximal humerus and
• Osteoblastoma femur
• Osteoid osteoma • Osteoid osteoma Femur ,tibia
• Enchondroma • Osteomas Skull, frontal sinuses
• Chondrosarcoma Pelvis, femur and
humerus

Slow growing benign tumors have a sharply

outlined and sclerotic border with narrow zone of
transition. Aggressive tumors have poorly defined
border with no sclerosis, and a wide zone of
transition. Giant cell tumor though a relatively
benign tumor, lacks sclerotic border.
4. Matrix of the tumor: Matrix of the tumors should be
noted on X-rays to differentiate between the two
common varieties of tumors, i.e. osteoblastic and
chondroblastic tumors. Osteoblastic matrix mani-
fest as cotton-like densities within the medullary
cavity as seen in osteosarcoma. Chondroblastic
matrix manifest as punctate, annular and popcorn
like calcifications with lobulated appearance as
Fig. 67.1: Location of tumor seen in chondrosarcoma.
5. Type of bone destruction: Geographic destruction is
2. Site of the tumor: Certain bone tumors have suggestive of a benign tumor, e.g. chondromyxoid
predilection for epiphysis, metaphysis or diaphysis fibroma whereas moth-eaten or permeative type of
(Fig. 67.1 and Table 67.2 ). destructions are indicative of aggressive tumor,
Certain tumors also have predilection for e.g. Ewing’s sarcoma.
particular bones (Table 67.3). 6. Periosteal response: The periosteal response to a
It should also be noted whether tumor is central tumor is usually categorized as interrupted or
or eccentric in position within the bone. Simple uninterrupted. Uninterrupted periosteal response
bone cyst, enchondromas and fibrous dysplasia is indicative of benignity and typically occurs in
are typically central whereas giant cell tumor, osteoid osteoma, osteoblastoma, aneurysmal bone
aneurysmal bone cyst, and chondromyxoid cysts, and chondromyxoid fibroma. Interrupted
fibroma are typically eccentric. periosteal reaction occurs in malignant tumors
3. Borders of the tumor: Evaluation of borders of a forming sunburst appearance and Codman
lesion is crucial in deciding whether it is a benign triangle in osteosarcoma and lamellated onion skin
or malignant tumor. appearance in Ewing’s sarcoma.
 Tumors of Bones and Joints 1215

Benign and Malignant Bone Tumors Table 67.4: Various bone tumors and their
corresponding incidence of peak age group
A bony lesion most likely represents a benign tumor
when it exhibits the following criteria: Types of bone tumors Peak age group
(in years)
a. Geographic bone destruction
b. Sclerotic margin • Simple bone cyst 1-20
c. Uninterrupted periosteal reaction, and • Aneurysmal bone cyst 5-20
d. Slow growth • Fibrous cortical defect 2-20
• Nonossifying fibroma 2-20
Whereas, the bony lesions most likely represents a • Chondroblastoma 5-25
malignant tumor when they exhibit the following • Ewing’s sarcoma 5-25
criteria: • Osteosarcoma 10-20
a. Rapid growth • Chondromyxoid fibroma 10-25
b. Permeative or moth-eaten destruction • Osteoblastoma 10-35
• Osteochondroma 10-35
c. Poorly defined margin
• Osteoid osteoma 10-35
d. Interrupted periosteal reaction, and • Enchondroma 15-40
e. Soft tissue mass. • Giant cell tumor 20-40
• Chondrosarcoma 40-60
Primary Bone Tumor versus Primary • Multiple myeloma 50 and above
• Metastasis 50 and above
Soft Tissue Tumor

Sometimes, when bone tumor involves the surround-

ing soft tissues and forms a soft tissue mass, it becomes which are useful in planning the treatment and
difficult to decide whether it is a primary bone tumor prognosis (Table 67.5).
or a primary soft tissue tumor that has invaded the
bone. OSTEOBLASTIC TUMORS
In primary bone tumor there will be:
a. Epicenter within the bone Enostosis (bone island)
b. Cortex bevelled toward soft tissue
Enostosis is commonly encountered in areas of
c. Periosteal reaction present, and
compact bone (Fig. 67.2). It may be single or multiple
d. Significant bone destruction and small soft
tissue mass. and is usually aligned in the long axis of the bone. It
Periosteal reaction is absent in primary soft tissue may be confused with osteoblastic metastasis. A bone
tumor and there is usually a small bone lesion with a scan is required to differentiate between the two.
large soft tissue mass. Ewing’s sarcoma is an exception Enostosis does not show increased tracer uptake
to this. It can present as a small bony destruction with whereas metastasis will have increased tracer uptake.
a large soft tissue mass.

Bone Tumors and Age Distribution

In a clinical setting, the knowledge of approximate

age distribution of bone tumors is essential to narrow
the differential diagnosis. The followings are the
age distributions for the various bone tumors (Table
67.4).

Staging of Bone Tumors

Both the benign and malignant tumors of bone have

been staged by Enneking in the following manners Fig. 67.2: X-ray left hip, showing endostosis (arrow)
1216 Essentials of Surgery

Table 67.5: Staging of bone tumors (Enneking) Pathology

Tumor stage Grade (G) Site (T) Metastasis (M) The tumor consists of a well-vascularized nidus made
Benign tumor up of osteoid trabeculae with fibrovascular
Stage 1 G0 T0 M0 intertrabecular tissue. The nidus may be radiolucent
Stage 2 G0 T0 M0 or lucent with dense center due to calcific deposits.
Stage 3 G0 T1-2 M0-1
Malignant tumor Clinical Features
Stage IA G1 T1 M0
Stage IB G1 T2 M0 Males and females are equally involved and the age
Stage IIA G2 T1 M0 range is from 10 to 30 years. The tumor commonly
Stage IIB G2 T2 M0
involves meta and diaphyses of proximal femur and
Stage IIIA G1-2 T1 M1
Stage IIIB G1-2 T2 M1 tibia (more than 50%) (Figs 67.3A and B). The other
sites involved are humerus, talus, and spine. Juxta-
G0—benign articular osteoid osteomas are rare clinical presenta-
G1—low grade malignant
G2—high grade malignant tions which are often difficult to diagnose clinically
T0—intracapsular (Figs 67.4A to C). It presents with pain that is more
T1—extracapsular intracompartmental severe at night and is dramatically relieved by
T2—extracapsular extracompartmental salicylates.
M0—no metastasis
M1—metastasis present
Radiology
The lesion appears as a well defined lytic lesion
Radiology surrounded by a zone of reactive sclerosis. A solid
periosteal reaction is present and is usually less than
X-rays reveal a characteristic brush border appearance. 1 cm in size unlike an osteoblastoma, where it is
usually more than 2 cm.
Osteoma Bone scan: This will show increased uptake and
Osteoma is a hamartoma containing only osseous double density sign may be seen.
tissues. It usually develops in membranous bones CT scan: This is the investigation of choice to
such as the outer table of calvarium, frontal sinus and demonstrate a nidus and localize it precisely within
mandible. Men and women are equally affected. the tumor mass. This is important for planning the
Osteomas may be associated with intestinal polyps, treatment.
cutaneous masses and sebaceous cysts in Gardner’s
syndrome. This is an autosomal dominant condition.

Clinical Features
They are generally asymptomatic but may cause sinus
obstruction or proptosis.

Radiology
An ivory like dense sclerotic mass of less than 1 cm,
attached to cortex with sharply demarcated margins
is seen.

Osteoid Osteoma
Figs 67.3A and B: X-ray showing osteoidosteoma (arrows)
Osteoid osteoma constitutes 10 percent of all benign arising from middle third of tibia (A) and proximal third of
bone tumors. femur (B)
 Tumors of Bones and Joints 1217

Differential Diagnosis

• Osteoid osteoma (cortical)

a. Osteosarcoma
b. Stress fracture
c. Cortical abscess
• Osteoid osteomas (medullary)
a. Brodie’s abscess
b. Bone island
c. Osteoblastoma

Management

En bloc resection of the nidus. Percutaneous CT-

guided radiofrequency ablation is a promising
A alternative.

Complications of Treatment

a. Recurrence
b. Accelerated bone growth
c. Scoliosis
d. Precocious arthritis if it is close to the joint.

Osteoblastoma

Osteoblastoma accounts for 1 to 2 percent of all

primary lesion bone tumors. It is histologically similar
B to osteoid osteoma but is characterized by a large size
(more than 2 cm). It is commonly seen in the age
group of 10 to 35 years with a male predominance of
2:1. The common sites of involvement are femur,
tibia, and spine (posterior elements).

Clinical Features

Generally, the patient presents with pain, which if

present is not relieved dramatically by salicylates.
Osteoid osteoma tends toward regression whereas
osteoblastoma tends toward progression and even
malignant transformation.
C
Figs 67.4A to C: X-ray Radiology
showing juxta-articular
osteoid osteoma of dis- The lesion is often eccentric or oval with a lytic area
tal end of humerus, with a sclerotic rim. The sclerosis is less as compared
X-ray (A) X-ray (B) CT
scan (C) MRI which
to osteoid osteoma whereas periosteal reaction is more
show the rounded nidus marked.
1218 Essentials of Surgery

Differential Diagnosis common sites of involvement are distal femur, proxi-

mal tibia and proximal humerus in order of frequency.
a. Aneurysmal bone cyst
b. Giant osteoid osteoma
Classification
c. Osteosarcoma
d. Enchondroma Osteosarcoma has been subclassified on the basis of
(A) pre-existing lesion and (B) site of origin.
Management of Osteoblastoma A. Pre-existing lesion
1. Primary osteosarcoma
Wherever possible, the tumor should be excised with
2. Secondary osteosarcoma
the margin of normal bone. Curettage and bone
grafting may be done if excision is not possible. B. Site of origin
Radiotherapy should be reserved for the sites that are 1. Intramedullary osteosarcoma
surgically inaccessible. 2. Surface osteosarcoma
Primary osteosarcoma It occurs with no evidence
Osteosarcoma of pre-existing lesion or prior radiation therapy. More
Osteosarcoma is the most common primary malignant than 95 percent of osteosarcoma involving children
tumor of the bone after myeloma. Almost 80 percent and young adults are primary. Primary tumors are
of osteosarcomas occur between the age group of 12 more aggressive than secondary osteosarcoma.
and 25 years (Fig. 67.5). It is the most common of
Secondary osteosarcoma It arises from pre-existing
malignant bone tumors to affect children and young
lesion or in bone that has been irradiated. These are
adults. Secondary osteosarcomas occur in older persons
usually encountered above the age of 40. Pre-existing
above the age of 40 years. Males are more commonly
lesions are Paget’s disease, multiple enchondro-
affected than females (2:1).
matosis, irradiation, fibrous dysplasia, bone infarct,
Pathology multiple osteochondroma, and non-ossifying fibroma.

Osteosarcoma is a highly malignant bone tumor of Intramedullary osteosarcoma It accounts for 90

mesenchymal origin characterized by spindle shaped percent of all intramedullary tumors. About 90 percent
stromal cells that produce osteoid. The finding of of these tumors extend outside the bone into the
sarcomatous stromal cells directly producing osteoid adjacent soft tissue and so are extraosseous and
defines the tumor as an osteosarcoma. extracompartmental at the time of diagnosis.
Osteosarcoma involves the metaphysis of long
Parosteal osteosarcoma Parosteal osteosarcoma
bones that have greatest growth potential. Thus, the
represents about 5 percent of all osteosarcomas arising
from metaphysis and characteristically is a large
ossified mass with a broader cortical bone. It may be
confused with osteochondroma or myositis ossificans.
It typically arises from posterior cortex of distal femur.
It is periosteal lucent zone consisting of periosteum
and fibrous tissue between the cortex and tumor that
is considered a diagnostic finding. Histologically, cells
demonstrate the features of low grade malignancy.
Treatment is wide excision.
Periosteal osteosarcoma It arises from the surface
of diaphysis most commonly distal tibia or femur. It
is considered to be a variant of parosteal osteosarcoma
Fig. 67.5: Clinical photograph showing osteogenic sarcoma
affecting left proximal humerus with prominent cartilage component.
 Tumors of Bones and Joints 1219

Histologic Subtypes are initially displaced by tumor but later become

encircled by tumor. A common site of joint penetration
Osteosarcomas are further subclassified based on the is at the junction between the articular cartilage and
cellular composition and predominance into the cortical bone.
following subtypes.
1. Osteoblastic osteosarcoma—is the most common Distant metastasis Distant metastasis to lungs, liver
accounting for half (50%) of tumors. Presents with and kidney occur in that order of frequency. Involve-
histological picture of malignant spindle shaped ment of soft tissue, and lymph nodes are less common.
tumor cells producing osteoid. Grossly, it is grayish
white, hard and gives gritty feeling when cut. Clinical features
2. Chondroblastic osteosarcoma—accounts for 25
Pain and swelling are the usual symptoms in young
percent of tumors. It is common in pelvis. It is patients. The pain is deep seated and continuous.
opalescent and bluish gray due to predominant Often, pain precedes the swelling. A pathological
cartilaginous matrix. fracture may be an uncommon presenting feature in
3. Fibroblastic osteosarcoma—accounts for nearly 20 delayed cases. There may be an incidental history of
percent. Spindle cells with foci of osteoid pro- trauma.
duction are seen. Physical examination will reveal that the skin over
4. Telangiectatic osteosarcoma—predominantly a lytic the swelling is shiny and warm. Veins over the tumor
tumor with aneurysmal or bag of blood consis- are distended.
tency containing vascular spaces separated Swelling is tender and margins are not well-
by septae lined with malignant stromal cells. defined. There may be involvement of adjacent
Osteoid production is minimal. Comprises neurovascular structures thereby producing neuro-
5 percent of all tumors and is considered to be vascular deficit. Limitation of range of motion of the
aggressive variant. Pathological fracture is joint is seen due to mechanical block or direct
common (25%). involvement by the tumor.
5. Mixed osteosarcoma—where the picture is of mixed
variety of the above described types. Investigations
6. Small cell variety of osteosarcoma—where the cells
are smaller than the all described varieties. X-rays X-rays of the involved area will reveal
irregular destruction of cortex along with new bone
Mode of Spread formation. The opacity of new bones within soft tissues
appear as of scattered cotton. There may be elevation
The tumor spreads directly at local sites or of periosteum producing characteristic Codman’s
hematogenously to distant sites. triangle and appearance of interrupted periosteal
reaction known as sun-ray spicules (Fig. 67.6). Chest
Local spread The tumor usually arises in the
X-rays should be done to detect the pulmonary
metaphysis and extends up into the epiphysis as well
metastasis.
as diaphysis. The epiphyseal plate is not an effective
barrier to tumor extension. Extension through CT Scan and MRI These are important investigation
subchondral bone and articular cartilage into joint is modalities to know the extent and spread of the tumor.
rare. With time, tumor involves the cortex, elevates The soft tissue involvement is best delineated with an
the periosteum, and extends into adjacent soft tissues. MRI.
Elevation of periosteum with subperiosteal new bone Bone scan This is helpful in detecting the skip
formation results in a triangular area known as lesions and distant metastasis.
Codman’s triangle. When the tumor spreads beyond Biopsy Either a core biopsy or an open biopsy is
the domains of periosteum, bone is laid down along done to confirm the diagnosis. Sometimes fine needle
the blood vessels within the tumor growing centri- aspiration cytology (FNAC), a relatively easier and
fugally presenting as sun-burst spicules (sun-ray quicker method, may establish the diagnosis in
appearance) on radiography. Neurovascular bundles selected cases.
1220 Essentials of Surgery

Figs 67.6A and B: X-rays AP (a) and lateral (B) views

showing osteogenic sarcoma affecting distal half of right
humerus with sun-ray appearance

Treatment

Surgery A limb-ablation surgery or a limb-salvage Figs 67.7A and B: Excised specimen of osteogenic sacroma
surgery may be done depending on the spread of the (A) and split specimen (B) showing necrotic area within the
tumor
tumor (Figs 67.7A and B).
i. Early presentation of tumor: When the tumor is
Chemotherapy Advances in chemotherapy has
diagnosed in the early stage, neoadjuvant
revolutionized the treatment of osteosarcoma.
chemotherapy is given to decrease the size and
Preoperative neoadjuvant chemotherapy decreases
vascularity of the tumor and limb salvage surgery
the size of the tumor and also ablates the micro-
is performed subsequently. The bone-defect of
metastases that have already occurred. It has made
the excised tumor is filled with bone grafts. possible the concept of limb salvage surgery. Drugs
Depending on the need of the situation long that yield best response are methotrexate, endoxan,
intramedullary nail or custom made prosthesis citrovorum rescue factor and cisplatin.
can be used.
ii. Locally advanced tumor: In patients with Radiotherapy This may be indicated in cases in
locally advanced disease, amputation has to be which tumor is not surgically accessible or patient
performed with complete removal of tumor. Pain refuses surgery.
relief is also obtained with amputation and is
an important indication for palliative amputa- CHONDROBLASTIC TUMORS
tion. A safe margin of at least 10 cm from
Osteochondroma
the preserved joint should be obtained while
doing amputation for the future prosthetic Osteochondroma is the most common benign bone
fitting. lesion seen in children and young adults. It constitutes
 Tumors of Bones and Joints 1221

Fig. 67.8: X-ray of knee joint AP (A) and lateral (B) views
showing exostosis, senile variety, arising from proximal fibula

about 20 to 50 percent of all benign bone lesions. The

most common sites of involvement are metaphysis of
distal femur, proximal tibia and proximal humerus.
The growth of tumor stops with cessation of growth Figs 67.9A and B: X-ray of forearm showing osteochond-
at the epiphyseal [Link] the attachment of the roma ulna leading to deformity of forearm
tumor is through a stalk, it is called a pedunculated
exostosis (Fig. 67.8) and when the attachment is usually a metaphysis. Pain may be seen due compres-
broad-based, it is called a sessile exostosis (Fig. 67.9). In sion of a neurovascular bundle, bursitis exostotica,
the long bones, osteochondromas generally point fracture through exostosis or a malignant transfor-
away from the joint. There is clear continuity of mation. A chondrosarcoma is suspected on the basis
marrow and cortex of host bone into osteochondroma of a rapid increase in the size of swelling and
that establish the diagnosis. Osteochondroma is lined occurrence of pain in a previously painless swelling.
by a cartilage cap which is usually 1 to 3mm wide.
The cartilage cap is lined by a thin fibrous layer called
Management
bursa exostotica.
If the patient present in late teens after cessation of
Multiple Hereditary Exostosis growth, then the patient must be counseled that the
Exostosis is also known as diaphyseal aclasis, which is further growth of exostosis is unlikely. In case of
an autosomal dominant disorder. Exostoses are most asymptomatic swelling in growing childeren nothing
common around knee, ankle and shoulder joint. Often, needs to be done. Such swellings are kept under
these are of sessile variety. The probability of a observation for any future symptoms or any signs of
malignant transformation is high as compared to malignant changes.
solitary exostosis. Those around shoulder and pelvis Surgery In case the swelling becomes painful,
are particularly at greater risk for malignant trans- sarcomatous transformation must be ruled out.
formation. Whenever necessary, as in neurovascular compression
or mechanical block to joint movement, tumor should
Clinical Features be excised along with periosteum over the exostosis.
The patient is usually an adolescent presenting with Leaving periosteum may leave few cartilage cells
a painless bony-swelling around a joint. The site is which may lead to recurrence of the tumor.
1222 Essentials of Surgery

complications. Chondrosarcoma is the most

dangerous complication in Ollier’s disease and even
short bones of hand and feet may undergo malignant
transformation. Maffuci syndrome consists of multiple
enchondromas and soft tissue hemangiomatosis.

Treatment

Curettage and bone grafting are the most common

Fig. 67.10: X-ray of showing multiple enchondromatosis
methods of treatment for the enchondromas.

Enchondroma Chondrosarcoma

Enchondroma is the second most common benign Chondrosarcoma is malignant tumor arising from
bone tumor, constituting about 10 percent of all benign cartilage cells. It typically occurs in flat bones at pelvis,
bone tumors. It consists of a lobulated mass of cartilage scapula and ribs. Among long bones, femur and
encapsulated by fibrous tissue. It is the most common humerus are most commonly involved. It commonly
cause of a solitary cyst-like lesion in short tubular occurs after 40 years of age with male predominance
bones of hand or foot (Fig. 67.10). (male: female = 2:1). Chondrosarcoma may be primary,
i.e. arising from a normal bone or secondary, i.e. arising
from pre-existing cartilage tumor as enchondroma or
Clinical Features
osteochondroma.
Enchondroma is commonly seen in young adults in
third decade with no sex predilection and often Clinical Features
presents as pathological fracture. After hands and
feet, knee is the second most common site of Primary chondrosarcomas are mostly slow growing
involvement. Malignant transformation may occur, tumors. Pain and tenderness occur later. Secondary
usually in long or flat bones, rarely short bones in chondrosarcomas develop in younger age group of 20
case of solitary enchondroma. to 40 years and is more benign as compared to primary
chondrosarcoma.
X-rays will show an expansile lesion, thickening
Radiology
of cortex, and endosteal scalloping. Soft tissue mass
The tumor in the short bones is entirely radiolucent may be seen along with characteristic punctate
and scalloping is present due to cartilaginous nature calcifications within the bone.
of tumor. In the long bones, tumor shows calcification,
which is annular, punctate, or popcorn like. A lesion Treatment
longer than 4 cm localized thickening of cortex and In case of low grade chondrosarcoma a wide resection
point toward malignancy. is done. Amputation is usually necessary for most
malignant tumors. Chemotherapy and radiotherapy
Differential Diagnosis are not of much help.

• Bone infarct
Fibrous Dysplasia
• Slow growing chondrosarcoma.
Multiple enchondromas are seen in a condition Fibrous dysplasia is a disorder where bone is replaced
known as enchondromatosis or Ollier’s disease. It is a by abnormal fibrous tissue containing abnormal bone
nonhereditary disorder and presents with multiple trabeculae. The mass of fibrous tissue grows inside
knobby swellings usually in unilateral distribution. the bone and erodes the cortices of the bone from
Deformities and growth discrepancy are the common within.
 Tumors of Bones and Joints 1223

The following types of fibrous dysplasias have Biopsy

been described.
1. Monostotic: In monostotic variety, only single bone Diagnosis is confirmed by biopsy.
involvement is seen. This form affects the femur,
tibia, ribs or craniofacial bones. Children in 5 to 15 Treatment
years of age are commonly affected. Pain,
The fibrous defect is thoroughly curetted out and the
deformity or fractures are the usual presenting
gap is filled with bone grafts.
features.
2. Polyostotic: Multiple bone involvement variety is
called polyostotic fibrous dysplasia. This type of Simple Bone Cyst
presentation may be associated with precocious This is the only true cyst of the bone. Generally, this is
puberty (Albright’s syndrome) and other endo- seen in childhood and adolescence. It is a fluid
crine disorders such as acromegaly, thyrotoxicosis, containing lytic bone lesion of unknown etiology. It is
or Cushing’s syndrome. Café-au-lait spots in skin more common in males.
may occur. Café-au-lait spots seen in Albright
syndrome have irregular ragged borders as
Sites of Involvement
opposed to smooth borders in neurofibromatosis.
Craniofacial bones are almost always involved in Metaphysis of proximal humerus and femur account
this form. for 90 percent of all simple bone cysts (Fig. 67.12).
Calcaneum and ilium are other sites of occurrence. In
Radiology older age group, involvement of calcaneum and ilium
increases.
An X-ray will show sharply defined, expansile,
centrally placed lytic areas with homogenous ground Pathology
glass appearance (Fig. 67.11). Alphabet soup or Chinese
ideograph shaped trabeculae is seen. Shepherd The cyst is lined by a thin fibrous membrane and
crook like deformity may be seen in case pathological contains within the cavity yellow serous fluid.
fracture occurs in neck of femur.

Fig. 67.11: X-ray of humerus, lateral Fig. 67.12: Unicameral bone cyst aring
view, showing fibrous displasia from proximal third of humerus
1224 Essentials of Surgery

Clinical Features

Often, simple bone cyst is asymptomatic and diagnosis

is by an accidental X-rays finding. Only pain or
pathological fracture are the presenting features.

Radiology

The X-rays show a well defined lytic lesion with

sclerotic border located at metaphysis. The lesion
may advance to diaphysis as the age advances. The
maximum width of the lesion is less than the width of
epiphyseal plate. Central position and absence of
periosteal reaction are the characteristic features and
differentiate it from aneurysmal bone cyst. Fallen Fig. 67.13: X-ray pelvis showing aneurysmal bone cyst
fragment sign may be seen in simple bone cyst. involving proximal third of right femur

Treatment Classical ballooned out appearance with trabeculation

Conservative Asymptomatic bone cysts need no within the tumor, if seen, clinches the diagnosis.
treatment. These are to be observed and future
probability of pathological fracture is told to the Treatment
parents or attendents of the patient. Pathological
fracture is treated conservatively and fracture heals Asymptomatic lesions, which are often detected by
in due course. Methyl prednisolone has been injected chance in X-rays, are left as such. Pathological fractures
within the cyst and results are found to be encouraging heal normally and therefore do not warrant any special
healing with the cysts. treatment. Simple immobilization is sufficient except
in certain cases where surgical intervention is needed
Surgery Curettage and bone grafting is done in
urgently.
selected cases.

Giant Cell Tumor

Aneurysmal Bone Cyst

It is an expansile lytic bone lesion, which generally Giant cell tumor (GCT) (osteoclastoma) is a potentially
occurs before the age of 20 years. Common sites are malignant bone tumor. Tumor has predilection for
the ends of long bones and dorso lumbar spines. females and commonly occurs after the closure of
epiphysis with peak incidence in third decade of life.
Pathology Approximately 40 to 50 percent of the tumors occur
around the knee, the other common site is distal radius
The lesion shows cystic spaces containing blood. The
(Fig. 67.14). Giant cell tumors arise eccentrically in
spaces are separated by multiple septae.
the epiphysis with tendency to approach adjacent
Clinical Features subarticular cortex as they enlarge.

Commonly, it presents as swelling increasing in size Pathology

at the end of long bones. Pathologicl fracture after a
trivial injury is another way of presentation. The tumor is composed of a dual population of
mononuclear stromal cells and multinucleated giant
Radiology cells. These giant cells resemble osteoclasts but are
X-rays reveal an expansile, eccentric, lytic, meta- not the true tumor cells; hence the name osteoclastoma
physeal lesion with thinned out cortex (Fig. 67.13). is misnomer.
 Tumors of Bones and Joints 1225

Figs 67.14A and B: X-rays (A and B) showing giant cell

tumor arising from distal third of radius

Clinical Features

Common presentation is vague pain and swelling

(Figs 67.15A and B). Pathological fracture is not
uncommon. Less than 5 percent of GCTs are malig-
nant. Secondary malignancies can occur in previously Figs 67.15A and B: A giant cell tumor of the wrist
irradiated GCT. Secondary malignancies include
fibrosarcoma, and osteosarcoma. Pulmonary meta-
stasis may occur.

Radiology
A well-defined multiloculated cystic lesion gives the
soap bubble appearance. The tumor expands the bone
and is usually not surrounded by sclerotic margin.
No periosteal reaction is seen unless fracture is present.

Differential Diagnosis
The following tumors may mimic the giant cell tumor.
1. Brown tumor seen in hyperparathyroidism
2. Aneurysmal bone cyst
3. Chondrosarcoma
4. Chondromyxoid fibroma (Fig. 67.16). Fig. 67.16: X-ray showing chondromyxoid fibroma
affecting proximal tibia
Treatment
Excision with reconstruction by turn-o-plasty,
Excision of the tumor alone whenever feasible as in fibular grafting or replacement of joint.
case of expendable bones such as fibula or ribs is Curettage and bone grafting is the most common
sufficient. course of treatment. It is done when the tumor has
1226 Essentials of Surgery

not yet involved the joint and not gone beyond the
cortex.
d. Cryotherapy with liquid nitrogen has been tried
with success.
e. Amputation for more aggressive cases or recur-
rence of GCT of limbs.
f. Radiotherapy has been tried for lesions either
nonoperable or at inacessible sites such as spinal
GCT.

Ewing’s Sarcoma

Ewing’s sarcoma is the fourth most common primary

malignant tumor of bone with myeloma, osteosarcoma
and chondrosarcoma being the leading three in the
same order. It is highly malignant tumor of bone
involving both long (60%) and flat bones (40%). Femur
is the most common bone involved followed by tibia,
fibula, humerus, pelvis and scapula. It is the only
Fig. 67.17: X-ray showing Ewing's sarcoma of humerus
malignant tumor that may present as infection. ESR
may be raised. It commonly occurs in the age group
of 5 to 25 years with male to female ratio of 2:1. This is Multiple Myeloma
the most common malignant tumor to metastasize to
bone. Multiple myeloma is most common primary
malignant neoplasm of the bone. It is derived from
plasma [Link] neoplasm has affinity for flat bones,
Clinical Features
i.e. pelvis, vertebrae, skull and ribs. When it occurs as
It commonly presents as pain and swelling. Fever, a solitary lesion it is known as plasmacytoma.
anemia and leukocytosis may be seen.
Pathology
Pathology
The bone is simply replaced by tumor. There is no
It consists of undifferentiated round cells in sheets. reactive new bone formation. Microscopically, the
These are hyperchromatic cells with scanty cytoplasm. tumor cells have an eccentric nucleus with clumped
So this tumor is also known as round cell tumor. chromatin.
An X-ray will demonstrate a permeative growth
with a soft tissue mass. A characteristic onion-peel Clinical Features
periosteal reaction (Fig. 67.17) is observed with
cortical saucerization that is characteristic. The tumor affects the elderly people at fifth decade
and above with male predominance. The most
Treatment common presentation is backache, which increases
progressively. Lumbar and thoracic vertebrae are
Excision biopsy is done to establish the diagnosis. commonly affected. Pathological fracture frequently
Radiotherapy is given to control the tumor. Chemo- occurs resulting in exacerbation of symptoms.
therapy is given to take care of distant metastasis. Paraplegia with varying degree of neurological
After radiotherapy, the tumor is excised , gap is filled involvement may occur due to compression of the
with bone, and the segments are internally supported spinal cord. Pathological fractures of the neck of femur
with plate and screws or nail. Amputation is done in and other long bones after a trivial injury are other
selected cases only. forms in which multiple myeloma may present.
 Tumors of Bones and Joints 1227

Investigations Tumor Metastasis in Bones

The salient features of findings of relevant investi- Metastatic tumors to the bone comprise of the majority
gations are given below: of malignant tumors of bone. These are more common
than primary malignant bone tumors. Bone metastasis
X-rays Multiple punched out lesions are commonly
occurs in elderly age group and the common
seen in skull, pelvis ribs and vertebrae. Pathological
presentation is backache due to involvement of spine.
fractures of long bones, if present, are seen. Wedge
It may present as a pathological fracture of the
collapse of vertebrae is common in thoracic vertebrae.
extremity. The most common tumors that metastasize
Erosion of border of ribs and rarefaction of the
to the bone are carcinomas of lungs, prostate breasts,
involved bones are some of the earlier findings.
thyroid and kidneys.
Blood The patient shows anemia with a high ESR, Metastasis of tumor to bones from distant primary
often in the range of 100 and above, is very suggestive lesion is by hematogenous route. The osteoblastic or
of multiple myeloma. The serum alkaline phosphatase sclerotic bony secondaries are usually from carcinoma
is characteristically normal but the serum calcium is of prostate, breast, thyroid, lung and lymphoma. The
raised. The A/G ratio is reversed. lesions like carcinoma of thyroid, bladder and renal
cell carcinoma may remain silent despite bony
Serum electrophoresis A characteristic M spike in secondary lesions giving symptoms.
the region of gammaglobulin is seen and is diagnostic
of multiple myeloma.
Investigation
Urine Bence Jones proteins, if seen, are indicative of
multiple myeloma. Skeletal survey Radiological skeletal survey is done
to see the extent of involvement.
Bone biopsy It is helpful in confirming the diagnosis
of multiple myeloma. Bone scan The increased uptake of bone scan helps
to detect the involved sites, which are not seen on
Bone marrow cytology It is also found to be helpful X-rays.
in certain occasions where diagnosis is not clear.
Serum chemistry Serum calcium and alkaline phos-
Bone scan This is done to detect all other skeletal phatase are usually raised.
sites involved due to multiple myeloma which are
not detected immediately by other means of Endoscopy, ultrasonography of abdomen, CT or
investigations. MRI It may be done to detect the primary sites.

Management Treatment

The main modality of treatment is chemotherapy. The initial treatment is to provide symptomatic relief
The drug of choice is melphalan. Others drugs used to the patient. Rest and splintage of limbs showing
are vincristine, prednisolone and cyclophosphamide. impending pathological fractures is helpful. Diagnosis
The treatment cycles are repeated every 4 weeks for 6 of the nature of tumor is made by biopsy. Attempts
to 12 cycles. are made to find the site of origin of primary tumor.
Radiotherapy is given in cases of multiple mye- Chemotherapy or radiotherapy is administered
loma with neurological compression of cord, painful depending on the nature of primary lesion. If feasible,
pathological fracture, and soft tissue masses. definitive operations are planned for the primary
Splinting and bracing are important to prevent lesion. Nature and extent of bone secondaries dictate
the impending pathological fractures during the the types of operative procedures for the bone
treatment of multiple myeloma of long bones. metastasis.
1228 Essentials of Surgery

SUGGESTED READING 4. Huvos AG: Bone tumors. Diagnosis Treatment Prognosis. WB

Saunders: Philadelphia, 1997.
1. Enneking WF: A system of staging musculoskeletal neoplasm. 5. Schojowics F: Tumor and tumor like lesions of bone. Pathology,
Clin Orthop 204: 9-24, 1986. Radiology and Treatment, (2nd edn), Berlin: Springer Verlag, 1994.
2. Enneking WF: Musculoskeletal Tumor Surgery. New York, 6. Schajowic ZF: Tumors and Tumor like Lesions of Bone. Springer
Churchill Livingstone 1: 1983. 1994.
3. Evart CM: Surgery of Musculoskeletal System, (2nd edn), New
York, Churchill Livingstone.