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TUMORS

Tumours, tumour-like lesions and cysts are considered together, partly

because their clinical presentation and management are similar and partly because

the definitive classification of bone tumours is still evolving and some Disorders may

yet move from one category to another.

Benign lesions are quite common, primary malignant ones rare; yet so often

do they mimic each other, and so critical are the decisions on treatment, that a

orking knoledge of all the important conditions ais necessary.

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CLASSIFICATION

!ost classifications of bone tumours are based on the recognition of the dominant

tissue in the various

lesions "Table #.$%. &noing the cell line from hich the tumour has sprung may

help ith both diagnosis and planning of treatment. There are, hoever, pitfalls in

this approach'

The most pervasive tissue is not necessarily the tissue of origin

There is not necessarily any connection beteen conditions in one category

There is often no relationship beteen benign and malignant lesions ith

similar tissue elements "e.g.

(steoma and osteosarcoma%

The commonlest malignant lesions in bone ) metastatic tumours ) are not,

strictly speaking,

*bone+ tumours, i.e. not of mesenchymal origin.

CLINICAL PRESENTATION

HISTORY

The history is often prolonged, and this unfortunately results in a delay in

obtaining treatment. atients may be completely asymptomatic until the abnormality

is discovered on -ray. This is more likely ith benign lesions; and, since some of

these "e.g. non-ossifying fibroma% are common in children but rare after the age of

/, they must be capable of spontaneous resolution. !alignant tumours, too, may

remain silent if they are slo-groing and situated here there is room for

inconspicuous epansion "e.g. the cavity of the pelvis%.

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Age may be a useful clue. !any benign lesions present during childhood and

adolescence ) but so do 0ome primary malignant tumours, notably 1ing+s Tumour

and osteosarcoma. 2hondrosarcoma and fibrosarcoma typically occur in older people

"fourth or sith decades%; and myeloma, the commonest of all primary malignant

bone tumours, is seldom seen before the sith decade. In patients over 70 years of

age, metastatic bone lesions are more common than all primary tumours together.

Pain is a common complaint and gives little indication of the nature of the lesion;

hoever, progressive and unremitting pain is a sinister symptom. 3t may be caused

by rapid epansion ith stretching of surrounding tissues, central haemorrhage or

degeneration in the tumour, or an incipient pathological fracture. 4oever, even a

tiny lesion may be very painful if it is encapsulated in dense bone "e.g. an osteoid

osteoma%.

Swelling, or the appearance of a lump, may be alarming. (ften, though, patients seek

advice only hen a mass becomes painful or continues to gro.

5 history of trauma is offered so frequently that it cannot be dismissed as having no

significance. 6et, hether the in7ury initiates a pathological change or merely dras

attention to hat is already there remains unansered.

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Neurological symptoms "paraesthesiae or numbness% may be caused by pressure

upon or stretching of a peripheral nerve. rogressive dysfunction is more ominous

and suggests invasion by an aggressive tumour.

Pathological fracture may be the first "and only% clinical signal. 0uspicion is aroused

if the in7ury as slight; in elderly people, hose bones usually fracture at the cortico-

cancellous 7unctions, any break in the mid-shaft should be regarded as pathological

until proved otherise.

EXAMINATION

3f there is a lump, here does it arise8 3s it discrete or ill-defined8 3s it soft or hard, or

pulsatile8 5nd is it tender8

Swelling

is sometimes diffuse, and the overlying skin arm and in9amed; it can be difficult to

distinguish a tumour from infection or a haematoma. 3f the tumour is near a 7oint

there may be an effusion and:or limitation of movement. 0pinal lesions, hether

benign or malignant, often cause musclespasm and back stiffness, or a painful

scoliosis.The eamination ill focus on the symptomatic part, but it should include

the area of lymphatic drainage and, often, the pelvis, abdomen, chest and spine.

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IMAGINGX-RAYS

lain -rays are still the most useful of all imaging techniques. There may be an

obvious abnormality in the bone ) cortical thickening, a discrete lump, a *cyst+ or ill-

defined destruction. here is the lesion' in the metaphysis or the diaphysis8 3s it

solitary or are there multiple lesions8 5re the margins ell-defined or ill-defined8

ook also at the soft tissues' 5re

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the muscle planes distorted by selling8 3s there calcification8 ?or all its informative

detail, the -ray alone can seldom be relied on for a definitive diagnosis. ith some

notable eceptions, in hich the appearances are pathognomonic "osteochondroma,

non-ossifying fibroma, osteoid osteoma%, further investigations ill be needed. If

other formsof imaging are planned (bone scans, ! or "#I$, they should be done

before undertaking a biopsy, which itself may distort the appearances.

RADIONUCLIDE SCANNING

0canning ith ##mTc-methyl diphosphonateTc!D% shos non-specific reactive

changes in bone; this canbe helpful in revealingthe site of a small tumour"e.g.an

osteoid osteoma% that does not sho up clearlyon-ray.0keletal scintigraphy is also

useful for detectingskip lesions or *silent+ secondary

deposits.

COMPUTED TOMOGRAPHY

2T etends the range of -ray diagnosis; it shos more accurately both intraosseous

and etraosseous

etension of the tumour and the relationship to surrounding structures. 3t may also

reveal suspected lesions in inaccessible sites, like the spine or pelvis; and it

is a reliable method of detecting pulmonary metastases.

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MAGNETIC RESONANCE IMAGING

!

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3t is also essential that the biopsy is carried out in the line of any further surgical

incision so that the tract can be ecised at the time of definitive surgery.

*pen biopsy This is a more reliable ay of obtaining a representative sample,

hoever it is associated ith significant morbidity "!ankin et al., $#F%. 3t is often

performed if a needle biopsy ould place the neurovascular structures at risk or if a

diagnosis has not been made after needle biopsy.

The site is selected so that it can be included in any subsequent operation. 5s little as

possible of the tumour is eposed and a block of tissue is removed ) ideally in the

boundary =one, so as to include normal tissue, pseudocapsule and abnormal tissue. 3f

bone is removed the ra area is covered ith bone a or methylmethacrylate

cement. 3f a tourniquet is used, it should be released and

full haemostasis achieved before closing the ound. Drains should be avoided, so as

to minimi=e

the risk of tumour contamination.5n eperienced histopathologist should be on hand

and the specimens should be delivered fresh, unfied and uncrushed. ?or tumours

that are almost certainly benign, ane+cisional biopsy is permissible "the entire lesion

isremoved%; ith cysts that need operations, representativetissue can be obtained by

careful curettage. 3n either case, histological confirmation of the diagnosis is

essential.

Biopsy should never be regarded as a *minor+ procedure. 2omplications include

haemorrhage, ound

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breakdon, infection and pathological fracture "!ankin at al., $#F, $##G;

0pringfield and

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loer limb. 0ometimes the -ray shos an irregular surface on the underlying bone.

3mportant clues are the history and the rapid onset of symptoms.

"yositis ossicans 5lthough rare, this may be a source of confusion. ?olloing an

in7ury the patient develops a tender selling in the vicinity of a 7oint; the -ray shos

9uffy density in the soft tissue ad7acent to bone. @nlike a malignant tumour,

hoever, the condition soon becomes less painful and the ne bone better defined

and ell demarcated.

Stress fracture 0ome of the orst mistakes have been made in misdiagnosing

a stress fracture. The patient is often a young adult ith locali=ed pain near a large

7oint; -rays sho a dubious area of cortical *destruction+ and overlying periosteal

ne bone; if a biopsy is performed the healing callus may sho histological features

resembling those of osteosarcoma. 3f the pitfall is recogni=ed, and there is adequate

consultation beteen surgeon, radiologist and pathologist, a serious error can be

prevented.

!endon avulsion in1uries 2hildren and adolescents ) especially those engaged in

vigorous sports ) are prone to avulsion in7uries at sites of tendon insertion,

particularly around the hip and knee "Donnelly et al., $###%. The best knon

eample is the tibial apophyseal stress lesion of (sgood)0chlatter+s disease "see

page HGH%, but lesions at less familiar sites "the iliac crest, the ischial tuberosity, the

lesser trochanter of the femur, the hamstring insertions, the attachments of adductor

magnus and longus and the distal humeral apophyses% may escape immediate

recognition.

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'one infection (steomyelitis typically causes pain and selling near one of the

larger 7oints; as ith primary bone tumours, the patients are usually children or

young adults. I-rays may sho an area of destruction in the metaphysis, ith

periosteal ne bone. 0ystemic features, especially if the patient has been treated ith

antibiotics, may be mild. 3f the area is eplored, tissue should be submitted for both

bacteriological and histological eamination.

2out (ccasionally a large gouty tophus causes a painful selling at one of the bone

ends, and -ray

shos a large, poorly defined ecavation. 3f it is kept in mind the diagnosis ill be

easily confirmed ) if necessary by obtaining a biopsy from the lump.

*ther bone lesions Aon-neoplastic bone lesions such as fibrous cortical defects,

medullary infarcts and

*bone islands+ are occasionally mistaken for tumours.

0T5C3AC (? B(A1 T@!(@

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5CC

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aggressive and take a long time to metastasi=e "e.g. secondary chondrosarcoma or

parosteal osteosarcoma%, hile the latter are usually very aggressive and metastasi=e

early "e.g. osteosarcoma or fibrosarcoma%.

0esions that are

confined to an enclosed tissue space "e.g. a bone, a 7oint cavity or a muscle group

ithin its fascial envelope% are called *intracompartmental3. Those that etend into

interfascial or etrafascial planes ith no natural barrier to proimal or distal spread

"e.g. perivascular sheaths, pelvis, ailla% are designated 4e+tracompartmental3. The

etent of the tumour and ad7acent *contaminated+ tissue are best shon by 2T and

!ocally recurrent sarcomas tend to be

more aggressive, more often etracompartmental and more likely to metastasi=e than

the original tumour.

Bone sarcomas are broadly divided as follos'

Stage I 5ll lo-grade sarcomas.

Stage II 4istologically high-grade lesions.

Stage III 0arcomas hich have metastasi=ed.

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?olloing 1nneking+s original classification, each category is further subdivided into

!ype % "intracompartmental% and !ype' "etracompartmental% "?ig. #.%.

Thus, a locali=ed chondrosarcoma arising in a cartilage-capped eostosis

ould be designated 35, suitable for ide ecision ithout eposing the tumour. 5n

osteosarcoma confined to bone ould be 335 ) operable by ide ecision or

amputation ith a lo risk of local recurrence; if it has spread into the soft tissues it

ould be 33B ) less suitable for ide ecision and preferably treated by radical

resection or disarticulation through the proimal 7oint. 3f there are pulmonary

metastases it ould be classified as stage 333.

0T5C3AC (? 0(?T-T300@1 T@!(@

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0oft-tissue tumours are staged using the 5merican Eoint 2ommittee for 2ancer

0taging 0ystem, according to their histological grade "C%, si=e "T%, lymph node

involvement "A% and hether they have metastasi=ed "!% "

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cooperation beteen the orthopaedic surgeon, radiologist, patho logist and "certainly

in the case of malignant tumours%

The oncologist is essential in the initial management. 3n many cases

physiotherapists, occupational therapists and prosthetists ill also be involved.

(nce clinical and radiological eamination have suggested the most likely

diagnosis, further management proceeds as follos.

'enign, asymptomatic lesions 3f the diagnosis is beyond doubt "e.g. a non-ossifying

fibroma or a small osteochondroma% one can afford to tempori=e; treatment may

never be needed. 4oever,

if the appearances are not pathognomonic, a biopsy is advisable and this may take the

form of ecision or curettage of the lesion.

'enign, symptomatic or enlarging tumours ainful lesions, or tumours that continue

to enlarge after the end of normal bone groth, require biopsy and confirmation of

the diagnosis. @nless they are unusually aggressive, they can generally be removed

by local "marginal% ecision or "in the case of benign cysts% by curettage.

Suspected malignant tumours 3f the lesion is thought to be a primary malignant

tumour, the patient is

admitted for more detailed eamination, blood tests, chest -ray, further imaging

"including pulmonary 2T% and biopsy. This should allo a firm diagnosis and staging

to be established. The various treatment options can then be discussed ith the

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patient "or the parents, in the case of a young child%. 5 choice needs to be made

beteen amputation, limb-sparing operations and different types of ad7uvant therapy,

and the patient must be fully informed about the pros and cons of each.

!1T4(D0 (? T

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belo $/ per cent. 4oever, ide ecision is also used in con1unction with

chemotherapy for grade 335 lesions.

#adical resection means that the entire compartment in hich the tumour lies is

removed enblocithout eposing the lesion. 3t may be possible to do this hile still

sparing the limb, but the surrounding muscles, ligaments and connective tissues ill

have to be sacrificed; in some cases a true radical resection can be achieved only by

amputating at a level above the compartment involved. This method is required for

high-grade tumours "335 or 33B%.

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>3!B 05>5C1

5mputation is no longer the automatic choice for grade 33 sarcomas. 3mproved

methods of imaging and advances in chemotherapy have made limb salvage the

treatment of choice for many patients. 4oever, this option should be considered

only if the local control of the tumour is likely to be as good as that obtained by

amputation, if it is certain that there are no skip lesions and if a functional limb can

be preserved. The ongoing debate around limb sparing versus amputation is

addressed in an ecellent paper by Di2aprio and ?riedlaender "//%.

5dvanced surgical facilities for bone grafting and endoprosthetic replacement

at various sites must be available. The first step consists of ide ecision of the

tumour ith preservation of the neurovascular structures. The resulting defect is then

dealt ith in one of several ays. 0hort diaphyseal segments can be replaced by

vasculari5edor non/vasculari5ed bonegrafts.>onger gaps may require custom/

madeimplants. (steo-articular segments can be replaced by large allografts,

endoprostheses or allograft6prostheticcomposites. 3t is recogni=ed, hoever, that the

use of large allografts carries a high risk of infection and fracture; this has led to

them not being used as idely as

3n the past. 1ndoprostheses used to be custom-made but noadays modular systems

for tumour reconstruction are available.

3n groing children, e+tendible implants have been used in order to avoid the

need for repeated operations; hoever, they may need to be replaced at the end of

groth. (ther procedures, such asgraftingandarthrodesisor distraction

osteosynthesis, are suitable for some situations. 0arcomas around the hip and

shoulder present special problems. 2omplete ecision is difficult and

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!@>T3-5C1AT 241!(T41

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inaccessible sites, lesions that are inoperable because of their si=e, proimity to

ma7or blood vessels or advanced local spread, for marro-cell tumours such as

myeloma and malignant lymphoma, for metastatic deposits and for palliative local

tumour control here no surgery is planned.