tumorsg
Post on 13-Apr-2018
221 Views
Preview:
TRANSCRIPT
-
7/26/2019 Tumorsg
1/22
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.
-
7/26/2019 Tumorsg
2/22
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%.
-
7/26/2019 Tumorsg
3/22
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.
-
7/26/2019 Tumorsg
4/22
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.
-
7/26/2019 Tumorsg
5/22
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
-
7/26/2019 Tumorsg
6/22
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.
-
7/26/2019 Tumorsg
7/22
MAGNETIC RESONANCE IMAGING
!
-
7/26/2019 Tumorsg
8/22
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
-
7/26/2019 Tumorsg
9/22
breakdon, infection and pathological fracture "!ankin at al., $#F, $##G;
0pringfield and
-
7/26/2019 Tumorsg
10/22
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.
-
7/26/2019 Tumorsg
11/22
'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@!(@
-
7/26/2019 Tumorsg
12/22
5CC
-
7/26/2019 Tumorsg
13/22
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.
-
7/26/2019 Tumorsg
14/22
?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@!(@
-
7/26/2019 Tumorsg
15/22
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 "!% "
-
7/26/2019 Tumorsg
16/22
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
-
7/26/2019 Tumorsg
17/22
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
-
7/26/2019 Tumorsg
18/22
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%.
-
7/26/2019 Tumorsg
19/22
>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
-
7/26/2019 Tumorsg
20/22
-
7/26/2019 Tumorsg
21/22
!@>T3-5C1AT 241!(T41
-
7/26/2019 Tumorsg
22/22
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.
top related