OrthoTrauma - 2017 - Cosker - SXTX of 1o Bone Sarcomas

ORTHOPAEDIC ONCOLOGY
Surgical management of any neo-adjuvant treatment (which may occur with open biopsy
whilst waiting for the wound to heal). When a percutaneous bi-
primary bone sarcomas opsy is inconclusive, an open biopsy is of course mandatory

(Tables 1 and 2).
Tom Cosker The orientation and location of the biopsy tract are critical.
C L M H Gibbons Before biopsy, the surgeon should review the radiographs with
the pathologist to plan the biopsy site. As in percutaneous bi-
opsy, one should attempt to factor in the future skin incisions
needed for definitive surgery. Whenever possible the surgeon
Abstract
should try to avoid using drains, however if needed these should
This article addresses the surgical management of bone sarcoma and
exit either from the corner of the wound or close to the skin
includes all the main tumour types, for example chrondrosarcoma, os-
incision that will make resection straight forward to include in
teosarcoma and Ewings sarcoma of bone. The article is aimed at the
future approaches. Transverse incisions should be avoided.
experienced orthopaedic surgeon who may not have specic knowl-
The surgeon must ensure careful attention to haemostasis to
edge of dealing with musculoskeletal tumours. Important principles,
prevent haematoma formation and subcutaneous haemorrhage.
such as biopsy, are discussed and appropriate resection methods
Biopsy incisions should ideally be made through muscle com-
described. The use of endoprosthetic replacement is a crucial tool in
partments so that the muscle layer can be closed tightly. Neu-
the orthopaedic oncologists armoury and principles of use are
rovascular structures are avoided. A tourniquet is used to obtain
described. Adjuvant therapy such as chemotherapy and radiotherapy
tissue in a bloodless field and are then released so that bleeding
is also discussed and some common protocols are also described.
points are fully controlled.
Keywords bone sarcoma; chondrosarcoma of bone; endoprosthetic All biopsy samples should be submitted for microbiological
replacement; Ewings sarcoma; orthopaedic oncology; osteosarcoma
culture and sensitivity. Antibiotics should not be delivered until
the cultures are obtained (Table 3).
Introduction
Treatment: general principles
The surgical management of bone sarcomas has changed over
the last 30 years from predominantly amputation to an approach The goal of the treatment of malignant bone tumours is to
that is focused on functional limb salvage as a result of improved remove the lesion with a clear margin to minimize the risk of
oncologic management and advances in biological orthoplastic local recurrence.
reconstruction techniques and implant design. Surgery remains Wherever possible, limb salvage is employed. This is only
pivotal in the treatment of bone sarcoma notably chon- possible where two essential criteria are met:
drosarcoma and an understanding of the principles involved is local control of the lesion must be at least equal to that of
essential for any orthopaedic surgeon. amputation surgery
There are a number of surgical reconstructive options which the limb that has been saved must be functional.
are available, with the use of implants and biological techniques Surgical margins are graded according to the system of the
including custom and modular endoprosthesis, allograft, vascu- Musculoskeletal Tumor Society.
larized free fibular grafting (VFFG) and bone transplant. Some Intralesional margin: The plane of dissection goes
bone sarcomas require neo-adjuvant chemotherapy and timing of directly through the tumour. When the surgery involves
surgery is of utmost importance. malignant tumours, an intralesional margin results in
The management of bone sarcoma may begin with neo- 100% local recurrence. This should only occur when an
adjuvant therapy in Ewings sarcoma and osteosarcoma or may inadvertent excision has been performed and sometimes
rely on surgical treatment alone in chondrosarcoma. in low-grade chondral lesions (see below). (It is also uti-
lized for benign tumours such as giant cell tumours e see
Biopsy below and the article on Management of benign bone
tumours elsewhere in this issue; http://dx.doi.org/10.
Biopsy is important because it determines the tumour type and
1016/j.mporth.2017.03.008.)
grade. Where possible, a biopsy is undertaken under radiological
Marginal margin: A marginal line of resection goes
control. This ensures that the most representative region identi-
through the reactive zone of the tumour; which contains
fied by various radiologic methods is biopsied. A percutaneous
inflammatory cells, fibrous tissue, and areas of tumour
image-guided biopsy also ensures that there is no delay in starting
cells. When malignant tumours are resected through the
reactive zone probably results in a local recurrence rate of
25e50%. A marginal margin may be safe and effective if
Tom Cosker Consultant Orthopaedic Oncology Surgeon, Nufeld the response to preoperative chemotherapy has been
Orthopaedic Centre, Oxford and Director of Human Anatomy, excellent (95e100% tumour necrosis).
University of Oxford, UK. Conicts of interest: none declared. Wide margin: This is the preferred margin. A wide line of
C L M H Gibbons Consultant Orthopaedic Oncology Surgeon, surgical resection is accomplished when the entire tumour
Nufeld Orthopaedic Centre and Chair, Oxford Sarcoma Service, UK. is removed with a cuff of normal tissue. The local recurrence
Conicts of interest: none declared. rate is around 10% when such a surgical margin is achieved.
ORTHOPAEDICS AND TRAUMA --:- 1 Crown Copyright 2017 Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Cosker T, Gibbons CLMH, Surgical management of primary bone sarcomas, Orthopaedics and Trauma (2017),
http://dx.doi.org/10.1016/j.mporth.2017.03.006
Bone lesions by age Tumourebone interaction (from Lodwick)

Lesion Type I Type II Type III
Age <5 <30 >30
(years)
Radiographic Geographic Moth eaten Destructive
appearance A-sclerotic
Malignant LCH (Letter-Siwe) Ewings sarcoma Chondrosarcoma
B-distinct
LCH (Hand- Osteosarcoma Metastases
ller-Christian) C-indistinct
Schu
Examples A-Non-ossifying Osteomyelitis Ewings
Metastatic Lymphoma
fibroma Metastases sarcoma
Rhabdomyosarcoma
B-Unicameral bone cyst
Metastatic Myeloma
C-Giant cell tumour
Neuroblastoma
Chordoma
Table 3
Adamantinoma
Benign Osteomyelitis Osteoid osteoma Giant cell
tumour The role of chemotherapy and radiotherapy are discussed in
Osteofibrous Osteoblastoma Pagets disease detail in the article on The non-surgical management of muscu-
Dysplasia loskeletal malignancy elsewhere in this issue (http://dx.doi.org/
Chondroblastoma 10.1016/j.mporth.2017.03.011; see also Table 6).
Aneurysmal bone
cyst Osteosarcoma
LCH
Spindle cell neoplasms that produce osteoid are arbitrarily
Osteofibrous
classified as osteosarcoma.
dysplasia
Many types of osteosarcoma (see below).
Non-ossifying
The most common subtypes are classic osteosarcoma,
fibroma parosteal osteosarcoma, periosteal osteosarcoma, telangi-
LCH, Langerhans cell histiocytosis. ectatic osteosarcoma, osteosarcoma occurring with Pagets
disease, and osteosarcoma after irradiation.
Table 1 Historically, osteosarcoma was treated by amputation;
long-term studies demonstrated a survival rate of only
Radical margin: A radical margin is achieved when the
10e20%, with metastatic lung disease being the com-
entire tumour and its compartment (all surrounding mus-
monest cause of death.
cles, ligaments, and connective tissues) are removed
(Tables 4 and 5).
Adjuvant therapy Classification of primary tumours of bone and bone

Chemotherapy matrixa
Radiation therapy
Histologic type Benign Malignant
Principles of musculoskeletal biopsy Hematopoietic Myeloma

Lymphoma
Principle Rationale
Chondrogenic Osteochondroma Primary chondrosarcoma
Longitudinal Incision in Longitudinal incision is extensile Chondroma Secondary chondrosarcoma
line with future resection Biopsy tract can be excised with Chondroblastoma Dedifferentiated
Chondromyxoid chondrosarcoma
Biopsy through a single final resection remaining extensile
fibroma Mesenchymal
compartment
chondrosarcoma
Avoid critical structures, Contamination of critical structures
i.e. neurovascular bundles precludes limb salvage Clear cell chondrosarcoma
Biopsy the soft tissue Bone is weakened when its cortex Osteogenic Osteoid osteoma Osteosarcoma
component when present is disrupted Osteoblastoma Parosteal osteosarcoma
Bone requires decalcification for Periosteal osteosarcoma
evaluation and this process may Unknown Giant cell tumour Ewings tumour
affect pathology origin (fibrous) histiocytoma Malignant giant cell tumour
Maintain strict haemostasis Avoid increased contamination Adamantinoma
Use a drain in line with outside of the biopsy tract by a
Classification is based on that advocated by Lichtenstein L: Classification of
the incision when needed iatrogenic tumour spread primary tumours of bone, Cancer 4:335e341, 1951.
Table 2 Table 4
Multi-agent chemotherapy has dramatically improved

Tumours by location long-term survival and the potential for limb salvage. The
Epiphyseal standard regime is:
C Chondroblastoma doxorubicin (cardiac toxicity)
C Giant cell tumour cisplatin (neuro toxicity)
C Clear cell chondrosarcoma (femoral head) methotrexate.
Metaphyseal The aim of giving chemotherapy is to kill the micro-
C Osteosarcoma metastases that are present in 80e90% of patients at pre-
C Chondrosarcoma sentation. It also effectively sterilizes the reactive zone
C Metastatic disease around the tumour.
Diaphyseal Osteosarcoma metastasizes most commonly to the lung
C A adamantanoma and next most commonly to bone.
C E eosinophilic granuloma With current treatment regimes the 5-year survival is
C I infection approximately 60e70%.
C O osteoid osteoma/osteoblastoma Prognostic factors that adversely affect survival include:
C U Ewings sarcoma expression of P-glycoprotein, high serum level of alka-
C Y mYeloma, lYmphoma, fibrous dYsplasia line phosphatase, high lactic dehydrogenase level,
C Metastatic disease vascular invasion, and no alteration of DNA ploidy after
Flat bones chemotherapy
C Chondrosarcoma the absence of antieshock protein-90 antibodies after
C Fibrous dysplasia chemotherapy
C Hemangioma a poor response to chemotherapy as seen on histologic
C Pagets disease tumour necrosis (<90%).
C Ewings sarcoma Osteosarcoma is associated with an abnormality in the
Spine tumour suppressor genes Rb (retinoblastoma) and p53 (Li-
Anterior column Fraumeni syndrome) (Table 7).
giant cell tumour
metastatic disease High-grade intramedullary osteosarcoma
Posterior column Also called classic osteosarcoma, this neoplasm is the
osteoid osteoma/osteoblastoma most common type of osteosarcoma. The most frequent
aneurysmal bone cyst site affected is around the knee. It is most common in
Sacrum children and young adults, but it does have a second peak
Midline in late adulthood.
chordoma Other common sites include the proximal humerus, prox-
Eccentric imal femur, and pelvis.
aneurysmal bone cyst/giant cell tumour/metastatic disease Patients present primarily with pain, swelling or a mass.
More than 90% of intramedullary osteosarcomas are high-
Table 5 grade and penetrate the cortex early to form a soft tissue
mass (stage IIB lesion).
About 10e20% of affected patients have pulmonary metas-
tases at presentation. One of the primary aims of improved
education of general practitioners with respect to the man-
American Joint Committee on Cancer staging system for agement of soft tissue lumps is to try and reduce the delay to
primary malignant tumours of bone for those tumours referral to a specialist centre which directly correlates to the
diagnosed on or after January 1, 2010 likelihood of pulmonary metastatic disease at presentation.
Stage Tumour grade Tumour size Radiographs demonstrate a lesion in which there is bone
destruction and bone formation. MRI defines the anatomy of
IA Low <8 cm the lesion with regard to intramedullary extension, involve-
IB Low >8 cm ment of neurovascular structures, and muscle invasion.
IIA High <8 cm Diagnosis depends on two histologic criteria: (1) the
IIB High >8 cm tumour cells produce osteoid and (2) the stromal cells are
III Any tumour grade, skip metastasesa frankly malignant.
IV Any tumour grade, any tumour size, distant Treatment: neo-adjuvant chemotherapy (i.e. before sur-
metastases gery), followed by wide-margin surgical resection and
a adjuvant chemotherapy (i.e. after surgery)
Skip metastases: discontinuous tumours in the primary bone site.
Surgical reconstruction usually involves endoprosthetic
Adapted from AJCC: Bone. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC replacement to reconstruct the resected joint although in
Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 281e90. young patients biological reconstruction with, for example,
a free fibular graft may be a viable alternative.
Table 6
Comparison of osteosarcomas
Classic (intramedullary) Periosteal Parosteal
Age (years) <30 and >60 <30 <45

Presentation Pain Pain Painless mass
Imaging C Mixed lytic/destructive C Sunburst saucerized C Ossified lobulated
aggressive intramedullary surface lesion surface lesion
bone producing lesion C Diaphyseal C Metaphyseal
C Common location metaphyseal C Characteristic location C Characteristic location
femur or tibia posterior distal femur
Histology C Poorly arranged osseous C Osseous trabeculae C Regularly arranged
trabeculae with malignant C Chondroblastic elements osseous trabeculae
rimming osteoblasts C Minimally atypical
C atypical spindle cells spindle cells
Biology 65% 5-year survival 80% 5-year survival 95% 5-year survival
Treatment Chemotherapy Chemotherapy Limb salvage surgery
Limb salvage surgery Limb salvage surgery
Table 7
Amputation of the limb is sometimes necessary in order Histologically we see regularly arranged osseous trabec-
to try to achieve a radical resection. This is normally the ulae; between the nearly normal trabeculae are slightly
case in advanced tumours. It is also considered in young atypical spindle cells, which typically invade skeletal
children where the residual limb length discrepancy at the muscle found at the periphery of the tumour.
end of growth would be more than 10 cm. A residual limb Treatment: resection with a wide margin, which is usually
with good soft tissue cover and appropriate length may be curative, similar as for classic osteosarcoma, usually again
fitted with a prosthetic leg. If chosen well, patients with with endoprosthetic reconstruction. Because this is typically a
amputations have good functional outcomes. lower grade lesion, chemotherapy is not usually administered.
Rotationplasty is the process of attaching the distal tibia Importantly however, of the lesions that appear radio-
and foot to the distal femur rotated through 180 , such that graphically to be parosteal osteosarcoma, up to 20% will
the foot points backwards. In doing so, an otherwise above turn out to be higher-grade malignancies (dedifferentiated
knee amputation is converted into a below knee amputa- parosteal osteosarcoma).
tion with the ankle functioning as the new-knee. This
gives longer lever arm onto which prosthesis can be fitted Periosteal osteosarcoma (intermediate grade,
on. Patients with rotationplasties have better outcome surface)
scores than patients with above knee amputations. This is a rare surface form of osteosarcoma which occurs
Growing prostheses can be used in children where the most often in the diaphysis of long bones (typically the
prosthesis will need to elongate as the child grows. His- femur or tibia).
torically this required multiple return visits to theatre but Radiographic appearance is fairly constant: a sunburst-
with modern devices this can now be achieved by the use type lesion rests on a saucerised cortical depression.
of external electromagnets and an internal servo-motor Histologic characteristics: The lesion is predominantly
such that lengthening can be undertaken in the outpa- chondroblastic, and the grade of the lesion is intermediate
tient setting. Elongation is usually undertaken at a rate of 5 (grade II). Highly anaplastic regions are not normally seen.
mm every 4e6 weeks and neurological function (particu- The prognosis for periosteal osteosarcoma is intermediate
larly the sciatic nerve) is monitored carefully. between those of very low-grade parosteal osteosarcoma
and high-grade intramedullary osteosarcoma. Preoperative
Parosteal osteosarcoma (low-grade surface) chemotherapy, resection, and maintenance chemotherapy
This is typically a lower grade osteosarcoma that occurs on constitute the preferred treatment. The risk of pulmonary
the surface of the metaphysis of long bones and affected metastasis is 10e15%.
patients usually present with a painless mass. Treatment again involves resection with a wide margin
The most commonly affected sites are the posterior aspect of and appropriate reconstruction which will usually be
the distal femur, proximal tibia, and proximal humerus. The endoprosthetic replacement if around the joint.
delay to diagnosis is frequently even longer in these patients
than for classic osteosarcoma, the disease often finally being High-grade surface osteosarcoma
diagnosed some years after it has first developed. This is an extremely rare form of surface osteosarcoma.
Parosteal osteosarcoma has a characteristic radiographic Radiographs usually shows a mixed lytic sclerotic aggres-
appearance with a heavily ossified, often lobulated mass sive surface lesion in the metaphysis or diaphysis of a long
arising from the cortex. bone.
Treatment is the same as conventional osteosarcoma. The tumour has characteristic bimorphic histologic and
Prognosis is the same as classic osteosarcoma. radiographic appearances.
There is a low-grade cartilagenous component that is
Telangiectatic osteosarcoma intimately associated with a high-grade spindle cell sar-
The tissue of the lesion can be described as a bag of blood coma (osteosarcoma, fibrosarcoma, malignant fibrous
with few cellular elements. histiocytoma).
The radiographic features of telangiectatic osteosarcoma More than 80% of the lesions are typical chondrosarcomas
are those of a destructive, lytic, expansile lesion. These with a superimposed, highly destructive area.
lesions should be approached with caution! Telangiectatic Manifestations are similar to those of low-grade chon-
osteosarcomas occur in the same locations as aneurysmal drosarcoma, including pain, swelling and reduced
bone cysts, and the radiographic appearances of both can movement.
be confused hence the diagnosis must always be considered The prognosis is very poor, and rate of long-term survival
before assuming a lesion is a simple aneurysmal bone cyst. is less than 10%.
Treatment: wide-margin surgical resection and multi-agent
Chondrosarcoma chemotherapy as for osteosarcoma above (Table 8).
Intramedullary chondrosarcoma
Malignant neoplasm of cartilage occurring in older adults. Tumours of unknown origin
The most commonly affected sites are the shoulder and Giant cell tumour of bone
pelvic girdles, knee and spine. Benign form
Patients usually present with pain or an enlarging mass. Distinctive neoplasm that has poorly differentiated cells
Radiographs usually show diagnostic findings, with Benign but locally aggressive
bone destruction, thickening of the cortex, and miner- Confusion in diagnosis results from the fact that in rare
alisation consistent with cartilage within the lesion. The cases (<5%), this benign tumour metastasizes to the
MRI scan will often reveal the characteristic feature of lungs (benign metastasizing giant cell tumour).
popcorn calcification. Giant cell tumour most commonly occurs in the epiph-
Differentiating malignant cartilage may be extremely ysis and metaphysis of long bones, and about 50% of
difficult on the basis of histologic features alone and lesions occur about the knee; the vertebra, sacrum, and
biopsy is frequently relatively unhelpful. distal radius are involved in about 10% of cases.
Histologically these lesions contain abundant cells with The sacrum is the most common axial location of giant
plump nuclei, more than an occasional cell with two cell tumours of bone.
such nuclei, especially large cartilage cells with large Unlike most bone tumours, which occur more often in
single or multiple nuclei containing clumps of chro- boys and men, giant cell tumours are more common in
matin, infiltration of the bone trabeculae. girls and women.
Surgical treatment must be meticulous to ensure clear- They are uncommon in children with open physes.
ance of disease and usually involves several important Giant cell tumour usually presents with pain around the
steps: involved joint.
e Mechanical clearance of tumour tissue by surgical Radiologically it is most common to see a purely lytic
excision and macroscopic curettage. destructive lesion in the metaphysis that extends into the
e Coating the cavity in methylene blue followed by use epiphysis and often borders the subchondral bone.
of a high-speed burr to clear the cavity. Only when Histologically we see a basic proliferating cell with a
all the blue has been cleared can the surgeon be round to oval or even spindle-shaped nucleus (giant cells
assured the cavity has been cleared. appear to have the same nuclei as the proliferating
e Phenol can then used to chemically lyse tumour mononuclear cells). Mitotic figures may be numerous.
cells. Giant cell tumours may undergo a number of secondary
e A water wash may also be employed followed by degenerative changes, such as aneurysmal bone cyst
hydrogen peroxide (with due care to nearby neuro- formation, necrosis, fibrous repair, foam cell formation,
vascular structures) to lyse tumour cells. and reactive new bone.
e Bone cement can be inserted into the cavity to pro- Treatment is aimed at removing the lesion as per treat-
vide structural support and to thermally kill any ment of chondrosarcoma above, with preservation of the
remaining tumour cells. involved joint where possible. Chemotherapy and
e Chemotherapy has not been shown to offer a sur- radiotherapy are not effective or required.
vival advantage in chondrosarcoma. Denosumab, a RANK-ligand inhibitor is now increasingly
commonly being used for the treatment of giant cell
Dedifferentiated chondrosarcoma tumour of bone, particularly in difficult to reach surgical
This is the most malignant cartilagenous tumour and is a locations such as the sacrum. It is given preoperatively
very aggressive tumour subtype. for 3 months followed by surgery. In difficult to reach
Most common locations include the distal and proximal locations it is sometimes given indefinitely although the
femur and the proximal humerus. impact of complications such as osteonecrosis of the jaw,
Cartilage tumours
Enchondroma Osteochondroma Chondrosarcoma
Age (years) Any Any >50

Symptoms Incidental Mechanical Pain
Imaging No change in bone architecture Sessile or pedunculated Bone architecture is altered
No endosteal scalloping or erosion lesion is confluent with Endosteal scalloping and erosion
the intramedullary canal Bone destruction
Soft tissue mass
Pathology Bland cartilage with Mature bone stalk with a Differing degrees of cellular
minimal cellular elements benign, mature cartilage cap atypia and a high rate of
mitotic fugures
Treatment Observation Observation unless mechanical Wide surgical resection
pain is significant
Caveats Pathology may have high Lesions should mature with Chemotherapy is added with
degree of cellularity in the the patient, a cartilage dedifferentiated and mesenchymal
hands and feet and can cap >2 cm requires observation chondrosarcoma
be confused with chondrosarcoma
Syndrome Olliers disease Multiple Hereditary Exostoses (MHE) Olliers disease
association Maffucci syndrome Maffucci syndrome
MHE
Table 8
stress fractures and the impact on female fertility remain often in a characteristically different location from pri-
areas yet to be fully understood. mary osteosarcoma or chondrosarcoma.
Malignant forms: primary and secondary malignant giant e The periosteum may be lifted off in multiple layers,
cell tumours which produces a Codmans triangle and an onion-
With primary malignant giant cell tumour of bone, skin appearance.
a benign giant cell tumour coexists in a high-grade e The soft tissue component is often large.
sarcoma (occurs with about 1% of giant cell tumours) Immunohistochemistry studies reveal CD99 positivity;
e most commonly aneurysmal bone cysts. the classic 11:22 chromosomal translocation produces
Secondary malignant giant cell tumour occurs after the EWS/FLI1 fusion gene.
irradiation to treat a giant cell tumour or after multiple As for primary osteosarcoma treatment involves a multi-
local recurrences. modality approach with multi-agent chemotherapy, irra-
diation, and wide surgical resection.
Ewings sarcoma of bone Standard treatment includes high-dose neo-adjuvant
Diagnosis chemotherapy.
Distinctive small, round cell sarcoma that occurs most Local tumour control may be achieved by irradiation or
often in children and young adults; most affected chil- surgery.
dren are older than 5 years. Major benefits of wide-margin surgical resection are a
When a small blue cell tumour is found in a child decrease in the risk of local recurrence and the avoid-
younger than 5 years, metastatic neuroblastoma and ance of the potential for post-irradiation sarcoma. There
leukemia are the main differential diagnoses. In patients is some controversy in terms of resecting either the pre-
older than 30 years, metastatic carcinoma must also be treatment (pre-chemotherapy) margins of the tumour or
considered. the post-treatment (post-chemotherapy) margins; it is
The most common locations include the pelvis, distal probably safest to opt for as wide a margin as possible.
femur, proximal tibia, femoral diaphysis, and proximal Unlike for osteosarcoma, Ewings tumour is somewhat
humerus. radiosensitive and radiotherapy may be used primarily
Ewings sarcoma usually presents with pain, and for pelvic and spine disease where resection involves
almost uniquely for primary bone sarcomas, systemic difficult to access sites, or as an adjunct to surgery to
symptoms such as fever and night sweats may be maintain function while sparing critical structures.
present. Survival:
Affected patients may demonstrate a raised erythrocyte The rate of long-term survival with multimodal treat-
sedimentation rate, leukocytosis, anemia, and an ment may be as high as 60e70% compared to 10%
elevated white blood cell count. before the advent of multi-agent chemotherapy.
Radiographs often show a large, destructive lesion that e Metastatic disease involves the lungs (50%), bone
involves the metaphysis and diaphysis of the long bone, (25%), and bone marrow (20%).
Poor prognostic factors include the following: Eilber FR, et al. Advances in the treatment of sarcomas of the ex-
e Spine and pelvic tumours tremity: current status of limb salvage. Cancer 1984; 54: 2695e701.
e Tumours larger than 100 cm3 in volume Enneking WF, et al. A system for the surgical staging of musculo-
e A poor response to chemotherapy (less than 90% skeletal sarcoma. Clin Orthop 1980; 153: 106e20.
tumour cell necrosis) Enneking WF, et al. The effect of the anatomic setting on the results of
e Elevated lactic dehydrogenase levels surgical procedures for soft parts sarcoma of the thigh. Cancer
e The P53 mutation and gene fusion products other 1981; 47: 1005e22.
than EWS-FLI 1. Heare TC, et al. Staging techniques and biopsy of bone tumours.
Orthop Clin North Am 1989; 20: 273e85.
Adamantinoma Madewell JE, et al. Radiologic and pathologic analysis of solitary bone
Adamantinoma is a rare low-grade, malignant tumour of lesions. Part I: internal margins. Radiol Clin North Am 1981; 19:
long bones that contains epithelium-like islands of cells 715e48.
The tibia is by far the most common site, although other Mankin HJ, et al. The hazards of biopsy in patients with malignant
long bones are infrequently involved (fibula, femur, ulna, primary bone and soft tissue tumours. J Bone Joint Surg Am 1982;
radius). 64: 1121e7.
Affected patients are usually young adults and experience Miller MD, et al. Malpractice maladies in the management of muscu-
pain over months to years. Many will have been given a loskeletal malignancies. Contemp Orthop 1991; 23: 577e84.
diagnosis of shin splints for some years before the diag- Pritchard DJ, et al. Chondrosarcoma: a clinicopathologic and statis-
nosis is eventually made. tical analysis. Cancer 1980; 45: 149e57.
Radiographic appearance: multiple, sharply circumscribed, Ragsdale BD, et al. Radiologic and pathologic analysis of solitary bone
lucent defects of different sizes, with sclerotic bone inter- lesions. Part II: periosteal reactions. Radiol Clin North Am 1981; 19:
spersed between the zones and extending above and below 749e83.
the lucent zones. Sim FH, et al. Reconstruction of musculoskeletal defects about the
Histologically the cells have an epithelial quality and are knee for tumour. Clin Orthop 1987; 221: 188e201.
arranged in a palisading or glandular pattern; the epithelial Simon MA. Biopsy of musculoskeletal tumours. J Bone Joint Surg Am
cells occur in a fibrous stroma. 1982; 64: 1253e7.
Treatment is usually via wide-margin surgical resection Simon MA. Current concepts review: limb salvage for osteosarcoma.
and reconstruction (although this is controversial). J Bone Joint Surg Am 1988; 70: 307e10.
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diaphyseal location of the tumour and therefore endo- sarcomas. J Bone Joint Surg Am 1986; 68: 1458e63.
prosthetic replacement is less commonly used for this Springeld DS, et al. Surgical treatment for osteosarcoma. J Bone
condition. Biological reconstruction including resection of Joint Surg Am 1988; 70: 1124e30.
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with the same caution as for the other primary bone 60e8.
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FURTHER READING
Bell RS, et al. The surgical margin in soft tissue sarcoma. J Bone Joint
Surg Am 1989; 71: 370e5.

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ORTHOPAEDIC ONCOLOGY

primary bone sarcomas opsy is inconclusive, an open biopsy is of course mandatory

Bone lesions by age Tumourebone interaction (from Lodwick)

Adjuvant therapy Classification of primary tumours of bone and bone

Principles of musculoskeletal biopsy Hematopoietic Myeloma

Multi-agent chemotherapy has dramatically improved

Age (years) <30 and >60 <30 <45

Age (years) Any Any >50

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