ORTHOPAEDIC ONCOLOGY

Management of benign Classification of tumour by histological subtype

bone tumours Cell type Tumour subtype

Chondrogenic Osteochondroma
Jonathan R Perera Chondromas: enchondromas,
Asif Saifuddin periosteal chondroma
Chondroblastoma
Rob Pollock
Chondromyxoid fibroma
Bizarre parosteal
osteochondromatous proliferation
Abstract Osteogenic Osteoma (enostosis/bone island)
Benign bone tumours are rare, occurring most commonly in skeletally Osteoid osteoma
immature patients, and usually arising from cartilage or bone. The Osteoblastoma
commonest locations are the distal femur, proximal tibia and proximal Fibrogenic Desmoplastic fibroma of bone
humerus. They present with pain, swelling or pathological fracture. Fibrohistiocytic Non-ossifying fibroma
Diagnosis is by plain radiographs, MRI and core needle biopsy if indi- Fibrous cortical defect
cated. More aggressive tumours may appear radiologically similar to Osteoclastic Giant cell tumour
malignant tumours. Management depends upon anatomical location, Vascular Haemangioma
symptoms, morbidity of treatment and the natural history of the Lipogenic Intra-osseous lipoma
tumour. In most cases it involves either simple excision or curettage, Tumours of undefined Aneurysmal bone cyst
although occasionally it is necessary to perform a complete excision neoplastic nature Simple bone cyst
using the same principles as for malignant tumours. Fibrous dysplasia
Keywords benign bone tumour; non-neoplastic tumour-like Osteofibrous dysplasia
conditions of bone
Table 1

Introduction an incidental finding on radiography in the case of a latent lesion,

Primary bone neoplasms are extremely rare, accounting for only
0.2% of human tumours.1 The majority are benign and typically
affect the skeletally immature patient but some are difficult to
distinguish from malignant lesions, have a significant incidence
of local recurrence and may undergo malignant transformation.
Diagnosis and treatment of bone tumours is complex and man-
agement of this group of patients is best undertaken at specialist
centres in a multidisciplinary (MDT) setting.
Classication
Two classification systems are commonly used (Tables 1 and 2).
The first is histological, based on the cell of origin. The second is
more clinically orientated and based on the pattern of behaviour
of the tumour.2
While tumours may arise from chondrocytes, osteoblasts,
osteoclasts, or soft tissue within bone such as fat, fibrous tissue
or smooth muscle, some fall into the tumours of undefined
neoplastic nature group and their pathogenesis is unclear. Their
behaviour dictates their clinical presentation, varying from being
Jonathan R Perera BSc (Hons) MBBS MRCS, Specialty Trainee Level
Seven, North-East London RNOH rotation, UK. Conicts of interest:
none declared.
Asif Saifuddin BSc (Hons) MBChB MRCP FRCR, Consultant
Musculoskeletal Radiologist, UK. Conicts of interest: none declared.
Rob Pollock MBBS BSc FRCS (Tr&Orth), Consultant Orthopaedic
Surgeon & Sarcoma Lead, Royal National Orthopaedic Hospital,
Stanmore, UK. Conicts of interest: none declared.
to a rapidly growing, painful lesion associated with functional
loss in the case of an aggressive lesion. This is reflected in
Ennekings classification (Table 2).2
Aetiology
The aetiology of the vast majority of benign bone tumours is un-
clear. Numerous theories have been proposed but none has ever
been substantiated apart from multiple hereditary exostoses (MHE),
which is also known as diaphyseal aclasis. MHE is inherited as an
autosomal dominant condition. Three genes are responsible,
namely the exostosin-1/2/3 (EXT-1,2,3) genes found at chromo-
some 8q24 (EXT-1) loci, chromosome 11p11-12 (EXT-2) loci and the
short arm of chromosome 19 (EXT-3), although its exact location
has yet to be precisely determined. Approximately 15% of patients
with osteochondromas have the inherited form of the condition.
Clinical features
The clinical features are non-specific and variable. Some long-
standing lesions present as incidental findings. For example, an
adolescent who attends the A&E department after a knee injury
and a fibrous cortical defect is found on radiography (Figure 1).
Some tumours such as osteochondromas present with a long
history of a painless swelling, but more aggressive lesions such
as giant cell tumours (GCT) usually present with a short history
of pain, swelling and loss of function.
Investigation
As some benign bone tumours can be difficult to distinguish from
malignant lesions, thorough and well-considered investigation

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Classification of tumour by biological behaviour according to Enneking2

Classification Behaviour Example of tumour

Latent Slow growth with spontaneous healing. Often Fibrous cortical defect
an incidental finding on X-ray. No treatment Non-ossifying fibroma
required. Osteoma
Active Progressive growth over time and usually Chondromyxoid fibroma
symptomatic. Treatment of choice; curettage. Enchondroma
Low incidence of local recurrence. Simple bone cyst
Aggressive Rapid growth of tumour often extending Chondroblastoma osteoblastoma
beyond periosteum into the soft tissues. Giant cell tumour aneurysmal bone cyst
Treatment of choice curettage or excision. 10
e15% chance of local recurrence

Table 2

should be undertaken in the MDT setting, in a specialist Management

musculoskeletal tumour centre.3 The absolute minimum includes
General principles
an adequate history, examination, and imaging by radiography,
Treatment is dependent upon many factors, particularly:
MRI and occasionally CT and ultrasound.
 patient symptoms
The radiological features may be so clear that if compatible
 natural history of the tumour
with the clinical picture, a histological diagnosis may not be
 morbidity of treatment.
necessary prior to definitive treatment. However, if there is any
Treatment varies from simple observation with repeat imag-
doubt about the diagnosis or worrying features on imaging or
ing, through to wide excision using the same surgical principles
examination, a tissue diagnosis should be obtained. This is best
as for malignant tumours. When treating bone tumours, the
achieved by image-guided percutaneous bone biopsy using a
surgeon has to balance excision margin against function. With
Jamshidi needle, with the biopsy route agreed between the
wider margins, there may be greater functional loss but a
surgeon and radiologist at the MDT.
reduced risk of local recurrence. Conversely, intra-lesional sur-
gery has less morbidity but a greater risk of local recurrence.

Non-operative
Asymptomatic lesions that fall into the Enneking latent group of
tumours can simply be observed, for example the natural history
of lesions such as non-ossifying fibromas is well-documented
and predictable, and it is safe to leave these alone. If the diag-
nosis has been made on imaging alone and non-operative treat-
ment has been chosen, then histological confirmation of the
diagnosis will not be available. Therefore, it is advisable to repeat
the plain radiograph after 3e6 months to ensure that the lesion is
showing no signs of progression. If clinical or radiological pro-
gression does occur, then there must be a low threshold for
biopsy.

Figure 1 Antero-posterior radiograph of the proximal tibia showing a
small brous cortical defect (arrow) in the medial tibial metaphyseal cortex.
Curettage
Curettage is the treatment of choice for the majority of benign
bone tumours requiring surgical intervention, and is by defini-
tion intra-lesional surgery. The intention is to achieve macro-
scopically clear margins, accepting that microscopic disease is
likely to be left behind.
The technique involves exposing the affected bone and
creating a bone window with osteotomes. The window needs to
be big enough to obtain an adequate view of the tumour but
small enough to ensure that the graft material can be contained
within the bone at the end of the procedure. Ideally, a range of
curettes with differing angles and head sizes should be used. In
addition, it is now standard practice to skim the edge of the
cavity with a high-speed burr to reduce the risk of local

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 ORTHOPAEDIC ONCOLOGY

recurrence. It is also helpful to use an image intensifier per-

operatively to ensure that the curettage has cleared the entire
lesion.
After curettage, dependant upon the histology of the tumour,
its anatomical location, the age of the patient, the likelihood of
local recurrence and pathological fracture, the resulting cavity
may be left unfilled or filled with iliac crest autograft, morcellized
allograft, synthetic bone substitute or polymethylmethacrylate
(PMMA) cement. Additionally, the curetted bone can be
augmented with internal fixation to prevent a pathological frac-
ture (Figure 2a and b).
After curettage, some tumours have a higher incidence of
local recurrence, such as GCT, osteoblastoma, chondroblastoma
and aneurysmal bone cyst. When treating these, it has been
shown that using some form of adjuvant treatment reduces the Figure 3 (a): Antero-posterior (AP) radiograph of the right knee
risk of local recurrence.4 These include treating the cavity wall showing a giant cell tumour of the distal femur (arrows) (Campanacci
with a high-speed burr, phenol, hydrogen peroxide or liquid ni- type 2). (b): Post-operative AP radiograph following curettage and
trogen, and filling the defect with PMMA producing a cemen- cementation (arrows).
toma (Figure 3a and b). Which of these adjuvants is used
depends upon the surgeons preference, but the incidence of local
recurrence after curettage of these more aggressive tumours is 10
e15% even with adjuvant treatment.

Curettage practice points

 Intra-lesional surgery leaving residual microscopic disease
 Image intensifier ensures macroscopic clearance of tumour
 Adjuvant treatment to cavity reduces incidence of local
recurrence
 Choice of filler depends on histology, location of tumour,
age of patient and risk of fracture
 Internal fixation strengthens construct and helps prevent
pathological fracture
Radiofrequency ablation (RFA)
RFA is the technique of choice for small, symptomatic lesions
less than 1.5 cm in diameter, classically osteoid osteomas
(Figure 4a).
RFA causes targeted thermal destruction of bone and under
general anaesthesia and CT guidance, a Bonopty needle is
inserted percutaneously into the centre of the lesion (Figure 4b).
Figure 4 (a): Sagittal CT multiplanar reconstruction of the tibial
diaphysis showing an osteoid osteoma nidus (arrow). (b): Axial CT
demonstrating placement of the radiofrequency probe.
The radiofrequency electrode is then passed down the needle
and its position checked. When the device is switched on, heat is
generated at the tip of the wire using radiofrequency energy. The
target temperature is 80e90 C and the duration of treatment is
typically 5e10 minutes. This produces a burn radius of 5e6 mm
around the active length of the treatment needle. For lesions greater
than 1 cm two probes can be used which are appropriately sited in
the tumour for an adequate coverage of the entire tumour volume.
The advantage of RFA is that the tumour is treated with
minimal destruction of surrounding bone, and it also permits
treatment of tumours in surgically inaccessible locations with
minimal morbidity. Occasionally, when the tumour is closely
related to vital structures such as the spinal cord or neuro-
vascular bundle, a safe treatment cannot be undertaken unless
combined with injection of saline at the interface between the
tumour margin and the anatomical structure potentially at risk of
thermal injury. A potential disadvantage is that a tissue diagnosis
is rarely obtained, but the diagnosis is typically clear-cut both
clinically and radiologically.

Excision and reconstruction

Figure 2 (a): Antero-posterior (AP) radiograph of the right hip showing
brous dysplasia of the proximal femur (arrows) with a minimally
displaced pathological fracture (arrowhead). (b): Post-operative AP
radiograph following curettage, bone grafting and internal xation.
Complete excision of a benign bone tumour is indicated when the
tumour is particularly aggressive. This may manifest as a poorly
defined margin, bone expansion, cortical destruction, extra-
osseous extension, pathological fracture or combinations of the

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 ORTHOPAEDIC ONCOLOGY
above. If the tumour is sub-articular, there may be no alternative
but to sacrifice the joint.
The surgical principles are similar to those used when treating
a malignant bone tumour. The intention is to perform a marginal
but complete en bloc excision and remove both macroscopic and
microscopic disease. After a section of bone has been excised,
some form of reconstruction will be necessary, which may be a
biological reconstruction such as a vascularized fibular graft
(Figure 5a and b), or a massive endoprosthesis.
The reconstruction used is dependent upon many factors,
particularly anatomical location, age, functional requirements of
the patient and the individual surgeons preferences.
Figure 5 (a): Antero-posterior (AP) radiograph of the wrist showing a
Giant cell tumour of the distal radius (arrows) (Campanacci type 2).
(b): Post-operative AP radiograph following resection and
reconstruction with a bular graft (arrows) and plate.
Figure 6 (a): Antero-posterior radiograph of the knees showing multiple osteochondromata (arrows) in a patient with diaphyseal aclasis.
(b): Sagittal T2 weighted MRI of the knee showing a distal femoral osteochondroma (arrow) with a thin cartilage cap (arrowhead).
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Management of specic tumours
Cartilage tumours
Osteochondroma (synonym: exostosis): osteochondromas are
cartilage-capped bony projections with a marrow cavity that is
continuous with the underlying bone. They account for approxi-
mately 35% of all benign bone tumours and present within the first
three decades of life.5 Most occur close to the physis of a long bone
but they can also arise from flat bones such as the scapula and
ilium. The commonest sites are the distal femur and proximal tibia,
followed by the proximal humerus and proximal fibula. Symptoms
are most commonly mechanical and caused by impingement of the
lesion on nearby tendons, especially around the knee.
In approximately 15% of cases they are multiple, a condition
known as diaphyseal aclasis (Figure 6a), which in most cases is
familial and inherited as an autosomal dominant, but sporadic
cases are well recognized. Malignant transformation of a solitary
osteochondroma to a peripheral chondrosarcoma occurs in
approximately 1% of cases and in up to 3% of patients with
diaphyseal aclasis.5 The warning signs include rapid increase in
size of an osteochondroma associated with increasing pain. Of
particular concern are pelvic osteochondromas that may be large
and silent until they transform. If there is any suspicion of ma-
lignant transformation, the osteochondroma should be imaged
with MRI and the cartilage-cap assessed (Figure 6b). A cartilage
cap greater than 1 cm in an adult suggests a high probability of
malignant transformation, usually to grade 1 chondrosarcoma.
Surgical excision of osteochondromas is usually curative, but
it is important to excise the cartilage cap in its entirety or local
recurrence is likely. In patients with diaphyseal aclasis it is
impractical to excise every osteochondroma and surgery is
reserved for those which are symptomatic, unsightly or which
are suspicious for malignant transformation.
Osteochondroma practice points
 Commonest benign bone tumour
 Most commonly seen in the metaphyseal region of long
bones
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 ORTHOPAEDIC ONCOLOGY
 Distal femur and proximal tibia are the commonest sites
 15% of cases are multiple (MHE/diaphyseal aclasis-auto-
somal dominant inheritance)
 1% risk of malignant transformation in solitary cases (3%
if multiple)
 Surgical excision if symptomatic, unsightly or suspicious
for malignant transformation
Enchondroma
Enchondromas are benign, intramedullary cartilage neoplasms
which account for approximately 10e25% of all benign bone
tumours and present at any age.5 They mainly affect the long
bones and are most commonly solitary. The hands and feet are
usually affected, followed by the proximal humerus, proximal
and distal femur and proximal tibia.
While enchondromas in large long bones such as the humerus
or femur may be asymptomatic and discovered incidentally, the
most common presentation is with a palpable swelling in the
hands or feet. Pain may or may not be present, and some are
associated with pathological fracture.
Radiologically, enchondromas of the hands and feet appear as
well-defined, lytic lesions exhibiting punctate matrix minerali-
zation, and may be associated with bone expansion (Figure 7a).
They are usually active on bone scintigraphy. In larger bones,
lobular chondral tissue is seen in the metaphyseal region.
Lesions greater than 5 cm in length and causing endosteal
scalloping should be considered as possible low-grade chon-
drosarcomas. The MRI features are classical (Figure 7b) and
allow a safe diagnosis without the requirement for needle biopsy.
The majority of enchondromas are successfully treated with
curettage and the local recurrence rate is extremely low.
Olliers disease and Maffucci syndrome
Olliers disease is a developmental disorder characterized by
multiple enchondromas in the long bones of the hands, feet and
limbs (Figure 8a). When associated with soft tissue or visceral
Figure 7 (a): Antero-posterior radiograph of the index nger showing
an enchondroma (arrow) in the middle phalanx. (b): Coronal T2
weighted MRI of the distal tibia showing a heavily mineralized
chondroma (arrows).
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Figure 8 (a): Antero-posterior radiograph of the right hand showing
multiple enchondromata (arrows) in a patient with Olliers disease. (b):
Coronal T1 weighted MRI of the foot showing multiple enchondromata
(arrows) and a soft tissue haemangioma (arrowheads) in a patient with
Maffucci syndrome.
haemangiomas, the condition is known as Maffucci syndrome
(Figure 8b).
The aetiology is unclear, most cases being sporadic rather
than inherited. The condition usually presents in early childhood
with lumps in the hands and feet, limb deformity and/or multiple
pathological fractures.
Diagnosis is based on the clinical picture and radiographic
appearances. Treatment is aimed at maintaining function, pre-
venting deformity and careful surveillance in order to pick up
malignant transformation early. The incidence of malignant
transformation to chondrosarcoma is approximately 15e30% in
patients with Olliers and much greater in those with Maffuccis.6
Chondroblastoma
Chondroblastoma is a cartilage-producing tumour typically arising
in the epiphysis of skeletally immature patients. There is a slight
male preponderance. About 75% occur in long bones and the
commonest sites are the proximal and distal femur, the proximal
tibia and the proximal humerus.7 Symptoms vary from mild pain
of many years duration to recent onset of severe pain. Clinically,
patients may develop an effusion in the hip or knee associated with
stiffness.
Radiographs show a well-defined, lytic lesion within the
epiphysis (Figure 9a) and MRI demonstrates a sharply demar-
cated chondral lesion with surrounding bone and soft tissue
oedema, as well as reactive joint effusion (Figure 9b). Core
needle biopsy will confirm the diagnosis. Curettage is the treat-
ment of choice and is successful in 80e90%.
Chondromyxoid broma
This is one of the least common bone tumours. It most
commonly affects the long bones, particularly around the knee,
of patients in their second and third decades. The clinical pre-
sentation is very similar to chondroblastoma and the treatment
and prognosis is also the same.
Osseous tumours
Osteoid osteoma
This is a small, bone-forming tumour which can occur in any
bone but is most commonly seen in the femur and tibia. It
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 ORTHOPAEDIC ONCOLOGY

Figure 9 (a): Antero-posterior radiograph of the knee showing a proximal tibial chondroblastoma (arrows). (b): Coronal short T1 inversion recovery
MRI of the knee showing a chondroblastoma (arrows) with associated marrow oedema (arrowhead).

usually affects skeletally immature patients, but it is also seen in

young adults. Histologically, the features are the same as an
osteoblastoma.8
The clinical presentation is classical with a history of con-
stant, unremitting pain often worse at night, which usually re-
sponds to aspirin and non-steroidal anti-inflammatory drugs
(NSAIDs). The site of the lesion often determines the clinical
findings; in the spine it may cause scoliosis, in the fingers it may
cause significant swelling and loss of function and if the lesion is
sub-articular, it may result in a joint effusion. The differential
diagnosis includes Brodies abscess, stress fracture or a subtle
area of osteomyelitis.
The lesion may be too small to biopsy and the diagnosis is
typically made on the basis of clinical presentation and imaging.
The findings of sclerosis on plain X-ray, high uptake on bone
scintigraphy or single photon emission computed tomography
(SPECT), a nidus on CT (Figure 4a) and the presence of oedema
on MRI are enough to make the diagnosis. The treatment of
Figure 10 Axial CT of the chest wall showing an osteoblastoma of the
choice is percutaneous, CT-guided radiofrequency ablation as
rib (arrow).
described above.4 The success rate in most series is 90e95%
after a single treatment, and 100% after two treatments. The
complication rate is extremely low.9
Giant cell tumour
Osteoblastoma GCTs are locally aggressive lesions that account for approxi-
This is a rare, bone-forming tumour that typically affects young mately 20% of all benign bone tumours. They occur in adults,
adults aged 10e30 years. It is twice as common in males and typically between the ages of 20e40 years with a slight female
over 50% affect the posterior elements of the spine, the pelvis or preponderance. They affect the metaphyseal regions of long
the sacrum. In the remainder of the skeleton, the proximal and bones, typically extending to the articular surface and are com-
distal femur and the proximal tibia are the commonest sites.10 monest in the distal femur, proximal tibia, proximal humerus
The clinical features are similar to those of an osteoid osteoma and distal radius. About 5% of GCTs affect the flat bones,
(i.e. chronic pain). Imaging reveals a well-defined, purely lytic or especially those of the pelvis.
mineralized lesion measuring greater than 2 cm in diameter Patients usually present with a short history of pain,
(Figure 10). Some osteoblastomas are associated with aneu- swelling and functional loss. Plain radiographs show a lytic
rysmal bone cyst (ABC) change. lesion with a narrow zone of transition extending to the sub-
Osteoblastoma can occasionally be hard to differentiate from articular surface. MRI reveals a haemosiderin-rich lesion with
osteoblastic osteosarcoma and therefore, needle biopsy is rec- low-to-intermediate signal on T1-weighted images and hetero-
ommended before definitive treatment is carried out. The geneous increased signal intensity on T2-weighted images
treatment of choice is curettage and cementation. The prog- (Figure 11a). Campanacci classified GCTs according to their
nosis is good and the incidence of local recurrence after radiological appearance.11
curettage is low.

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 ORTHOPAEDIC ONCOLOGY
Figure 11 (a): Coronal T1 weighted MRI of the knee showing a prox-
imal tibial giant cell tumour (GCT) (arrow) containing areas of low signal
due to haemorrhage. (b): Antero-posterior radiograph of the knee
showing a proximal tibial GCT (arrows) (Campanacci type 3).
Type 1 or quiescent tumours have a well-defined margin,
surrounding sclerosis and no cortical involvement. These can
be asymptomatic and are therefore rarely seen.
Type 2 or active tumours lack surrounding sclerosis and
demonstrate cortical expansion but are still well-defined
(Figure 3a).
Type 3 or aggressive tumours have ill-defined margins,
show cortical destruction and extend into the soft tissues
(Figure 11b).
The differential diagnosis of GCT includes brown tumours of
hyperparathyroidism and aneurysmal bone cyst, and therefore
needle biopsy is essential. The treatment of GCTs depends on
the anatomical location, the aggressiveness of the tumour
clinically and radiologically, and the functional expectations of
the patient. If the joint can be saved, the initial treatment of
choice is curettage, some form of adjuvant treatment, e.g. high-
speed burring and cementation with PMMA (Figure 3b). If the
lesion is very destructive, i.e. Campanacci type 3, or if there is a
pathological fracture or the joint is involved, then wide excision
may be necessary. Following wide excision, tumours around
the knee are best reconstructed with a massive endoprosthesis
to allow immediate weight bearing and early recovery of
function. Tumours around the wrist are better treated with a
vascularized fibular graft, with or without wrist fusion12
(Figure 5b).
The medical management of GCTs has significantly pro-
gressed since the discovery of osteoclast signalling pathways in
the late 20th century, leading to the development of new medi-
cations. Receptor activator of nuclear factor kappa-B (RANK) and
RANK ligand (RANKL) altering medications such as denosumab
are extremely important for treatment. It acts as a RANKL
antagonist, leading to the loss of osteoclasts from bone surfaces
and provides excellent medical treatment for GCT.13
Even though the reports of denosumab in the treatment of
GCT are encouraging, more studies and long-term follow-up are
required. Also, indications for the use of denosumab in the
treatment of GCTs are still not clearly defined.
The incidence of local recurrence after curettage in most
series is 10e15% and usually occurs within the first 2
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years.4,12,14 It manifests radiologically as the development of
lysis around the cementoma. Some studies have suggested that
bisphosphonates post-operatively may reduce the incidence of
local recurrence.15
Despite GCT being histologically benign, pulmonary metas-
tases are seen in up to 2% of patients, occurring on average 3e4
years after diagnosis. Most metastases grow slowly, some regress
and some progress leading to death. However, true malignant
transformation of a GCT is extremely rare.
GCT practice points
 Locally aggressive
 Distal femur, proximal tibia, proximal humerus, distal
radius
 Differential diagnosis ABC, brown tumour of
hyperparathyroidism
 Campanacci types 1, 2 and 3
 Treat with curettage and some form of adjuvant
 10e15% local recurrence rate
 Bisphosphonates may reduce rate of local recurrence
 The role of RANKL antagonists, e.g. denosumab, is unclear
Miscellaneous lesions
Non-ossifying broma/brous cortical defect
By convention, a fibrous cortical defect (FCD) measures less than
1 cm in diameter (Figure 1) and a non-ossifying fibroma (NOF) is
larger than 1 cm, but histologically these two conditions are
identical. Their aetiology is unclear. They fall into the latent
category by Ennekings classification and are most commonly
seen as incidental findings in skeletally immature patients. They
are rarely symptomatic and can be safely observed. Occasionally,
a large NOF can be complicated by a stress or pathological
fracture in the adjacent bone (Figure 12), in which case curettage
and internal fixation may be indicated.
Simple bone cyst (synonym: unicameral bone cyst)
Simple bone cysts (SBC) are fluid-filled, metaphyseal cysts of
unclear aetiology. They are most commonly seen in the first two
decades of life with a M:F ratio of 3:1. The proximal humerus and
proximal femur account for 90% of cases.16 Most patients pre-
sent with a pathological fracture after minimal trauma. Radio-
graphs are diagnostic and show a well-defined, lytic metaphyseal
lesion often extending into the diaphysis but only occasionally
involving the epiphysis. The cortex is usually thin and may be
mildly expanded. If a fracture is present, a fallen fragment sign
may be seen (Figure 13).
Numerous methods of treating SBCs have been described,
including aspiration and injection with steroid, injection with
bone marrow, inserting a cannulated screw to decompress the
cyst, fixation with Nancy nails and curettage combined with in-
ternal fixation.17 Even after curettage, recurrence rates of 10
e20% are reported. By the time the patient is skeletally mature
the cyst will have healed. The treatment modality chosen de-
pends partly on the anatomical location of the cyst. Cysts in
weight-bearing bones such the femoral neck are more frequently
curetted, grafted and internally fixed than those in non-weight
bearing bones.
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Aneurysmal bone cyst

Aneurysmal bone cysts (ABC) are multilocular, expansile,
aggressive, destructive lesions that may expand into the soft
tissues. They are most common in the first two decades of life
and typically arise eccentrically in the metaphyses of long bones.
They can arise de novo or can complicate other benign tumours
such as chondroblastoma, fibrous dysplasia, GCT and NOF,
which are then described as showing secondary ABC change.
Most patients present with a short history of pain and
swelling. The characteristic radiological findings are of an
eccentric, expansile, well-defined lytic lesion with associated
laminated periosteal response (Figure 14a), while MRI classically
demonstrates multiple fluidefluid levels (Figure 14b). The dif-
ferential diagnosis includes GCT and telangiectatic osteosar-
coma. Biopsy should be attempted, although frequently only
blood clot is obtained. It is essential that a non-diagnostic biopsy
is discussed at a bone sarcoma MDT meeting to avoid the po-
tential disastrous curettage of a telangiectatic osteosarcoma.
The majority of ABCs are suitable for curettage and grafting.
They can be extremely vascular and it is advisable to perform
arteriography and embolization pre-operatively in lesions where a
tourniquet cannot be used. Local recurrence occurs in approxi-
mately 25% and is usually apparent within a year of surgery.18
ABCs at the aggressive end of the spectrum may require mar-
ginal but complete excision. Those in difficult locations and sur-
gically morbid areas such as the pelvis or sacrum can be treated in
a novel way by using CT-guided intraosseous injection of a
tetracycline antibiotic, doxycycline. The results show a 95%
Figure 12 Antero-posterior radiograph of the knee showing a proximal
success rate at 2-year follow-up. However, similar to denosumab
tibial non-ossifying broma (arrow) with a minimally displaced patho-
logical fracture (arrowhead).
use in GCT, the use of doxycycline for ABC is a relatively new
treatment and further clinical trials are underway.19

Fibrous dysplasia
Fibrous dysplasia (FD) is a tumour of uncertain neoplastic and
may affect any bone. Clinical presentation with pain, deformity

Figure 14 (a): Antero-posterior radiograph of the knee showing a prox-

Figure 13 Antero-posterior radiograph of the shoulder showing a imal bular aneurysmal bone cyst (ABC) (arrows). (b): Sagittal T2 weighted
fractured proximal humeral simple bone cyst containing a fallen MRI of the wrist showing a distal ulnar ABC containing uideuid levels
fragment (arrow). (arrowheads).

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10.1016/j.mporth.2017.03.008
 ORTHOPAEDIC ONCOLOGY
Figure 15 Lateral radiograph of the lower limb showing osteobrous
dysplasia in the anterior tibial cortex (arrows).
or pathological fracture can occur at any age. It may be mono-
stotic or polyostotic. McCune-Albright syndrome comprises pol-
yostotic FD, pigmented skin lesions resembling the coast of
Maine and endocrinopathy. The association with soft tissue
myxomas is termed Mazabrauds syndrome.
Radiographic appearances are often diagnostic, showing a
ground glass appearance of the affected bone (Figure 2a). ABC
change can occur, resulting in a lytic appearance. Treatment is
aimed at symptom control, and bisphosphonates have been
shown to be effective in reducing pain.13 Surgery is reserved for
pathological fracture (Figure 2b) or severe deformity.
Osteobrous dysplasia
Osteofibrous dysplasia (OFD) is a slow growing fibro-osseous
lesion classically affecting the anterior tibial diaphyseal cortex
(Figure 15). It is most commonly seen in skeletally immature
males, and presentation varies from an incidental finding to a
painful deformity. It is usually self-limiting and spontaneous
healing often occurs. The differential diagnosis includes ada-
mantinoma, a low-grade malignant lesion with similar radio-
logical appearances. Indeed, there is controversy as to whether
OFD has the potential to transform into adamantinoma, or
whether the two are separate entities.
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Conclusions
Benign bone tumours vary greatly in their clinical presentation
and degree of aggressiveness, and therefore management options
also vary significantly from simple clinical and radiological
observation to endoprosthetic replacement. The requirement for
patient care in the setting of a specialist musculoskeletal sarcoma
MDT is essential for optimal outcome. A
Research directions
C Phase 2 clinical trials studying the use of denosumab in the
management of giant cell tumours. This agent is a monoclonal
antibody that targets the receptor activator of nuclear factor
kappa-B ligand, part of the signalling pathway that stimulates
osteoblasts
C Phase 2 clinical trials studying the use of doxycycline in the
management of aneurysmal bone cysts
C Use of novel bone substitutes to promote bone healing after
curettage
C Molecular genetic studies to identify genes and chromosomal
aberrations responsible for these tumours
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Please cite this article in press as: Perera JR, et al., Management of benign bone tumours, Orthopaedics and Trauma (2017), http://dx.doi.org/
10.1016/j.mporth.2017.03.008