GIANT CELL TUMOR

Giant cell tumour of bone

Rare (12 per million of population
per year)
solitary,
locally aggressive lesion seen at the
epiphyseal ends of long bones
(typically distal femur 25%, proximal
tibia 25% and distal radius 10%)
in the third decade
 Giant cell tumour of bone
X-ray shows an aggressive lytic
lesion in the metaphyseal/diaphyseal
part of the bone, usually juxta-
articular.
There is often complete cortical
destruction and an associated soft-
tissue mass
 Giant cell tumour of bone
Histologically,
the tumour comprises spindle-
shaped tumour cells and
multinucleated giant cells which are
indistinguishable from osteoclasts.
 Giant cell tumour of bone
These are recruited from the
monocytemacrophage population.
Agents which interfere with
osteoclast recruitment (e.g.
bisphosphonates and Rank-ligand
inhibitors) may be helpful in difficult
cases.
 Giant cell tumour of bone
This lesion needs to be distinguished
from giant cell-rich osteosarcoma
Treatment is by curettage and
grafting and the use of a surgical
adjuvant (e.g. high-speed burr, liquid
nitrogen, cement).
Local recurrence rates can be high,
particularly in difficult sites such as
the pelvis, spine and distal radius
 Giant cell tumour of bone
Malignant transformation and
metastasis is a rare but well
recognized complication.
Locally recurrent tumours are
associated with a higher risk of
metastatic disease.
 Pathology
Giant-cell tumour is a lesion of uncertain
origin that appears after the end of bone
growth, most commonly in the distal
femur, proximal tibia, proximal humerus
and distal radius.
The tumour has a reddish, fleshy
appearance; it comes away in pieces
quite easily when curetted but is difficult
to remove completely from the
surrounding bone.
 Pathology
Aggressive lesions have a poorly defined
floor and appear to extend into the
surrounding bone.
Histologically the striking feature is an
abundance of multinucleated giant cells
scattered on a background of stromal cells,
with little or no visible intercellular tissue.
About one-third of these tumours remain
truly benign; one-third become locally
invasive and onethird metastasize.
 Clinical features
The patient is usually a young adult
who complains of pain at the end of a
long bone; sometimes there is slight
swelling.
Pathological fracture occurs in 1015
per cent of cases.
 Imaging
Although this is a solid tumour, it appears
on x-ray as a cystic (i.e. radiolucent)
area situated eccentrically at the end of a
long bone.
Unlike any of the other cystic lesions, it
always extends right up to the
subchondral bone plate.
The endosteal margin is usually clear-cut,
but in invasive lesions it is illdefined.
 Imaging
Considering the tumours potential for
aggressive behaviour, detailed staging
procedures are essential.
CT scans and MRI will reveal the extent of
the tumour, both within the bone and
beyond.
It is important to establish whether the
articular surface has been broached;
arthroscopymay be helpful.
Biopsy is essential.
 Treatment
Well-confined, slow-growing lesions
with benign histology can safely be
treated by thorough curettage and
stripping of the cavity with burrs
and gouges, followed by swabbing
with hydrogen peroxide or by the
application of liquid nitrogen; the
cavity is then packed with bone
chips.
 Treatment
More aggressive tumours, and
recurrent lesions, should be treated
by excision, followed, if necessary, by
bone-grafting or prosthetic
replacement.
 Benign form
Distinctive neoplasm that has poorly
differentiated cells Benign but
aggressive
Confusion in diagnosis results
from the fact that in rare cases
(<2%), this benign tumor
metastasizes to the lungs (benign
metastasizing giant cell tumor).
 Most common in the
epiphysis and
metaphysis of long
bones, and about
50% of lesions occur
about the knee; the
vertebra, sacrum,
and distal radius are
involved in about
10% of cases (Figure
9-40).
 The sacrum is the most common axial
location of giant cell tumors of bone.
Unlike most bone tumors, which occur
more often in boys and men, giant cell
tumors are more common in girls and
women.
They are uncommon in children with open
physes.
Manifest with pain that is usually
referable to the joint involved
 A purely lytic
destructive
lesion in the
metaphysis
that extends
into the
epiphysis and
often borders
the
subchondral
bone (Figure
9-41)
 Early in the symptomatic phase, the
radiographs may appear normal; a
small lytic focus is difficult to detect.
Basic proliferating cell has a round
to oval or even spindle-shaped
nucleus (giant cells appear to have
the same nuclei as the
proliferating mononuclear cells).
Mitotic figures may be numerous.
 Giant cell tumors may
undergo a number of
secondary
degenerative
changes, such as
aneurysmal bone cyst
formation, necrosis,
fibrous repair, foam
cell formation, and
reactive new bone
(Figure 9-42).
 Treatment is aimed at removing the lesion,
with preservation of the involved joint.
Extensive exteriorization (removal of a large
cortical window over the lesion)
Curettage with manual and power instruments
Chemical cauterization with phenol
Area of defect is usually reconstructed with
subchondral bone grafts, methylmethacrylate,
or both.
Local control with this treatment regimen has
a success rate of 85% to 90%.
2. Malignant forms:
Primary and secondary malignant giant cell
tumors
With primary malignant giant cell tumor of
bone, a benign giant cell tumor coexists
with a high-grade sarcoma (occurs with
about 1% of giant cell tumors).
Secondary malignant giant cell tumor
occurs after irradiation to treat a giant cell
tumor or after multiple local recurrences