European Journal of Radiology 77 (2011) 3–5

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European Journal of Radiology

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A strange giant cell tumor

Marco Gambarotti a,∗ , Maurizio Donato a,b , Marco Alberghini a , Daniel Vanel a
a
Musculoskeletal Anatomical Pathology Department, Istituto Ortopedico Rizzoli, Via di Barbiano 1/10, 40136, Bologna, Italy
b
Department of Anatomical Pathology Hospital Dr. R.A. Calderón Guardia, Universidad De Costa Rica, Formation Scholarship of Instituto Costarricense Contra El Cáncer, Fellowship
P.O. Box 6098-1000 San José, Costa Rica

a r t i c l e i n f o

Article history:
Received 14 June 2010
Accepted 15 June 2010

Keywords:
Giant cell rich osteosarcoma
Bone tumors
Osteosarcoma

A 29-year-old male came in 2008 for pain in the knee for 2
months, without swelling. Radiographs (Fig. 1a and b) and CT
(Fig. 1c) revealed an osteolytic lesion of the distal metaphysis of
the left femur. A CT-guided biopsy was performed.
Histological diagnosis was giant cell tumor (Fig. 1d). The patient
underwent a curettage.
A diagnosis of giant cell tumor with abundant bone produc-
tion and histological aggressive features was made. Three months
later the patient developed a recurrence, with well limited lytic
lesions at the border of the cement, easily detected on CT (Fig. 2a).
After a second CT-guided biopsy an histological diagnosis of recur-
rence of giant cell tumor of bone was made. However, some
features raised the suspicion of malignancy (atypical spindle cells,
abundant bone production). For this reason a surgical resection
of the distal femur was performed. On the resected specimen
there were 2 brownish and soft lesions around the cement, and
a third grayish and lardaceous lesion in the distal part of the femur
and in the soft tissues around the bone. An histological diagno-
sis of giant cell-rich osteosarcoma was finally made (Fig. 2b). The
patient received adjuvant chemotherapy and is free of disease until
now.
1. Discussion
Giant cell rich-osteosarcoma is a rare entity, whose incidence
varies from 0.3% to 13% of all osteosarcomas [1–3]. Histologically it
consists of a proliferation of atypical stromal cells, with an abun-
∗ Corresponding author.
E-mail address: marco.gambarotti@ior.it (M. Gambarotti).
dance of osteoclast-like giant cells. Osteoid is usually focally found.
It can mimic other giant cell-rich lesions, like giant cell tumor of
bone. In particular when a giant cell-rich osteosarcoma with min-
imal atypia is located in a bone extremity the diagnosis can be
extremely difficult [4].
The peak incidence is in the third decade, and it represents a sub-
type of conventional osteosarcoma. Many osteosarcomas contain
osteoclast-like giant cells, which occasionally form clusters, in an
otherwise usual osteosarcoma. The term osteoclast-rich osteosar-
coma is reserved for those osteosarcomas that contain abundant
osteoclast-like giant cells distributed throughout the tumor [5]. At
low power view, these lesions show multinucleated giant cells sim-
ulating a giant-cell tumor; but on high power cytologic anaplasia
of the stromal cells and malignant osteoid production can usually
be identified [4,6,7].
The main differential diagnosis is a giant cell tumor, especially
when the lesion involves the epiphysis of bones. It is particularly
difficult when the stromal cells in the background show slight
degree of anaplasia. Fortunately, the vast majority of osteosarco-
mas are metaphyseal or metadiaphyseal, whereas most giant cell
tumors occur in the meta-epiphysis. The peak age of osteosarcoma
is in the second decade, and that of giant cell tumor is in the third
[5]. Giant cell-rich osteosarcoma can occur in different location
from giant-cell tumor: in these cases, even if the differential diag-
nosis between these tumors on histology is difficult, the location
can lead to the correct diagnosis. However, when a giant cell-rich
osteosarcoma occurs in a common location for giant-cell tumor,
histology is the most important factor in the differential diagnosis,
especially the sarcomatous aspect of the stroma. If a giant cell-rich
osteosarcoma with minimal atypia is located in the bone extremity
the diagnosis is extremely difficult [4].

0720-048X/$ – see front matter © 2010 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ejrad.2010.06.050
4 M. Gambarotti et al. / European Journal of Radiology 77 (2011) 3–5

Fig. 1. Initial evaluation. On radiographs ((a) AP and (b) lateral views) and CT (c), lytic well limited epiphyseal lesion. The sclerotic component is obvious on CT. Histologically
(d) proliferation of mononuclear cells with round to ovoid nuclei without atypia, interspersed with multinucleated giant cells; the nuclei in giant cells are identical to the
nuclei in mononuclear cells. There is focal osteoid production and scattered mitosis. (Hematoxiline–Eosin, 10×).

Fig. 2. On CT (a) the recurrence is well visible, as a lytic well limited lesion near the cement (arrow). Histologically (b) on the entire specimen the lesion is more cellular
with mononuclear cells showing a more spindle appearance. The production of osteoid is abundant in some areas. These features are consistent with an osteosarcoma. The
presence of numerous multinucleated giant cells defined the lesion as a giant cell rich osteosarcoma. (Hematoxiline–Eosin, 10×).
 M. Gambarotti et al. / European Journal of Radiology 77 (2011) 3–5
Also, giant cell-rich osteosarcoma should be differentiated from
malignancy in giant cell tumor. The latter consists of a high grade
sarcoma arising in a giant cell tumor or at the site of a previously
documented giant cell tumor [8]. In this case the confusion could
arise when a primary giant cell tumor with proliferations of round
to oval mononuclear cells and multinucleated giant cells coexist
with an osteosarcoma, which can be the malignant component.
However, there is an abrupt change from the benign giant cell
tumor and the malignant component, so areas of benign giant cell
tumor are identified, and the malignant component that can cause
confusion is a conventional osteosarcoma, which usually does not
have abundant giant cells.
Thirteen percent of osteosarcomas may show moderate to large
numbers of benign osteoclast-like giant cells [2]. Therefore, it is use-
ful to widely sample the specimens. Telangiectatic osteosarcoma
can have a similar histological picture, especially in more cellular
areas, since it is composed of malignant spindle cells with little
osteoid production. The difference is made by the detection of cys-
tic blood spaces surrounded by septae containing the sarcomatous
cells.
It is difficult to correctly assess the characteristics of giant cell-
rich osteosarcoma, since its incidence is very low, and only a few
previous reports have been made in literature [1,4,9,10]. Two cases
were dedifferentiation in a parostal osteosarcoma [5,11], and one
case in the radius was initially diagnosed as a giant cell tumor [12].
This and our case demonstrate that a giant cell-rich osteosarcoma
with little atypia can simulate a giant cell tumor when it presents
in the epiphysis of a long bone.
On imaging studies it is also difficult to distinguish giant cell-
rich osteosarcoma from giant cell tumors [13], but a dense focus of
bone on radiograph or CT (as in our case) should raise the suspicion
and guide the biopsy on this special location.
The diagnosis of giant cell-rich osteosarcoma must be made
following strict criteria, since most of the times a lesion with
abundance of giant cells can be a conventional osteosarcoma with
areas of abundant osteoclast-like giant cells, or a telangiectatic
osteosarcoma with focal solid areas. Also, care should be made
when diagnosing a lesion as a giant cell tumor, especially if it is
in a young patient or in an unusual location.
5
2. Conclusion
Giant cell-rich osteosarcoma is a rare subtype of osteosarcoma
that should always be kept in mind when examining an osteolytic
lesion that resembles a giant cell tumor both radiologically and
histologically, especially in young patients or in unusual locations,
such as the metaphysis and diaphysis of long bones. The presence of
a sclerotic component on imaging examinations, or atypical spindle
cells in the stroma between the giant cells is the most useful diag-
nosis key. The diagnosis of giant cell-rich osteosarcoma should be
made only if a conventional osteosarcoma with giant cells, a telang-
iectatic osteosarcoma with solid areas, and other giant cell-rich
lesions have been ruled out.
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