Downloaded from www.ajronline.org by 36.72.215.220 on 01/08/18 from IP address 36.72.215.220. Copyright ARRS.

For personal use only; all rights reserved

Original Report
Murali Sundaram 1
William G. Totty 2
Imaging Findings in Pseudocystic
Michael Kyriakos 3 Osteosarcoma
Douglas J. McDonald 4
Kurt Merkel 5 OBJECTIVE. Our purpose was to describe four female patients with osteosarcoma whose
clinical and imaging findings primarily suggested either simple or aneurysmal bone cyst. All le-
sions were osteolytic, intracompartmental, and expanded bone without periosteal reaction. None
of the patients presented during the peak age incidence for osteosarcoma. From imaging to histo-
logic diagnosis, the discovery of osteosarcoma ranged from 1 week to 3 years.
CONCLUSION. Atypical osteosarcoma may rarely mimic simple or aneurysmal bone cyst
radiologically and may show a nonmalignant rate of growth. It may be more frequently encoun-
tered in females and may not present during the peak age incidence for osteosarcoma. Micro-
scopically, the tumors were not cystic, necrotic, or telangiectatic but were conventional
osteosarcoma and osteoclast-rich osteosarcoma.

O steosarcoma is the most common

Received July 17, 2000; accepted after revision
August 21, 2000.
1 Department of Radiology, St. Louis University Health
Sciences Center, 3635 Vista at Grand, St. Louis, MO 63110-
0250. Address correspondence to M. Sundaram.
2
Mallinckrodt Institute of Radiology, Washington
University School of Medicine, 510 S. Kingshighway,
St. Louis, MO 63110.
3
Department of Surgical Pathology, Washington University
School of Medicine, St. Louis, MO 63110.
4
Department of Orthopedic Surgery, Washington
University School of Medicine, St. Louis, MO 63110.
5
Department of Orthopedic Surgery, St. Louis University
Health Sciences Center, St. Louis, MO 63110-0250.
AJR 2001;176:783–788
0361–803X/01/1763–783
© American Roentgen Ray Society
nonhematologic primary malignant
tumor of bone [1–4]. The radiologic
appearance of osteosarcoma is usually that of an
aggressive destructive lesion with a mixed os-
teolytic and sclerotic pattern and an associated
periosteal reaction and soft-tissue mass. Os-
teosarcoma, however, may show considerable
variability in its radiologic and histologic ap-
pearances. A purely osteolytic form of osteosar-
coma occurs in approximately 10% of all
patients and is associated with a pattern of rapid
growth [5]. Telangiectatic osteosarcoma, addi-
tionally, may have the appearance of an aneu-
rysmal bone cyst.
We present four female patients with os-
teolytic intracompartmental tumors without a
periosteal reaction or soft-tissue mass that ra-
diographically suggested either simple or aneu-
rysmal bone cyst but microscopically proved to
be conventional osteosarcoma and osteoclast-
rich osteosarcoma.
Materials and Methods
Four female patients were identified in the past
5 years whose lesions, based on age, location, and im-
aging findings, suggested simple or aneurysmal bone
cysts but radiologically proved to be osteosarcoma.
Their ages were 3, 7, 26, and 34 years. All patients
had radiographic studies, three had MR imaging, and
one had CT. Two of the osteosarcomas were in the
proximal tibia; one, in the femoral head and neck; and
the fourth, in the navicular bone (Figs. 1–4). Three pa-
tients were white, and one was black.
Both children had large well-demarcated osteolytic
lesions in the proximal diametaphysis of the tibia that
were initially interpreted as simple bone cysts and on
follow-up as aneurysmal bone cysts (Figs.1 and 2).
The 7-year-old white girl, who was first seen in No-
vember 1995 and whose lesion had been injected with
steroids twice because of lack of regression (Figs. 1A
and 1B), presented 6 months after her initial presenta-
tion with a fracture after an injury (Fig. 1C). Because
there was a focal bump, CT was performed and
showed the cortex intact without extraosseous mass.
IV contrast material showed enhancement of the tis-
sue element in the lesion thought to be inconsistent
with a simple bone cyst and more likely to represent
an aneurysmal bone cyst (Fig. 1D). The 3-year-old
black girl, who was first seen in June 1998 (Figs. 2A
and 2B), had her leg placed in a cast because of a frac-
ture, and when her lesion was injected with steroids in
August 1998, aspiration yielded bloody fluid. Radio-
graphs in November 1998 showed minimal change in
the size of the lesion, and the diagnosis was changed
to aneurysmal bone cyst. MR imaging in December

AJR:176, March 2001 783

 Sundaram et al.

1998 showed the lesion expanded, occupying the
proximal one third of the tibia with no evidence of an
extraosseous mass (Figs. 2C and 2D). Both patients at
this point had open biopsies performed, and the diag-
nosis of conventional osteosarcoma was made. They
received chemotherapy, after which the 7-year-old pa-
tient (Fig. 1) was treated by a wide local excision of
Downloaded from www.ajronline.org by 36.72.215.220 on 01/08/18 from IP address 36.72.215.220. Copyright ARRS. For personal use only; all rights reserved
woman, who presented with a 4-month history of left
hip pain, radiographs of the hip showed an eccentric
osteolytic lesion occupying the femoral neck and lat-
eral aspect of the femoral head (Figs. 3A and 3B).
The differential diagnosis included giant cell tumor
and aneurysmal bone cyst. CT showed the lesion en-
tirely osteolytic and intracompartmental, with an in-
and neck were then resected, and curettage of the tro-
chanteric extension was performed with a total hip ar-
throplasty reconstruction. Histologically, the curetted
material showed numerous collapsed cystic walls and
a few intact cystic spaces filled with blood. There
were neither atypical mitosis and destruction of bony
trabecula at the perimeter nor infiltrative growth. The

the tumor and reconstruction with intercalary al-
lograft. The 3-year-old patient (Fig. 2) was treated by
knee disarticulation in March 1999.
The two adult patients also presented with tumors
in the lower extremity. In the 34-year-old white
tact thin rim of cortical bone superiorly and laterally
(Fig. 3C). MR imaging (Fig. 3D) confirmed the intra-
compartmental confines of the tumor. Frozen-section
biopsy tissue was histologically diagnosed as consis-
tent with an aneurysmal bone cyst. The femoral head
histologic diagnosis was that of an aneurysmal bone
cyst. Four months after surgery, the patient developed
an enlarging mass in her left thigh. Radiographs
showed extensive local recurrence in the proximal fe-
mur around the arthroplasty and diffuse bilateral met-

A B C

Fig. 1.—Proximal tibial lesion in 7-year-old girl.

A, Lateral radiograph of proximal leg shows expansive osteolytic lesion of proximal metaphysis. Note
scalloping of anterior endosteal cortex.
B, Lateral radiograph obtained 3 months after injection of steroids shows no change in metaphyseal lesion.
C, Lateral radiograph obtained 33 months after A and 36 months after B shows interval growth of lesion.
Note transverse fracture through middle of lesion. Minimal callus bridges fracture line anteriorly, indi-
cating that fracture is subacute.
D, Contrast-enhanced axial CT scan corresponding to C shows marked enhancement through medial
portion of lesion.
D

784 AJR:176, March 2001

 Pseudocystic Osteosarcoma
Downloaded from www.ajronline.org by 36.72.215.220 on 01/08/18 from IP address 36.72.215.220. Copyright ARRS. For personal use only; all rights reserved

A B C

Fig. 2.—Tibial lesion in 3-year-old girl.

A and B, Anteroposterior (A) and lateral (B) radiographs of right leg, June1998, show well-defined diameta-
physeal osteolytic lesion of tibia with slight endosteal thinning. Lesion is well marginated, and on lateral image,
fracture is identified in anterior cortex.
C and D, Coronal (C) (TR/TE, 4500/96) and axial (D) (3500/119) MR images, 6 months after A and B, confirm intra-
compartmental confines of tumor with aneurysmal configuration.
D

astatic pulmonary disease. Histologic sections of
tissue obtained from open biopsy of the mass showed
areas characteristic of aneurysmal bone cysts, but sec-
tions of the more ossified tumor tissue showed solid
areas with abundant osteoid and bone production.
These areas merged with an atypical chondroid and
spindle cell stroma. Separate foci of atypical chon-
droid stroma believed to be part of the lesion, rather
than reactive callus, were seen. These latter patterns
were believed to represent features of a conventional
osteosarcoma. Review of the prior biopsy tissue failed
to show any cartilage or atypical stroma. The patient
was treated with chemotherapy.
The fourth patient in this series is a 26-year-old
woman who presented with a 1-year history of pain
and swelling in the medial aspect of her right mid
foot. Radiographs of the foot showed the medial half
of the navicular bone expanded by a multilocular os-
teolytic lesion with thinning of the cortex. The pre-
sumptive radiologic diagnosis was aneurysmal bone
cyst (Figs. 4A and 4B). MR imaging on coronal and
sagittal T1-weighted sequences revealed nearly com-
plete replacement of the navicular bone by tumor, and
on T2-weighted sequences, the lesion was multilocu-
lar with fluid–fluid levels (Figs. 4C and 4D). Curet-
tage with bone grafting was performed. Histologic
examination of the curettage specimen revealed iso-
lated cystic blood spaces whose walls contained atyp-
ical stromal cells with abundant mitotic figures and
islands of osteoid produced by these cells. A diagnosis
of telangiectatic osteosarcoma was made. The patient
received a 3-month course of chemotherapy after
which the navicular, talus, and medial cuneiform
bones were resected and the foot was reconstructed
with a segment of iliac crest and allograft. Histologi-
cally, the tumor contained areas indistinguishable
from benign giant cell tumor with nodular areas of be-
nign osteoclast-type giant cells. Anaplastic stromal
cells and multinucleated giant tumor cells with ana-
plastic pleomorphic nuclei populated other areas.
Stromal spindle cells in storiform pattern and numer-
ous highly atypical mitotic figures were present. Os-
teoid formation by malignant stromal cells was
evident. Only a rare aneurysmal blood space was
found. The tumor was confined to the navicular bone.

AJR:176, March 2001 785

 Sundaram et al.
Downloaded from www.ajronline.org by 36.72.215.220 on 01/08/18 from IP address 36.72.215.220. Copyright ARRS. For personal use only; all rights reserved

A B C

Fig. 3.—34-year-old woman with eccentric osteolytic lesion in left femoral head and neck.
A, Anteroposterior radiograph of left hip shows intracompartmental osteolytic lesion eccentrically located in
femoral head and proximal neck and extending to intertrochanteric line.
B, Frog lateral image confirms eccentric intracompartmental lesion.
C, Axial CT scan of left hip joint reveals thin shell of bone containing lesion superolaterally without soft-tissue
mass or intraosseous matrix.
D, T2-weighted MR axial image (TR/TE, 1950/90) shows no extraosseous mass.
D

A diagnosis of osteoclast-rich osteosarcoma with fo-
cal aneurysmal bone cyst–like areas was made.
Results
From imaging to histologic diagnosis, the
discovery of osteosarcoma ranged from 1
week to 3 years. Three patients had conven-
tional osteosarcoma, and one, an osteoclast-
rich osteosarcoma. All lesions were osteolytic,
intracompartmental, and expanded bone with-
out periosteal reaction. In one patient, the tu-
mor remained intracompartmental for 3 years
before the diagnosis was made (Fig. 1). In an-
other, the lesion did not significantly change
for 6 months after a pathologic fracture
through the lesion (Fig. 2). In three patients,
diagnosis of osteosarcoma was made at the
time of biopsy, whereas in one patient, an
initial diagnosis of aneurysmal bone cyst
was made after curettage (Fig. 3). This pa-
tient subsequently developed metastases and
died. The two children are disease-free 20
and 17 months postoperatively. The patient
with an osteosarcoma in the tarsal navicular
bone was disease-free 14 months postopera-
tively (Fig. 4).
Discussion
Osteosarcoma is the most common nonhema-
tologic primary malignant tumor of bone whose
peak age incidence is usually from 15 to 20
years old with a 2:1 male predominance [1–4].
Most conventional osteosarcomas have a mixed
lytic and sclerotic radiographic pattern, reflect-
ing the type of tissue or matrix in the tumor.
Purely osteolytic lesions are uncommon, ac-
counting for approximately 10% of all patients
and usually encompassing the fibroblastic, fibro-
histiocytic, telangiectatic, or giant cell–rich
forms of the tumor [1–4]. Except for the subtype
of low-grade well-differentiated osteosarcoma,
purely lytic osteosarcoma has been associated
with a pattern of rapid growth [5]. Low-grade
osteosarcomas, however, in greater than 50% of
the patients, have a soft-tissue mass, and most
show poor margination [3]. Mirra [4] indicated
that 80% of osteosarcomas will have a periosteal
reaction, and even slow growing or low-grade le-
sions, which he describes as “incipient,” will, on
CT or MR imaging, show a soft-tissue mass.
All our patients were female, and none were
within the peak age of incidence for osteosar-
coma. Two were younger than 10 years old, an
uncommon age for osteosarcoma. In the Mayo
Clinic series of 1649 osteosarcomas, only seven
patients were younger than 10 years old, six of
whom were girls [3]. The radiographic features
common to all our patients were the intracom-
partmental location of the tumor, a purely os-
teolytic pattern, and an absence of a periosteal
reaction or soft-tissue mass. In all patients, the
involved bone was expanded, with variably
thinned endosteum and without a cortical
breech (Figs. 1–4). The radiologic interpretation
of the imaging features was influenced by the
age of patients and the location of the lesions so
that simple bone cyst or aneurysmal bone cyst
was considered the most likely diagnosis in
three patients, whereas giant cell tumor was fa-
vored over aneurysmal bone cyst in one patient.

786 AJR:176, March 2001

 Pseudocystic Osteosarcoma
Downloaded from www.ajronline.org by 36.72.215.220 on 01/08/18 from IP address 36.72.215.220. Copyright ARRS. For personal use only; all rights reserved

A B C

Fig. 4.—26-year-old woman with lesion in tarsal navicular bone.

A and B, Coned-down anteroposterior (A) and oblique (B) radiographs of mid foot show multilocular osteolytic
lesion expanding tarsal navicular bone medially with intact cortex.
C, T1-weighted coronal MR image (TR/TE, 500/17) shows complete replacement of tarsal navicular bone by tumor.
D, Fat-suppressed coronal MR image (4500/105) reveals no extraosseous mass. Tumor has multilocular appear-
ance with high signal intensity and fluid–fluid levels.
D

Three of the tumors were conventional
osteosarcomas, and one, a giant cell–rich vari-
ant of osteosarcoma with focal aneurysmal
bone cystlike areas. In three patients, the diag-
nosis of osteosarcoma was initially made on ex-
amination of both biopsy sample and the
resection specimen. In the other patient, tissue
was diagnosed as an aneurysmal bone cyst.
However, when this patient returned with pulmo-
nary metastases, the rebiopsy tissue showed not
only features identical to those in the original bi-
opsy sample but also small areas of conventional
osteosarcoma. The tumor in the navicular bone
was originally diagnosed as telangiectatic
osteosarcoma on biopsy tissue, but the diagnosis
was changed to osteoclast-rich osteosarcoma
after examination of the resected tumor.
The tumors in both children showed relatively
indolent rates of growth for osteolytic osteosar-
comas. In the 7-year-old child (Fig. 1), the tumor
did not change its appearance over a 3-year pe-
riod, and when the diagnosis of osteosarcoma
was finally made, there was no evidence of met-
astatic disease. This patient, whose tumor was
first observed almost 5 years ago, remains dis-
ease-free. In the 3-year-old child, the initial pre-
sumptive diagnosis of a simple bone cyst was
changed to an aneurysmal bone cyst when an as-
pirate of the lesion, 3 months after it was first ob-
served, yielded bloody fluid. Six months after
the lesion was initially discovered, MR images
showed no evidence of a soft-tissue mass (Figs.
2C and 2D).
Both patients’ imaging studies would fit the
characterization by Mirra [4] of “incipient” os-
teosarcomas, because neither of the lesions
showed any of the described features associated
with osteosarcoma, and despite the relatively in-
dolent rates of growth, the tumors were conven-
tional and not low-grade osteosarcoma. In both
patients, a radiologic diagnosis of solitary bone
cyst was considered most likely. However, the
tibia, the location of both tumors, appeared to be
an uncommon location for simple bone cysts. In
the Netherlands’ Bone Tumor Registry [6], of
209 simple bone cysts, only nine were so lo-
cated. Of the nine cysts, only five were in the
proximal tibia.
The age of the patient with an eccentric loca-
tion of the lesion in the femoral head and neck
favored a diagnosis of giant cell tumor rather
than aneurysmal bone cyst, but the tissue was
thought to be aneurysmal bone cyst. When this
patient developed metastatic disease, the most
likely source of error was a telangiectatic os-
teosarcoma mistakenly diagnosed as an aneurys-
mal bone cyst. Although the recurrent tumor also
showed features of aneurysmal bone cyst, solid
areas of conventional osteosarcoma were
present. Review of the prior biopsy tissue failed
to show such areas. Location of osteosarcoma in
the femoral head and neck is distinctly uncom-
mon, constituting less than 1% of all osteosarco-
mas in four large series [1–4].
In the patient with a lesion in the tarsal navic-
ular bone, the imaging features of an expanded
osteolytic lesion with thinning of the endosteum
and fluid–fluid levels suggested aneurysmal
bone cyst. There was no soft-tissue mass. Al-
though histologic examination of this lesion
suggested a diagnosis of telangiectatic osteosar-
coma, the resected tumor showed an osteoclast-

AJR:176, March 2001 787


rich osteosarcoma with only rare aneurysmal
blood spaces. This patient appears to be the first
one with osteoclast-rich osteosarcoma in a foot
bone [7]. Osteosarcoma of any type in a foot
bone is an exceptional occurrence. Of 1929
cases of osteosarcoma at the Rizzoli Institute
Downloaded from www.ajronline.org by 36.72.215.220 on 01/08/18 from IP address 36.72.215.220. Copyright ARRS. For personal use only; all rights reserved
(Bologna, Italy), only 12 tumors (0.6%) were in
the foot, none of which were in the navicular
bone [8]. By contrast, the occurrence of aneurys-
mal bone cyst in the small bones of the hands
and feet is significantly higher, with an incidence
ranging from 9% to 12% [9, 10]. In a recent re-
view of 52 foot osteosarcomas by Choong et al.
[11], the radiologic patterns were consistent with
conventional osteosarcoma, and all cases were
associated with a soft-tissue mass. The calcaneus
was the most commonly involved bone, with the
navicular bone involved in only two cases.
Even in retrospect, our patients do not lend
themselves to a presumptive diagnosis of os-
teosarcoma. Because simple bone cysts and an-
eurysmal bone cysts were the primary
diagnostic considerations in this group of pa-
tients, we have elected to collectively describe
these cysts as “pseudocystic” osteosarcoma.
Our series is too small to make any determi-
nations about the relationship of the described
atypical features to clinical outcome. In this
subset of patients, the unpredictability of the
disease is underscored by the extremes of out-
788
Sundaram et al.
come in two of our patients who did not initially
receive optimal treatment. One patient was un-
treated for 3 years and remains alive and well,
whereas another developed metastatic disease
4 months after presentation and died 13 months
later. We could not identify a similar series of
osteosarcoma with a pseudocystic radiologic
appearance, although it has been stated, without
references or illustrations, that rarely does os-
teosarcoma have such an appearance [1, 3]. A
pictorial essay reviewing subtle, rare, and mis-
leading radiologic appearances of osteosarco-
mas did not have cases similar to those reported
in our study [12]. To determine the incidence
and prognostic significance, if any, of the imag-
ing and other associated atypical features of os-
teosarcoma described herein, a review of a
significantly larger series is required.
Acknowledgment
We appreciate the considerable secretarial as-
sistance of Lois Hebel in the preparation of this
manuscript.
References
1. Dorfman HD, Czerniak B. Osteosarcoma. In:
Dorfman HD, Czerniak B, eds. Bone tumors. St.
Louis: Mosby, 1998:128–152
2. Mulder JD, Schütte HE, Kroon HM, Taconis WK.
Intraosseous osteosarcoma. In: Mulder JD,
Schütte HE, Kroon HM, Taconis WK, eds. Ra-
diologic atlas of bone tumors. Amsterdam:
Elsevier, 1993:51–53
3. Unni KK. Osteosarcoma. In: Unni KK, ed. Dah-
lin’s bone tumors, 5th ed. New York: Lippincott-
Raven, 1996:143–183
4. Mirra JM. Osseous tumors of intramedullary ori-
gin. In: Mirra JM, ed. Bone tumors, vol. 1. Phila-
delphia: Lea & Febiger, 1989:250–289
5. DeSantos LA, Edeiken B. Purely lytic osteosar-
coma. Skeletal Radiol 1982;9:1–7
6. Mulder JD, Schütte HE, Kroon HM, Taconis WK.
Solitary bone cyst. In: Mulder JD, Schütte HE,
Kroon HM, Taconis WK, eds. Radiologic atlas of
bone tumors. Amsterdam: Elsevier, 1993:579–581
7. Ostrowski, ML, Spjut HJ. Lesions of the bones of
the hands and feet. Am J Surg Pathol 1997;21:
676–690
8. Biscaglia B, Gasbarrini A, Bohling T, Bacchini P,
Bertoni F, Picci I. Osteosarcoma of the bones of
the foot: an easily misdiagnosed malignant tumor.
Mayo Clin Proc 1998;73:842–847
9. Mulder JD, Schütte HE, Kroon HM, Taconis WK.
Aneurysmal bone cyst. In: Mulder JD, Schütte HE,
Kroon HM, Taconis WK, eds. Radiologic atlas of
bone tumors. Amsterdam: Elsevier, 1993:557–577
10. Dahlin DC, McLeod RA. Aneurysmal bone cyst
and other non-neoplastic conditions. Skeletal Ra-
diol 1982;8:243–250
11. Choong PFM, Quereshi AA, Sim FH, Unni KK. Os-
teosarcoma of the foot: a review of 52 patients at the
Mayo Clinic. Acta Orthop Scand 1999;4:361–364
12. Rosenberg ZS, Lev S, Schmahmann S, Steiner GC,
Beltran J, Present D. Osteosarcoma: subtle, rare,
and misleading plain film features. AJR 1995;
165:1209–1214
AJR:176, March 2001