Osteogenic
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LUIS
Sarcoma
AUONSO
deSANTOS,1
after
the Age
JAN-ERIK
ROSENGREN,
of 50: A Radiographic WAYNE
Twenty-four cases of primary osteosarcoma in individuals over the age of 50 are described. This represents 6% of all osteosarcomas and 80% of osteosarcomas occurring over the age of 50 at this institution. Radiographically, features were similar to osteosarcoma in younger individuals. Females were more frequently affected than males (2.4:1). The radiographic features are discussed in detail, and the differential diagnosis outlined.
,
older
attention primary
of
has been paid osteosarcomas
to in
patients.
This
Radiographic
paper
illustrates
osteosarcoma ologic
little
the
in the elderly
radiographic
and
spectrum
presents
some
Site.
epidemi-
Within fected and
skeletal osteosancoma oven the age of 50 were
seen between selected for this
or a history
particularly
radiation
mal bone anatomic
of prior therapy.
treatment All
cases
1946 study.
administered with
underlying
in the area as well as those with prior region were eliminated. The history
and The
locally, abnor-
radiation to the of trauma not
leading to fracture at the site of tumor or fracture remote to the present tumor were not considered eliminating factors. The clinical course, particularly the presence on appearance of metastatic disease, was recorded. When available, autopsy data were reviewed. All nadiographs were studied for site of origin within boundaries,
the bone, size of the tumor, character nature of the tumor matrix, type
of the tumor of peniosteal
calcified
reaction, and presence of soft tissue mass. In patients admitted to M. D. Anderson Hospital after primary care of the local lesion, the outside
nadiognaphs
ated to assure
and
consistency
pathologic
material
of diagnostic
were
Received ,
reevalu-
criteria.
the
Bertner
Avenue,
Am J Roentgenol © 1978 American
20, 1978; accepted
Department
Houston,
after revision
of Diagnostic
Radiology.
Texas 77030. Address
131 :481-484, September Roentgen Ray Society
1978
reprint
locations
are
location
was
the
iliac
shown
in
table
1.
and left sides were afIn the pelvis the most bone,
with
only
one
case
osteoid
be made
in the
films
with
certainty
(fig.
midshaft
of
the
tibia
had
calcified
matrix
that
was
considered cartilaginous (fig. 1). Tumor boundaries. With the exception of four lesions, radiographs showed poorly defined aggressive zones of transition between the tumor and the recognizable adja-
patients over the age of 50 with de novo were found among 397 cases of osteosar-
January
All authors:
anatomic
3). Two lesions were considered purely lytic: one in the scapula (fig. 4) and one in the maxilla. Two lesions had a purely permeative pattern radiographically. One tumor in
Findings
Twenty-four osteosarcoma
The
the mandible, the right with equal frequency.
seen in the pubis. Within the long bones the metaphyseal or metaphysodiaphyseal junction was the site of origin of all tumors with the exception of two: one that was middiaphyseal in the tibia (fig. 1) and one seen in the distal phalanx of a digit. All except one lesion had the epicentrum within the medullary cavity of the involved bone. The exception was a cortical tumor located in the medial aspect of the distal femoral metaphysis (fig. 2). There were nine lesions on the left side and six on the right. Size. The lesions ranged from 1 .5 x 2 cm to 9 x 14 cm (average, 8 x 7 cm). Most of the lesions were over 5 cm when first seen. Tumor matrix. Most of the lesions were osteoblastic radiographically, but only in eight could the diagnosis of
medical records were reviewed for age, sex, presenting symptoms, and presence of any underlying local disease in the area involved
A. MURRAY
Features
common
Methods
The clinical and radiographic files of M. D. Anderson Hospital and Tumor Institute were reviewed for all cases of histologically documented 1975. Those
JOHN
of
considerations. Materials
AND
coma seen between 1946 and 1975. All patients were Caucasian except one. The 17 females and seven males ranged in age from 50 to 75 (median, 60). Eleven patients were 50-59 years old, 12 were 60-69 years, and two were over 70. The female-male ratio was 2.4:1. The most common presenting symptom was pain for a median of 2 months before diagnosis. All patients had surgical resection of the tumor. Approximately one-third also had local radiotherapy, another third had systemic chemotherapy, and the remaining third had no additional treatment. Fifteen patients developed metastatic disease; 13 had lung metastases. Four patients had metastases to the skeleton; of these, three also had lung metastases, and three had metastatic deposits to the local and regional lymph nodes. The time of appearance of metastatic disease ranged from 0 to 64 months (median, 5 months). Survival ranged from 1 month to 12 years (median, 2 years). Only two patients are presently alive 7 and 5 years, respectively, after diagnosis.
The occurrence of osteosarcoma over the age of 50 has generally been considered a sarcomatous transformation of Paget’s disease or some other benign bone lesion as well as a complication of irradiation, either endogenous or exogenously administered [1 2]. Isolated cases and general statements about primary osteosarcoma in patients over the age of 50 have appeared within general studies of osteosarcoma [3-6]. Although extensive literature is available on the clinical and radiographic features of osteosarcoma, the radiographic features
B. WOOTEN,
Evaluation
April 18, 1978. University
requests
of Texas
System
Cancer
Center,
M. D. Anderson
Hospital
and
Tumor
Institute,
6723
to L. A. deSantos.
481
0361
-803X/78/0900-0481
$00.00
482
deSANTOS TABLE Location
ET
AL.
1
of Osteosarcomas Location
No.
Head:
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Maxilla
and
nasal
and
frontal
bone
.
...
1
Maxilla
6
Mandible
Upper extremity: Scapula Humerus
1 1
Digit
Pelvis
Lower
4
extremity:
8
Femur
1
Tibia
24
Total
Fig.
involves medullary
2.-Cortical osteosarcoma. Relatively homogenous dense lesion medial cortex of distal femoral metaphysis (arrowheads). No involvement could be seen, but no tomograms were available.
Fig. 1 . -54-year-old male with midtibial osteosarcoma. Margins of lesion cannot be defined. Area of endosteal scalloping (arrow) and calcified matrix in form of isolated densities (arrowheads) simulate radiographic
features
of central
chondrosarcoma.
cent normal bone. Of the four exceptions, two were the previously indicated purely lytic lesions (fig. 4). One was a cortical lesion in the distal femur that appeared as a dense tumor attached to the cortex without invasion (fig. 2). The other was a midshaft tumor lesion of the tibia in which calcified matrix could be seen in the medullary cavity, but the tumor margins could not be defined (fig. 1). Periosteal reaction. Periosteal reaction of any kind
Fig. 3. -59-year-old female with distal femoral osteosarcoma. Large extremely dense lesion is demonstrated. Presence of calcified osteoid matrix outside
medullary cavity of shaft monic for osteosarcoma.
is radiographically
pathogno-
was
extremely
infrequent,
and
even
extensively
tive lesions presented scanty reaction. type of peniosteal response was seen
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one
in the
mandible
and
one
in the
destruc-
The “spiculated’ in only two cases,
vascular
mass
vessels
within
outlined
the
was
its
demonstrated
interior.
tumor
with
The
obvious
arteniognam
tumor
accurately
extent.
pelvis.
Extraosseous extent. The presence of a soft tissue mass component beyond the area of cortical on cancelbus bone destruction was demonstrated nadiognaphically in 14 cases (fig. 5). Osteoid calcification was seen within the mass in approximately half of these cases (fig. 6). Angiography was performed only in one case of osteosarcoma of the proximal humerus. A large hypen-
Discussion Osteosarcoma
is a rather
uncommon
tumor
compris-
of all malignancies, with an incidence of 0.4 pen 100,000 population [7, 8]. Despite its infrequency, it represents from 21% to 39% of all malignant primary bone tumors and therefore is an important consideration in the differential diagnosis of bone lesions [8]. The highest incidence is in the second and third decades, with 70%-91% of all lesions occurring below the age of 30 [5]. It is commonly stated in the literature that osteosancomas appearing over the age of 50 are secondary lesions and not “de novo” [1 2, 9]. However, in one series of 23 osteosarcomas in patients over the age of 50 [10], 50% ing
approximately
0.2%
,
had
no
evidence
Similarly,
of
antecedent
1 1 of 22 patients
disease
with
or
treatment.
osteosarcoma
occurring
oven the age of 40 reported by Weinfield and Dudley [4] were considered primary in origin. In our series 24 cases of primary osteosarcoma were seen among 30 patients with
osteosarcoma
than
those
The graphic
osteosarcomas features and
all respects
contrary older ratio
matrix
heads). coma.
Fig.
region.
4.-Purely is present.
Myeloma Open
arrow
lytic osteosarcoma Margins of lesion
was considered, indicates
r
biopsy
in 50-year-old are sharp without
but open biopsy
female. No visible sclerosis (arrow-
revealed
osteosar-
defect.
5.-Distal femoral osteosarcoma in a 53-year-old female. A, Anteroposterior B, Lateral projection reveals large soft tissue mass component posteriorly
the
age
previous
has
was
2.4:1 in
,
the
All
lesions
osteoid-type
projection (arrowheads)
were
incidence
compared second
[10]. to
and
the third
of considerable
calcification
had radiosimilar in
patients.
is
in
features
The
1 .5-2:1
and
female:male male
decades
size
could
This
occurring
radiographic
distribution
demonstrates simulating
an
osteosarcoma
reason for this is not clear, and it would see if this occurs in other series. and
50,
in younger
that
atypical
anatomic
of
groups.
in the present series an anatomic distribution
belief
patients
derence
two
to osteosarcoma
to the
different
Fig.
over
higher
prepon-
[3,
5].
The
be of interest when
first
seen,
be demonstrated
permeative lesion in lateral primary soft tissue lesion.
to
metaphyseal
in
The differential diagnosis of osteosarcoma oven the age of 50 rests primarily upon the differentiation from metastatic disease and multple myeloma. As a general rule,
metastatic
deposits
in bone
appear
radiognaphically
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as permeative lesions with poorly defined tumor boundaries. Scanty periosteal reaction and either no soft tissue mass or a relatively small one favor metastatic disease. Metastatic disease, primarily from prostatic and breast carcinoma
and
less
frequently
from
other
etiologies,
may
appear nadiognaphically as osteoblastic lesions resembling a primary bone tumor. However, calcified matrix is not seen outside the host bone in metastatic deposits. Some metastases, particularly from prostate, may in rare instances
Fig.
6. -Osteosarcoma
seous osteoid bone indicate
of ileum.
Unquestionable
is seen (arrows). In addition, osteoblastic intraosseous extent of disease.
calcified extraosareas within iliac
produce
patient
approximately one-third of the cases (figs. 3 and 6). The presence of osteoid calcification outside the bone of origin clearly defines the osteogenic nature of the lesion. Occasionally the destructive process of metastatic disease may carry small fragments of cortical bone into the soft tissues simulating tumor new bone formation. The linear rather than poorly defined nature of the calcification is usually sufficient for differential diagnosis. The presence of a soft tissue mass can be seen in both metastatic and primary bone tumors. However, the presence of a large soft tissue mass favors a primary malignant tumor such as osteosarcoma (fig. SB). In the purely lytic lesions the diagnosis may be difficult. Depending upon
the
location,
metastatic
as
well
as benign
was
present,
but due
to the
blastic
nature
of the
spicules
this probably represented tumor new bone formation rather than peniosteal reaction pen se. Two varieties of osteosarcomas are known to occur in older individuals. The first is osteosarcoma in the jaw bones, which has its maximum occurrence approximately 10-15 years later than peripheral osteosancoma [11]. The second is parosteal osteosarcoma, with a peak incidence two decades beyond that of the juvenile type osteosarcoma [12]. These two types of osteosancomas, however, are distinctly uncommon beyond the sixth decade. The two combined constitute approximately one-third of the total number in this series.
peniosteal
remains
an
unusual
reaction
tumor,
its
significant. seen with
Unlike other bone tumors, some frequency throughout
incidence
slowly
coma should nant-appeaning
simu-
tapers be
with
occurrence
is
osteosarcoma life, though
is its
increasing
considered tumor in the
age.
Osteosar-
when evaluating skeleton of an
a maligelderly pa-
tient.
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spiculated
lating the presence of tumor osteoid in the adjacent soft tissues. Multiple myeloma will be a differential diagnostic consideration only with purely iytic osteosancomas in which no calcified osteoid is seen and in which the tumor margins are better defined. In our two cases with purely lytic presentation (fig. 4), no differentiation could be made by radiographic features alone. In the rare case of myeloma presenting with increased bone density, no calcification occurs outside the bone of origin. Although osteosancoma arising ‘de novo” in the older
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