323

Parosteal Osteosarcoma:
Radiologic-Pathologic Correlation with
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Emphasis on CT

Marvin M. Lindell, Jr.1 Parosteal osteosarcoma, a distinct entity in which the neoplasm arises on the bone
Ali Shirkhoda1 surface, presents characteristic features. Thorough radiologic and histologic evaluation
A. Kevin Raymond2 and early definitive surgery usually result in a favorable prognosis and make limb
salvage feasible in many adult patients. Twenty-six patients with proven parosteal
John A. Murray
osteosarcoma were seen at The University of Texas M. D. Anderson Hospital and Tumor
Thomas S. HarIe1
Institute at Houston. All were examined by conventional radiography, 16 by CT, and one
by both CT and MR. CT is valuable for the evaluation of the medullary
of tumor invasion
canal, involvement of the cortex, and extension into the soft tissues, findings frequently
not demonstrable by other noninvasive techniques. Additional information is obtained
regarding the presence and location of satellite lesions and intralesional radiolucent
areas and the relationship of the vascular bundle to the tumor mass. These findings are
important for planning both percutaneous biopsy and surgery.

The radiographic and pathologic features of conventional parosteal osteosar-

coma (POS) have been well documented [1-1 5]. P05 is a relatively rare primary
neoplasm of bone. In some series it constitutes about 1 .7% of all benign and
malignant tumors of bone [1 2]. The tubular long bones of the appendicular skeleton
are usually involved, most often the popliteal surface of the femur (79%).
Recently, a subset or variant of conventional POS has been recognized: dedif-
ferentiated POS. In most cases dedifferentiated POS occurs as a result of recur-
rence of a previously treated conventional POS; however, dedifferentiated POS
may also occur as the primary manifestation. In either case, this neoplasm is a
high-grade malignant tumor with full capacity for systemic metastasis and death in
more than 50% of affected patients. The behavior of dedifferentiated POS, there-
fore, is more akin to conventional high-grade intramedullary osteosarcoma than to
conventional POS.
Conventional POS is a tumor of indolent behavior in which the potential morbidity
Received February 28, 1986; accepted after re- and mortality of contemporary chemotherapy are unjustified and unwarranted.
vision September 19, 1986.
Treatment is aimed at the primary lesion and is surgical. However, considering the
Presented at the annual meeting of the American
mortality in dedifferentiated POS (>50%), chemotherapy may affect it significantly.
Roentgen Ray Society, Washington, DC, April 1986
1 Department of Diagnostic Radiology. The Uni- At M. D. Anderson Hospital the diagnosis of dedifferentiated POS indicates the use
versity of Texas M. D. Anderson Hospital and Tu- of preoperative chemotherapy: intraarterial cis-platinum and systemic adriamycin.
mor Institute, 6723 Bertner Ave. , Houston, TX Therefore, accurate evaluation of these patients before definitive surgery is essen-
77030. Address reprint requests to M. M. Lindell.
tial in order to identify those who might benefit from preoperative chemotherapy.
2 of Pathology, The University of
Twenty-six cases of conventional and dedifferentiated POS, (1 6 had CT and one
Texas M. D. Anderson Hospital and Tumor Institute,
6723 Bertner Ave., Houston, TX 77030. MR) are reviewed to determine the radiographic spectrum of conventional POS
of Orthopedic Surgery. The Uni- and which features might suggest the diagnosis of dedifferentiated POS.
versity of Texas M. D. Anderson Hospital and Tu-
mor Institute, 6723 Bertner Ave.. Houston, TX
77030. Materials and Methods
AJR 148:323-328, February 1987
0361 -803X/87/1 482-0323 The clinical, radiographic, and histologic materials of 26 patients with either conventional
© American Roentgen Ray Society P05 (23) or dedifferentiated P05 (three) seen at The University of Texas M. D. Anderson
 324 LINDELL ET AL. AJR:148, February 1987
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Hospital and Tumor Institute at Houston between 1976 and 1986 By using proper window and center settings, the CT images
were reviewed. Of the 26 patients, 1 6 (seven men and nine women, obtained were reviewed for evaluation of the size and extent
ranging in age from 1 6 to 47 years) were examined by CT and one
of the tumor mass. There was excellent demonstration of
(a woman) was studied
by CT and MR. The neoplasm was located
radiolucent areas within the tumors as well as visualization of
in the distal femur in 1 3 patients and in the proximal tibia in three
satellite nodules. Of the six cases with radiolucent areas
patients. All tumors were treated surgically and histologically docu-
mented as either conventional or dedifferentiated P05. In six cases,
shown on CT, only two were seen on conventional radio-
percutaneous biopsy was done before resection of the neoplasm. graphs. In two cases, the radiolucent areas were shown to
The surgical specimens were first correlated with the preoperative be hypervascular angiographically and corresponded to his-
radiographs and then sectioned for further correlation with the CT tologically documented foci of high-grade sarcoma, thereby
images. Specimens from patients receiving preoperative chemother- establishing the diagnosis of dedifferentiated POS (Fig. 4).
apy were sectioned according to protocol to ensure that qualitative Satellite lesions were present in seven cases; all were seen
and quantitative evaluation of tumor necrosis could be determined. on CT and six were visible on radiographs. In two cases, CT
This also provided three-dimensional specimen preoperative radi- showed the satellite lesion to a much better extent than
ographic comparison and precise spatial localization of viable tumor.
conventional radiographs did (Fig. 5).
The CT scans were obtained on Siemens Somatom II, GE 8800,
The cortical bone was evaluated in all patients who had CT
and EMI 5005 scanners. Depending on the scanning device used,
scans were obtained by using an 8- to 1 0-mm slice interval and table
or MR. In 1 2 patients (71 %), the cortex appeared definitely
increment. Window widths and levels were selected for optimal image invaded, and in two there was no demonstable abnormality
display. The one MR study was done on a Diasonics superconductive of the cortical bone. In two patients, the judgment was
0.35-T unit, and the images were obtained in axial, coronal, and equivocal and no firm diagnostic conclusion could be made
sagittal planes. (Fig. 3). In one patient, the cortical involvement was better
seen on the sagittal MR images than on axial CT or MR (Fig.
SD). Furthermore, by using the same CT setting, the density
Results
of the medullary cavity was compared with the contralateral
The size of the neoplasms ranged from 2 to 1 2 cm by limb at the same level. Eleven patients having CT did not
measurement of the greatest transverse dimension on the CT have evidence of medullary involvement, while in five cases
images. In nine patients, significant intralesional components the marrow cavity was invaded by the neoplasm (Fig. 4).
of the tumor were nonossified. In three of these, the area of There was no tumor in the marrow cavity of the patient
lucency was large, well defined, and located deep within the examined by both CT and MR.
main portion of the tumor. In six they tended to be small, Histologically, all cases of conventional POS were com-
poorly defined, and peripherally located at the interface of posed of relatively bland spindle cells, which produce well-
tumor and normal tissue. In seven patients, the tissue plane formed bone. The peripheral areas of radiolucency corre-
surrounding the neoplasm was obliterated, probably second- sponded to either normal or neoplastic elements. The normal
ary to edema. As shown by CT, three patterns of calcification structures in these areas included foci of entrapped fibrous
within the tumor masses were recognized: amorphous and connective tissue, tendon, adipose tissue, vessels, and skel-
irregular in five patients, localized and homogeneous in seven, etal muscle. Radiolucent neoplastic elements in conventional
and a mixture of both in four (Figs. 1-3). POS corresponded to foci of nonmineralized low-grade carti-
 AJR:148, February 1987 PAROSTEAL OSTEOSARCOMA 325

Fig. 4-A, Anteroposterior radiograph shows

large dedifferentiated parosteal osteosarcoma
of distal femur.
B, Lateral radiograph shows dedifferentiated
parosteal osteosarcoma of distal femur having
large radiolucent area (arrow). (Levels A and B
correspond to CT levels in C and D).
C, CT at level A shows dedifferentiated paros-
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teal osteosarcoma of distal femur (long arrows)

with cortical erosion. Soft-tissue plane is oblit-
erated. Cursor shows tumor within medullary
canal (short arrow).
D, CT at level B shows calcified tumor matrix
in lower area of mass.

lage (i.e., cartilage cap or satellite lesions) or areas of other- was identified before surgery (i.e., at preoperative needle
wise typical low-grade tumor that did not produce bone biopsy), each patient underwent preoperative chemotherapy.
matrix. Histologic examination of the subsequent resection speci-
The three cases of dedifferentiated POS presented a con- mens showed a consistent phenomenon: the low-grade com-
sistent pattern. In each, large areas were similar to conven- ponent showed minimal or no tumor necrosis and was left
tional POS: low-grade bland spindle cells producing well- virtually unaltered by the chemotherapy. However, the high-
formed bone. These areas corresponded to those parts of grade component showed changes similar to those observed
the tumor that were radiopaque and radiographically indistin- in conventional high-grade intramedullary osteosarcoma.
guishable from conventional POS. However, superimposed These latter changes consisted of massive tumor necrosis,
on otherwise typical conventional POS were areas of high- with reparative changes and an ingrowth of granulation tissue
grade sarcoma. These high-grade components corresponded [16].
to the large, well-defined radiolucent areas deep within the Four patients with conventional POS have had local recur-
lesions. In the two cases in which the high-grade component rence only. These have been managed successfully with
 326 LINDELL ET AL. AJR:148, February 1987

Fig. 5.-A, Anteroposterior radiograph shows

parosteal osteosarcoma of distal femur to be
ossified and enveloping bone. Radiolucent
cleavage planes (arrows) between normal cor-
tex and neoplasm.
B, CT shows parosteal osteosarcoma of distal
femur eroding cortex posterolaterally. Satellite
lesion anteriorly (arrow).
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C, Transaxial MR image (TR 2 sec, TE 60

msec) shows parosteal osteosarcoma of distal
femur as tumor with mixture of intermediate- and
low-intensity signals (long arrows). Level corre-
sponds to CT image. Note erosion of posterior
cortex in same area as on CT (short arrow).
D, Parasagittal MR image (TA 0.5 sec, TE 40
msec) of parosteal osteosarcoma of distal femur
shows craniocaudad extent of tumor (long
arrows) and erosion of posterior cortex (short
arrow).

localized therapy. Reresection was performed in three pa- [1 7], dedifferentiated POS [1 8], and high-grade surface os-
tients and an elective amputation in one patient who believed teosarcoma [i9].
that she could not tolerate the attendant postoperative train- Conventional POS remains a distinct pathologic/biologic
ing required for a new prosthesis. One patient with dediffer- entity. Conventional P05 is seen most often in the third and
entiated POS had pulmonary metastases at presentation and fourth decades, affects women more often than men, and
died of systemic disease within 6 months of surgery. The has a marked predilection for the long bones of the appen-
other patients are alive and well without evidence of local or dicular skeleton, in particular the posterior aspect of the distal
systemic recurrence. femur [4, 20, 21]. Histologically, conventional P05 is a low-
grade form of osteosarcoma in which bland spindle cells
produce well-formed bone. Biologically, it is a slowly progres-
Discussion
sive disease. Pulmonary metastases tend to appear late in
Since its original description, parosteal osteosarcoma has the course of the disease, frequently following one or more
been traditionally regarded as the single manifestation of local recurrences. Therefore, therapy is directed toward con-
osteosarcoma arising on the periosteal aspect of bone. How- trol of the primary tumor and is surgical. Early diagnosis
ever, it has become progressively more apparent that con- (which depends on clinical suspicion, thorough radiologic
ventional POS represents only one form of a heterogeneous evaluation, and accurate histologic interpretation followed by
group of osteosarcomas that arise on the cortical surface; appropriate therapy) usually presages a favorable prognosis.
the other members of this group are periosteal osteosarcoma Within technical limitations, adult patients may qualify as
 AJR:148, February 1987 PAROSTEAL OSTEOSARCOMA 327

Fig. 6.-A, Lateral radiograph shows large

parosteal osteosarcoma of distal femur demon-
strating radiolucent cleavage plane between hi-
mor and cortex (arrows).
B, Specimen photograph shows large paros-
teal osteosarcoma of distal femur. Note erosion
of cortex by lower aspect of tumor.
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candidates for limb salvage. Long-term survival is in excess The differential diagnoses of POS are well known, having
of 80-90% in patients receiving appropriate therapy after the been the subject of a number of excellent studies. Included
onset of the disease. are osteochondroma, myositis ossificans, extraosseous os-
Dedifferentiated POS is a rare and unusual bone tumor. teosarcoma, osteosarcoma, ossifying or calcifying hematoma,
Most cases have originated as typical examples of conven- and exuberant callus [7, 9, 12, 15, 21 , 22].
tional POS that, after multiple local recurrences, have under- Many investigators believe that the medullary cavity is
gone “transformation” into a histologically high-grade sar- usually spared, except in cases of previous treatment or a
coma. However, dedifferentiated POS as a primary manifes- history of long duration of growth. The precise biologic signif-
tation of an osteosarcoma arising on a cortical surface is also icance of medullary involvement in conventional P05 is un-
well described by Wold et al. [1 8] (Case 1 1). In this primary clear. Some authors [20, 23] have interpreted the presence
form the radiographic, gross, and histologic features of the of tumor within the marrow cavity as indicative of a more
neoplasm closely mimic conventional POS. However, super- aggressive lesion, which is invariably associated with pulmo-
imposed on the low-grade tumor are areas of high-grade nary metastases and has a poorer prognosis. However, 0th-
sarcoma. In either case, primary or secondary, the ultimate ers [5, 9, 12, 1 5] have submitted data that the prognosis is
prognosis is a function of the high-grade component and not significantly affected by medullary extension. Although
survival is <50% at 5 years. the present attitude seems to indicate that the ultimate prog-
Several parameters were investigated to help define more nosis may be related to the degree of medullary involvement
fully the spectrum of the radiographic features of conventional [24], our experience has been that medullary disease does
POS and attempt to identify those unique features of dedif- not exclude successful limb salvage or significantly alter prog-
ferentiated POS. These included the presence or absence of nosis. Furthermore, none of the three cases in our series with
cortical or medullary cavity invasion, the nature of tumor soft- pulmonary metastases had marrow invasion. Others [25-33]
tissue interfaces, and the evaluation of areas of intralesional have reported malignant bone and soft-tissue sarcomas that
radiolucency. have been examined by CT for evaluation of tumor invasion
Radiographically, conventional POS is dense and lobular into the medullary canal.
with a broad base at the level of attachment to the cortical Identification of satellite nodules, which are so well shown
bone. Early tumors may have only a small sessile attachment on CT, provides further valuable information for treatment
to the bone surface [4]. The presence of a radiolucent cleav- planning. If the patient is having a limb-salvage procedure and
age plane between portions of the tumor and cortex is helpful these nodules are not totally removed, the frequency of
for diagnosis (Fig. 6). These slow-growing, bone-producing recurrent tumor would undoubtedly be higher.
malignant neoplasms tend to grow circumferentially along the Although the density of the calcific tumor is often homo-
cortical surface and may eventually envelop the bone shaft. geneous, the next most common pattern is amorphous and
If left untreated, they may eventually attain considerable size. irregular. These findings are best appreciated on CT and do
The cases in our series follow this pattern well. not correlate with the degree of malignancy of the tumors.
 328 LINDELL ET AL. AJR:148, February 1987

With rare exceptions, a notable feature of POS is the absence coma). In: Bone tumors, 2nd ed. Springfield, IL: Thomas, 1967:176-185
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10. Van der Heul RO, Von Ronnen JR. Juxtacortical osteosarcoma. J Bone
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so, they often constitute high-grade sarcoma. If biopsy con-
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cartilage. In: Atlas of tumor pathology, fasc 5. Washington, DC: Armed

firms this, the tumor may then be diagnosed as dedifferen- Forces Institute of Pathology, 1971:166-174
tiated POS [1 8-20] (Fig. 4). The ability of CT to localize these 13. Farr GH, Huvos AG. Juxtacortical osteogenic sarcoma. J Bone Joint Surg
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on all suspected malignant bone tumors that exhibit areas of
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hypervascularity on angiography. CT is used in guiding the Cancer 1976;37:2466-2475
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CT scanning is extremely useful in the determination of the
sarcomas. Am J Surg Pathol 1984;8:181-186
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