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Fibrous Dysplasia vs
Adamantinoma of the Tibia:
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Johan L. Bloem1 Differentiation between benign fibrous dysplasia and malignant adamantinoma of the
Roy 0. van der HeuI2 tibia is challenging because of the impact the diagnosis has on the choice of treatment
Herman M. Schuttevaer1 (none or extensive surgery). The histologic and pathologic similarities of the lesions and
Dirkjan Kuipers1 the controversial relationship between fibrous dysplasia, osteofibrous dysplasia, and
adamantinoma complicate the matter. We found a large overlap of histologic features
in lesions considered either fibrous dysplasia or osteofibrous dysplasia on the basis of
the radiologic findings. The purpose of this study was to determine the value of the
plain radiograph of the lower leg in combination with clinical findings to differentiate the
benign from the malignant condition The clinical symptoms, radiographs, and histologic
slides of 46 patients with fibrous dysplasia and 22 with adamantinoma in the tibia were
reviewed retrospectively. In only one of 12 patients with radiologic or histologic char-
acteristics of osteofibrous dysplasia were both radiologic and histologic criteria for the
diagnosis present. A linear discriminant analysis was performed on six clinical (age,
spontaneous pain, pain after trauma, swelling only, pain and swelling, and bowing
deformity) and 25 radiologic signs. Fibrous dysplasia and its variant osteofibrous
dysplasia could be identified correctly in 87% (40 of 46 patients) and adamantinoma in
95% (21 of 22 patients) by using the patient’s age and four radiologic signs. When
results from the discnminant analysis of a randomized subgroup of patients (32) were
used on the other subgroup (36 patients), fibrous dysplasia was correctly identified in
84% (21 of 25) and adamantinoma in 82% (nine of 11). Fibrous dysplasia is more
prevalent than adamantinoma in a young patient, when radiographs show a ground-
glass appearance and anterior bowing and when there is no multilayered periosteal
reaction and moth-eaten destruction.
When radiologic signs and the patient’s age are combined, fibrous dysplasia and
adamantinoma can be discriminated in a high percentage of patients.
The tibia is one of the preferential sites of fibrous dysplasia, a benign skeletal
developmental anomaly of bone-forming mesenchyma [1 -4]. A related condition
called osteofibrous dysplasia or ossifying fibroma is found exclusively in the tibia
or fibula [5-7]. Adamantinoma is a rare malignant neoplasm of bone that is typically
found in the tibia [8-i 1].
Differentiation between these three lesions is important in planning therapy.
Because fibrous dysplasia and adamantinoma both have large amounts of fibrous
stroma, radiologic and histologic differentiation between the two is difficult [1 4, ,
Received March 30, 1990: accepted after revi-
sion November 20, i990. 9-i 3]. The matter is further complicated by the fact that the relationship between
I Department of Radiology, Bldg. I, C2-S, Uni- fibrous dysplasia, osteofibrous dysplasia, and adamantmnoma is controversial [5-
versity Hospital Leiden, Rijnburgerweg 10, 2333 7, 1 2, 1 3]. The purpose of this study is to determine if clinical or radiologic features
AA Leiden, the Netherlands. Address reprint re can be used to differentiate the two benign disorders from adamantinoma.
quests to J. L. Bloom.
2 Department of Pathology, University Hospital
Dijkzicht Rotterdam, Dr. Molewaterplein 4, 3015
Materials and Methods
GD Rotterdam, the Netherlands.
036i-803x/91/1565-1017 We reviewed the files of the Registry of the Netherlands Committee on Bone Tumors,
0 American Roentgen Ray Society which contained 7500 files on patients at the time of the study. Although most new patients
1018 BLOEM ET AL. AJR:156, May 1991
2
E
z
No. (%)
Finding
Fibrous Dysplasia Adamantinoma
(n=46) (n=22)
Results
Fig. 4.-9-year-old boy with fibrous dysplasia who had a fracture after minor trauma. Lateral radiograph shows a lobulated, osteolytic lesion with an
oblique fracture, located centrally in diaphysis.
Fig. 5.-7-year-old girl with fibrous dysplasia and acute onset of pain in lower leg. Lateral radiograph shows two lesions in tibia and one in fibula.
Lesion in proximal part of tibia is central; lesion in distal part of tibia is eccentric. All three lesions are osteolytic with marked septation. Lesions are
expansive and involve cortical bone. Anterior bowing of tibia is present.
Fig. 6.-i-year-old infant with pseudarthrosis and bowing deformity of lower leg. Lateral radiograph shows a defect in fibula with narrowing of marrow
canal and tapering of both ends. Lesion in tibia is cystic and is characterized by anterior bowing and valgus deformity (not shown). Diameter of tibia is
enlarged by callus formation. Tibial fracture is consolidated but still visible. Histologic diagnosis was fibrous dysplasia.
Fig. 7-19-year-old woman with adamantinoma who had pain after trauma. Lateral radiograph shows multiple lobulated osteolytic lesions in both tibia
and fibula. Lesion in proximal part of tibia is eccentric; lesions in midshaft and distal part of tibia are central. Cortical bone is thinned at endosteal side.
Penosteal bone formation resulted in an intact neocortex, which confines lesions.
One of the patients with a bowing deformity (Figs. 2 and 5) peniosteal reaction, moth-eaten destruction, anterior bowing,
had infantile pseudarthrosis (Fig. 6). Neither this patient nor skip lesions in tibia, and additional lesions in fibula) were used,
his family had stigmata of neurofibromatosis. No tumor, but fibrous dysplasia was identified correctly in 87% (40 of 46)
cellular fibrous tissue with woven bone rimmed by osteo- and adamantinoma in 95% (21 of 22). Six patients with fibrous
blasts, was found. Lamellar bone was present in the peniph- dysplasia and one with adamantinoma were not identified
ery. The osteoblastic activity was graded as high. correctly. Use of the results from the discriminant analysis of
Patients with fibrous dysplasia could be identified in 89% one of the randomized patient groups (32 patients) on the
(41 of 46 patients) and patients with adamantinoma could be other (36 patients) yielded the following results: fibrous dys-
identified in 95% (21 of 22) of cases when age, presenting plasia was correctly identified in 84% (21 of 25) and adaman-
symptoms, and all 25 radiologic signs (Table 1) were sub- tinoma in 81 % (nine of 1 1).
jected to a discniminant analysis. Five patients with fibrous The five signs with the greatest impact on making a diag-
dysplasia and one with adamantinoma were not identified nosis were, in order of decreasing importance, age, ground-
correctly. The one patient with histologic and radiologic signs glass appearance with or without ossification or septation (in
of osteofibrous dysplasia was identified as having fibrous favor of fibrous dysplasia), multilayered lamellar peniosteal
dysplasia and not adamantinoma. Fibrous dysplasia was iden- reaction (in favor of adamantinoma), moth-eaten destruction
tified correctly in 84% of cases (21 of 25) and adamantinoma (in favor of adamantinoma), and anterior bowing (in favor of
in 73% (eight of 1 1) when all radiologic and clinical signs used fibrous dysplasia). The other radiologic and clinical signs were
in a discniminant analysis on a randomized subset of patients not important in differentiating fibrous dysplasia from ada-
(32 patient) subsequently were evaluated on the other subset mantinoma.
(36 patients). None of the patients with adamantinoma had distant me-
When our selected signs (pain after trauma, age, ground- tastases on conventional chest radiographs at the time of
glass appearance with and without densities, mutilayered presentation. Later, metastases developed in the lung in three
AJR:156, May 1991 TIBIAL FIBROUS DYSPLASIA VS ADAMANTINOMA 1021
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Fig. 8.-6i-year-old man with adamantinoma and pain in tibia of 2 month’s duration. Lateral radiograph shows intramedullary osteolytic lesion and
endosteal thinning of cortex. Although bone destruction is partly geographic, especially in proximal and most distal parts, a local area with moth-eaten
destruction can be seen (arrows).
Fig. 9.-37-year-old man with fibrous dysplasia and pain in lower leg for 18 months. Anteroposterior radiograph shows typical ground-glass appearance.
Endosteal scalloping is present. Newly formed cortex (ballooning) confines lesion.
Fig. 10-34-year-old woman with an adamantinoma and pain in lower leg for 3 months.
A, Lateral radiograph shows a large expansive intracortical lesion and scalloping of endosteal margin of cortex. Intralesional calcifications can be seen.
B, Radiograph 8 months later shows enlargement of tumor and soft-tissue extension.
patients, and osseous and lymph node metastases developed relation of fibrous dysplasia to osteofibrous dysplasia, also
in another one. In one patient, a solitary liver metastasis was called ossifying
fibroma, and adamantinoma is controversial
found. In these five patients, at least one metastasis was [5-8, 13, 1 9]. The name osteofibrous dysplasia [1 6] is pre-
proved histologically. The disease-free interval in these five ferred to ossifying fibroma [7] because it better reflects the
patients ranged from 6 to 1 3 years. relation with fibrous dysplasia and does not cause confusion
with ossifying fibroma of the mandible and maxilla, which is a
nonrelated entity [20, 21 ]. Osteofibrous dysplasia has been
Discussion described as a bone-forming lesion located in the ventral,
intracortical area of the tibial shaft with histology different
Differentiation between fibrous dysplasia and adamanti- from fibrous dysplasia [5, 6, 19]. Contrary tofibrous dysplasia,
noma may be difficult when the two conditions are confined the formed, woven bone trabeculae in osteofibrous dysplasia
to the tibia because their radiologic appearance is similar. are rimmed by cuboidal osteoblasts.
Histologic findings for the two entities may be similar and, In our patients, radiologic signs of osteofibrous dysplasia
according to some authors, may even be related [1 , 4, 9-1 1, or ossifying fibroma (eight patients) and histologic evidence
16]. of high osteoblastic activity of osteofibrous dysplasia (five
Fibrous dysplasia may be monostotic (70-80%) or polyos- patients) were combined in only one patient. In lesions con-
totic (20-30%); only 2-3% of polyostotic cases are associ- sidered radiologically to be fibrous dysplasia or osteofibrous
ated with endocninopathy such as the McCune-Albnight syn- dysplasia, a marked remodeling activity was found; overlap
drome [1 -3]. Any bone may be affected, but the tibia is one was large and no basic difference was seen. Although osteo-
of the most frequently involved bones. Others are the rib, blastic rimming is not a feature of classic fibrous dysplasia,
femur, craniofacial bones, and humerus [1 4]. The true prey- , plump, elongated cells rimming woven bone and layering of
alence of fibrous dysplasia is not known because the lesion lamellar bone can be found in fibrous dysplasia [22]. We think
may be asymptomatic and thus may escape detection. The that ossifying fibroma or osteofibrous dysplasia is not a well-
1022 BLOEM ET AL. AJR:156, May 1991
defined entity [7, 8, 19] but an active variant of fibrous the patients with an adamantinoma (Fig. 2) warrants some
dysplasia, an opinion shared by other authors [5, 6, 13]. constraint in using this criterion.
The prevalence of adamantinoma is estimated to be 0.1% The presence of infantile or congential pseudarthrosis,
of primary bone tumors [8]. A less frequently used synonym which was present in one of our patients (Fig. 6), allows the
is malignant angioblastoma. Typically, adamantinoma is found exclusion of adamantinoma from the list of differential diag-
in the tibia, but the tumors may occasionally be found at other noses [4, 1 7]. As was the case in our patient, on histologic
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sites [9]. Although the histology of adamantinoma is reminis- examination, the area of pseudarthrosis may not contain
cent of that of ameloblastoma of the jaw, sometimes called tumorous tissue but only fibrous tissue with increased osteo-
adamantinoma of the jaw, no evidence has been reported blastic activity.
that suggests a relationship between adamantinoma of the Although patients with fibrous dysplasia more often had
tibia and ameloblastoma of the jaw [8-i 1]. Usually fibrous additional lesions in the fibula (1 3%, Fig. 5) than did patients
dysplasia and adamantinoma have clearly different histologic with adamantinoma (5%, Fig. 7), these additional lesions were
features. However, large fields of fibrous stroma (like those a relatively insignificant sign in differentiating between the two
that occur in fibrous dysplasia or osteofibrous dysplasia) may lesions. In their review of the literature, Moon and Mori [9]
mask adamantinomous tissue [1 2, 13]. We encountered this also found simultaneous localizations of adamantinoma in the
problem in four of our patients. The diagnosis of adamanti- tibia and fibula in 5% of their 200 reviewed patients. Solitary
noma could be made with confidence in these patients be- adamantinoma of the fibula may occur [18].
cause we included in this study only patients with ample Entirely intracortical localizations were seen in both fibrous
material available for histologic examination. Immunohisto- dysplasia (Fig. 3) and in adamantinoma (Fig. 1 0). This sign is
chemistry and electron microscopic demonstration of cyto- also not important in differentiating fibrous dysplasia from
keratins is helpful in identifying adamantinoma, especially in adamantinoma.
equivocal cases [14]. No metastases were found on chest radiographs of our
Depending on the signs used, fibrous dysplasia could be patients at presentation. Because metastases develop late in
identified correctly in 84% and adamantinoma in 73-82% of the course of adamantinoma, the absence of metastases
our patients. We used discriminant analysis and evaluated does not assist in making a differential diagnosis [1 1 1 2, 23, ,
3. Lichtenstein L, Jaffe HL. Fibrous dysplasia of bone. Arch Patho! Lab Med 17. Resnick D. Additional congenital or heritable anomalies and syndromes.
1942:3:777-816 In: Resnick D, Niwayama G, eds. Diagnosis of bone and joint disorders,
4. Wilner D. Fibrous dysplasia of bone. In: Wilner D, ed. Radiology of bone 2nd ed. Philadelphia: Saunders, 1988:3540-3595
tumors and allied disorders. Philadelphia: Saunders, 1982:1443-1581 18. Sowa DT, Doriman HD. Unusual localization of adamantinoma of long
5. Campbell CJ, Hawk T. A variant of fibrous dysplasia (osteofibrous dyspla- bones. J Bone Joint Surg (Am] i986:68-A:293-296
sia). J Bone Joint Surg (Am] i982;64-A:231-236 19. Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J
6. Nakashima Y, Yamamuro T, Fujiwara Y, Kotoura Y, Mori E, Hamashima Bone Joint Surg (Am] i981;63-A:367-375
Y. Osteofibrous dysplasia (ossifying flbroma of long bones). Cancer 20. Waldron CA. Fibro-osseous lesions of the jaws. J Oral Maxll!ofac Surg
1983:52:909-914 1985:43:249-262
7. Kempson RL. Ossifying fibroma of the long bones. Arch Patho! Lab Med 21 . Eversole LR, Leider AS, Nelson K. Ossifying fibroma: a clinicopathologic
1966:82:218-233 study of sixtyfour cases. Oral Surg Oral Med Oral Patho! 1985:60:
8. Mm-a JM. Adamantinoma and osteofibrous dysplasia. In: Mirra JM, ed. 505-511
Bone tumors. Philadelphia: Lea & Febiger, 1989: 1203-1 233 22. Kransdorf MJ, Moser RP, Gilkey FW. From the archives of the AFIP:
9. Moon NF, Med H. Adamantinoma of the appendicular skeleton-updated. fibrous dysplasia.
RadioGraphics 1990:10:519-537
C!in Orthop 1986:204:215-237 23. Schneider H, Enderle R. Zur Differentialdiagnose eines metastasierenden
1 0. Resnick D, Kyriakos M, Greenway GD. Tumors and tumor-like lesions of Adamantinoms der Tibia und Fibula. Arch Orthop Trauma Surg
bone: imaging and pathology of specific lesions. In: Resnick D, Niwayama i979;94: 143-1 49
G, eds. Diagnosis of bone and joint disorders, 2nd ed. Philadelphia: 24. Cohn BT, Brahms MA, Froimson A. Metastasis of adamantinoma sixteen
Saunders, 1988:3616-3889 years after knee disarticulation. J Bone Joint Surg (Am] 1986:68-A:
1 1 . Wilner D. Adamantinoma of long bones. In: Wilner D, ed. Radiology of 772-776
bone tumors and allied disorders. Philadelphia: Saunders, 1982: 25. Daffner RH, Kirks DR, Gehweiler JA, Heaston DK. Computed tomography
2387-2410 of fibrous dysplasia. AJR 1982:139:943-948
1 2. Weiss SW, Dorfman HD. Adamantinoma of long bone: an analysis of nine 26. Dominguez R, Saucedo J, Fenstermacher M. MRI findings in osteofibrous
new cases with emphasis on metastasing lesions and fibrous dysplasia- dysplasia. Magn Reson Imaging 1989:7:567-570
like changes. Hum Patho! i977;8:141-153 27. Zeanah WR, Hudson TM, Springfield DS. Computed tomography of ossi-
1 3. Schajowicz F, Santini-Araujo E. Adamantinoma of the tibia masked by tying fibroma of the tibia. J Comput Assist Tomogr i983:7(4):688-691
fibrous dysplasia. C!in Orthop 1989:238:294-301 28. Utz JA, Kransdorf MJ, Jelinek JS, Moser RP, Berrey BH. MR appearance
1 4. Mori H, Yamamoto 5, Hiramatsu K, Miura T, Moon NF. Adamantinoma of of fibrous dysplasia. J Comput Assist Tomogr 1989:13(5):845-851