British Journal of Oral and Maxillofacial Surgery (1986) 24, 442-447 0 1986 The British Association of Oral and Maxillofacial Surgeons
DESMOPLASTIC J. HIETANEN, P.-L. Department
FIBROMA
INVOLVING
THE MANDIBLE
LUKINMAA, P. E. B. CALONIUS and
of Oral Pathology,
Institutes of Dentistry. Helsinki. Finland
0.
KASSILA
University of Helsinki,
Summary. Desmoplastic fibroma is a rare, benign, fibroblastic tumour arising intra-osseously. A a 13 year-old male is described. A tumour, situated between the roots of the right mandibular and premolar, was excised in 1984. Morphologically, the characteristics of the tumour conformed those of a desmoplastic fibroma. The differential diagnosis of desmoplastic fibroma with reference histopathological and ultrastructural appearance of the tumour is discussed.
case in
canine well to to the
Introduction Desmoplastic fibroma is a benign primary intra-osseous tumour originating in connective tissue. This rare tumour, closely resembling the soft tissue desmoid. was first described by Jaffe (1958). The first case of desmoplastic fibroma of the jaw was presented by Griffith and Irby (1965). In the mandible, the tumour occurs predominantly in the region of the molar/ramus (Freedman et al., 1978). It occurs extremely rarely in the maxilla (Pindborg 81 Hjorting-Hansen, 1974; Sood & Chatterjee, 1975; Summers & Matz, 1976; Eisen & Butler, 1984). Desmoplastic fibroma of the jaw usually grows slowly, sometimes causing pain and loosening of teeth. In most cases, the tumour occurs in the second decade of life with no obvious predilection for either sex (Freedman et al., 1978). The same age and sex distribution are found in desmoplastic fibroma of the entire skeleton (Sugiura, 1976). There is a strong tendency for the tumour to recur. Radiographically, desmoplastic fibroma of the jaw appears as an unspecific, well-delineated or irregular radiolucency with sclerotic margins. The bony cortex of the mandible is expanded and thin. The pattern of bone destruction may sometimes suggest the presence of a malignancy. Histopathologically, desmoplastic fibroma is characterised by more or less cellular areas of either small or large, but always uniform, fibroblasts scattered in a stroma of varying amounts of collagen. The nuclei may be oval or elongated (Fisker & Philipsen. 1976; Freedman et al., 1978). In most cases, the histopathological appearance of the tumour is characteristic enough to enable its distinction from other benign fibrous lesions, but distinguishing it from a highly differentiated fibrosarcoma may be difficult. Treatment of the tumour should primarily be conservative, though its tendency to soft tissue invasion and recurrence has to be recognised. In order to examine desmoplastic fibroma of the jaw further we present a case and discuss the entity with special reference to the histopathological and ultrastructural characteristics of the tumour. Case Report In April the buccal
1984. an asymptomatic aspect of the alveolar (Received
swelling (diameter about 10 mm) was found on crest between the right mandibular canine and
21 June 1985; 442
accepted
8 July
1985)
DESMOPLASTIC
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Fig.
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Fig. 2
3
Fig. 4
Figure l-Radiograph of the desmoplastic fibroma displacing the roots of the adjacent teeth. The outline of the tumour is poorly demarcated. Figure 2-Photomicrograph of the desmoplastic fibroma showing the uniform appearance of the tumour. Haematoxylin and eosin x 83. Figure 3-Photomicrograph of the desmoplastic fibroma showing the elongated or oval nuclei of the fibroblasts. Haematoxylin calcification occluding the capillary lumen can be seen in the and eosin x 207. Figure L-Intravascular desmoplastic fibroma. Haematoxylin and eosin x 336.
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premolar of a healthy 13 year-old male during routine dental treatment. The radiograph showed bone destruction with irregular margins between the roots of the teeth. The lesion caused displacement of the roots but no root resorption (Fig. 1). The medical history was noncontributory. The patient was referred for further treatment by his dentist. As there were no complaints, he did not present himself for treatment until September 1984. Radiographic examination suggested a slight enlargement of the lesion. The tumour was excised under local anaesthesia and submitted for histopathological examination. Serial paraffin sections were prepared and stained with haematoxylin and eosin. Each section was seen by two of the authors (J.H. and P.-L.L.). The tumour was characterised by uniform fibroblasts scattered in a stroma of abundant collagen (Fig. 2). The nuclei were either plump and large or elongated in appearance (Fig. 3). There was no pleomorphism nor hyperchromatism, and no mitoses were seen. Nor were odontogenic epithelium, giant cells, xanthomatous areas or haemosiderin deposits visible. Hard tissue in the form of bone was visible in the peripheral areas of the tumour only. Additionally, intravascular calcification, sometimes causing complete occlusion of the capillary lumen, was present (Fig. 4). The tumour was not encapsulated. A sample of the tumour was removed from the paraffin block for ultrastructural study. The paraffin was removed by xylol immersion. The sample was then rehydrated in alcohol series decreasing to buffer, postfixed in osmium tetroxide, dehydrated through increasing grades of alcohol and embedded in LX-112. The ultrathin sections were stained with uranyl acetate and lead citrate. All the micrographs were taken with a JEOL 12OOEX electron microscope operating at 60 kV. The sections were made in the Department of Electron Microscopy, University of Helsinki. For comparison, an identical method was used to study a sample from a patient having a histological diagnosis of dermatofibroma. This tumour was removed from the skin of the back in a 37 year-old man. Ultrastructurally, the cells in the desmoplastic fibroma were either elongated with large oval nuclei or stellate, with large corrugated nuclei. Heterochromatin was evenly distributed throughout the nucleus. Many dilated sacs of rough endoplasmic reticulum were visible in the cytoplasm. The stroma was composed of thick collagen bundles running perpendicular to each other (Fig. 5). However, the cells in the dermatofibroma were rounded, with a rounded or oval nucleus in the centre of the cell. The heterochromatin was concentrated around the periphery of the nucleus. The cytoplasm of some of the cells showed abundant endoplasmic reticulum. The areas studied contained many cells and the amount of stroma was considerably less than in the desmoplastic fibroma. The stroma was composed of collagen bundles, but their perpendicular arrangement was not so clear as in the desmoplastic fibroma (Fig. 6). Discussion Desmoplastic fibroma is morphologically a benign tumour. However, it may be locally aggressive, which is sometimes suggested by its radiographic appearance. The tendency of the tumour to recur is generally recognised. The abundantly cellular tumours with large nuclei have a considerably higher recurrence rate than those with few cells and uniformly small nuclei (Rabhan & Rosai, 1969). The histopathological criteria for desmoplastic fibroma appear to be inconsistent. Jaffe (1958) originally included in this entity only those tumours with
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Fig. 6 Figure 5-An electron micrograph of desmoplastic fibroma. The fibroblast has a large nucleus with prominent nucleolus. Dilated sacs of endoplasmic reticulum can be seen in the cytoplasm. Thick perpendicular collagen bundles are present in the stroma. The bar is 1 ym long x 7100. Figure &An electron micrograph of dermatofibroma. Closely packed, rounded fibroblasts are seen with peripheral arrangement of heterochromatin in the nucleus. The number of collagen bundles in the stroma is small. The bar is 1 pm long X 4900.
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uniformly small fibroblasts in a stroma of abundant collagen, and classified tumours with more cells and large nuclei, as well-differentiated fibrosarcomas, although they are completely benign in appearance. The WHO classification (Schajowicz et al.,1972) recognises tumours with few cells, with ovoid or elongated nuclei and abundant collagen formation as being desmoplastic fibroma. Rich cellularity. pleomorphism or mitoses are not accepted. Several authors seem to accept greater variation in the histopathological appearance of desmoplastic fibroma (Whitesides & Ackerman, 1960; Rabhan & Rosai, 1968; Freedman et al., 1978), also including in this entity tumours with a variable number of cells, size and form of cells and nuclei. Nor is classification of the tumour uniform (Fisker & Philipsen, 1976). The absence of hard tissue formation usually allows desmoplastic fibroma to be distinguished from fibro-osseous lesions (Fisker & Philipsen, 1976). In the present tumour, the existence of hard tissue in the form of bone in the peripheral areas only, as well as intravascular calcification, does not justify a diagnosis of ossifying fibroma. The giant cells, xanthomatous areas and haemosiderin deposits characterising non-ossifying fibroma of the jaws (Elzay et al., 1984), are not found in desmoplastic fibroma (Freedman et al., 1978). It has been stressed that distinguishing between desmoplastic fibroma and well-differentiated fibrosarcoma may be particularly difficult (Lichtenstein, 1972). Desmoplastic fibromas have at first been incorrectly diagnosed as highly differentiated fibrosarcomas (Jaffe, 1958; Whitesides & Ackerman, 1960) and vice versu (Dahlin, 1967; Slootweg & Miiller, 1983). In the present case, the clinical characteristics of the tumour were similar to those in previously described cases. However, in our case, the location of the tumour was exceptional, as Freedman et al. (1978) have found that only 9% of 26 desmoplastic fibromas occurred anterior to the mandibular premolars. At the same time. only five tumours of 22 produced irregular radiolucency on X-ray, but this was also found in our case. Histopathologically. the present tumour had uniformly-sized fibroblasts with plump and oval, or elongated nuclei evenly scattered in an abundantly collagenous stroma. No mitoses or abnormal cells could be seen. Ultrastructurally, the desmoplastic fibroma showed features which could distinguish it from dermatofibroma of the skin. Our electrom microscopic findings in desmoplastic fibroma were in accord with those of Sugiura (1976). The histopathological appearance of the tumour conformed well to the criteria established for desmoplastic fibroma by Rabhan and Rosai (1968), Whitesides and Ackerman (1960), and Freedman et al. (1978). Also, our diagnosis was supported by the absence of capsule formation. References Dahlin, D. C. (1967). Bone Tumors, 2nd Ed.. pp. 212-221. Springfield. 111.: Charles C. Thomas. Eisen, M. Z. & Butler, H. E. (1984). Desmoplastic fibroma of the maxilla: report of case. Journal ofthe Americun Den& Associafion, 108, 60X. Elzay, R. P.. Mills, S. & Kay, S. (19X4). Fibrous defect (nonossifying fibroma) of the mandible. Orul Surgery, Oral Medicine, Oral Pathology. 58, 402. Fisker, A. V. & Philipsen, H. P. (1976). Desmoplastic fibroma of the jawbones. fntrrntrriona/JournaI of Oral Surgery. 5, 285. H. (1978). Desmoplastic fibroma Freedman, P. D.. Cardo. V. A., Kerpel, S. M. & Lumcrman. (fibromatosis) of the jawbones. Report of a case and review of the literature. Orul Surgery, Oral Medicine, Orul Pathology, 46, 3%. Griffith, J. G. & Irby, W. B. (196.5). Desmoplastic fibroma. Report of a rare tumor of the oral structures. Orul Surgery, Orul Medicine, Oral Pathology, 20, 269.
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H. L. (1958). Tumors and Tumorous Condirions of theBones rind Joints. pp. 29%313. Philadelphia: Lea and Febiger. Lichtenstein, L. (1972). Bone Tumors, 4th Ed., p. 132. St. Louis: C. V. hlosby. Pindborg, J. J. & Hjorting-Hansen, E. (1974). Allus of Diseases of the Jaws, pp. 6465. Copenhagen: Munksgaard. Rabhan, W. N. & Rosai, J. (1968). Desmoplastic fibroma. Report of ten cases and review of the The Journal of Bone und Joint Surgery, SOA, 487. literature. Schajowicz, F., Ackerman. L. V. & Sisson, H. A. (1972). Hisiologicul fyping of bone tumowx. International histological classificcrtion of fum0ur.y. No. 6. Geneva: World Health Organization. Slootweg, P. J. & Mtiller, H. (1983). Central fibroma of the jaw. odontogenic or desmoplastic. A report of five cases with reference to differential diagnosis. Owl Swgery, Oral Medicine. Oral Pufhology, 56, 61. Sood, V. P. & Chatterjee, A. K. (1975). Desmoplastic fibroma of the maxilla. Journul ofLaryngo/ogy und Otology, 89, 329. Sugiura. I. (1976). Desmoplastic fibroma. Case report and review of the literature. The Journal of Bone und Joint Surgery, 50A, 126. Summers. L. & Matz, L. R. (1976). Recurrent desmoplastic fibroma. Internarionul Journal of Orul Surgery, 5, 100. Whitesides. T. E. & Ackerman, L. V. (1960). Desmoplastic fibroma. A report of three cases. The Journal of Bone and Joint Surgery, 42A, 1143.
Jaffe,