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References Alth, G., G. Canigiani, R. Fries, H. Neumann: Zur

computergerechten Dokumentation der Tumoren des Viszerokraniums. (Computererizable Documeitaon of Tumours of the Viscerocranium.) Mschr. Ohrenheitk. 105 (1971) 463 Chiang, Ch.: Introduction to Stochastic Processes in Biostatics. Wiley, New York 1968 Fries, R., H. Grabner, H. Langer Jun., ]. Lejhanee, B. Kriinzl, H. Neumann, K. S~hroll, K. H. Wil[ert:

Comparative Investigation on the CIassification of Carcinoma of the Oral Cavity. J. max.-fac. Surg. 1 (1973) 222 Spiessl, B., R. Fries: Das TNM-System als Grundlage ffir die Klassifikation des Mundh6hlenkarzinoms. (The TNM-System as a basis for the classification of carcinoma of the oral cavity.) Dtsch. Zahn-, Mund- u. Kieferheilk. 54 (1970) 179 Spiessl, B., ]. von Albert, K. Bitter, W . Busch, H. von Domarus, D. Gasser, H, Grasser, W . Hahn, ]. E. Hausamen, H. Koch, H. Mehnert, H. Meissel, H. D. Pape, ]. Prein, F. Schrgder, P. Sehulz, W . Steinhilber, E. Waldhart: A Clinical Examination on

Value Determination of the TNM-Classification of the Carcinoma of the Buccal Cavity. Z. Krebsforsch. 80 (1973) 83 Watson, F. R.: Statistical Method in Cancer Research. In: J. S. Spratt, W. L. Donegen (Ed.): Cancer of the Breast. Saundcrs, Philadelphia 1967

Univ.-Doz. Rudolf Fries, M.D., D.M.D, Chief of the Department of MaxilIo-FacialSurgery, General State Hospital of the City of Linz, Kranhenhausstrasse9, A-4020 LinzlDonau, Austria Collaborating clinics and departments: Clinic and Polyclinic for Oral and Maxillo-Facial Surgery (Chief: Prof. E. W. StelnMiuser, M.D., D.M.D.) University a~ ErIangen-Nnremberg, W. Germany Clinic and Polyclinic for Oral and Maxillo-Facial Surgery (Director. Pro[. W. Sd~illi, M.D., D.M.D,) University of Freiburg. i. Br., W. Germany University Clinic for Oral and Maxillo-Facial Surgery (Chief: Prof. H. KOle, M.D., D.M.D.) Graz, Austria Clinic and Polyclinic for Oral and Maxillo-Facial Surgery (Chle[: Priv.-Doz. P. S~hulz, M.D., D.M.D.) University of Heidelberg, W. Germany University Clinic for Oral and Maxillo-Facial Surgery (Chief: Prof. H. Wunderer, M.D., D.M.D.) Innsbru&, Austria Department of Maxillo-Facial Surgery General State Hospital (Chief: Univ.-Doz. R. Fries, M.D., D.M.D.) Linz, Austria University Clinic for Maxillo-Facial Surgery (Chief: Prof. S. Wunderer, M.D., D.M.D.) Vienna, Austria Data Processing Centre (Chief: Prof. G. Grabner, M.D.), Medical School, Univeslty of Vienna, Vienna, Austria

J. max.-fac. Surg. 4 (1976) 238-241 © Georg Thierne Verlag, Stuttgart

Desmoplastic Fibroma of the Mandible Case report

Luis Calatrava, Manuel Donado Department of Surgical Stomatology, University of Madrid and Maxillo-Facial Surgical Service of the Central A i r Force Hospital (Head: Prof. L. Calatrava, M.D., D.M.D.), Madrid, Spain

Summary This report describes the case of a 2-year-old girl with desmoplastic fibroma of the mandible, with swelling in the region of the mandibular angle which had been wrongly diagnosed several times. A biopsy was interpreted as showing a low-grade fibrosarcoma. The patient then received treatment with cytotoxic drugs, and later a mandibular hemi-resection was performed. The postoperative diagnosis was Jaf[g's desmoplastic fibroma (non-osteogenic). The age of the patient, the rapid development of the tumour and tile accompanying pain suggested a sarcoma, and the first pathological examination seemed to provide confirmation. The post-o,perative course was very favor-

able, and the patient is considered to be clinically cured, after one year.

Key-Words: Desmoplastic fibroma. Introduction Fibromas are b e n i g n tumours of the connective tissues. The appear on the skin, in the mucous membrane, bones, in various organs and on the a b d o m i n a l wall (desmoid fibroma of the abdominal muscles, R i b b e r t and H a m p e r l 1959). I n 1958, ] a f [ d described the desmoplastic fibroma of bone as a rare, benign tumour consisting

Desmoplastic Fibroma of the Mandible

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Fig. 1 Girl of two years of age with tumefaction in the left angle of the mandible.

Fig. 2 Posterior-anterior radiograph, Lysis in the body and left ascending portion.

of small, mononuclear fibroblasts and an intercellular substance very rich in collagenous fibres having a dense, fibrous appearence, similar to the desmoid fibroma of the abdominal wall. The desmoplastic fibroma of bone is so rare that a review made by Dahlin and Hoover (1964) at the Mayo Clinic of 3,000 tumours, revealed only two cases. Jaf[d has described five cases; three in the tibia, one in the femur and one in the scapula; he reported none in the jaws. Cases of desmoplastic fibroma of bone have also been described by Cohen and Goldenberg (1965), Hardy and Lehrer (1967). Whitesides and Acherman (1960) have compiled 36 cases from the literature.

swelling continued to progress towards the angle of the mandible, becoming harder and more tender, but not causing spontaneous pain. It was then diagnosed and treated as a cellulitis of odontogenic origin. Nevertheless, the lesion continued to enlarge, becoming spontaneously painful. Clinical examination: A mass measuring approximately 6 x 4 cm was found at the left angle of the mandible. The mass was hard, and extended from the posterior third of the horizontal portion of the mandible toward the ascending ramus. The colour of the overlying skin was normal, and the skin was not bound to the deep layers (Fig. 1). Intrabuccal exploration revealed obliteration of the lower buccal sulcus and the tumour extended to the anterior pillar of the pharynx and toward the palate and maxillary tuberosity. Individual and family antecedents gave no relevant information. Haematological investigations showed a slight rise in the numbers of lymphocytes and monocytes, and an increase in the sedimentation rate. The pre-operative impression was that the lesion was a malignant osteolytic tumour of the ascending portion of the mandible (possibly a sarcoma. or Ewing's tumour). Radiographic [indings: Radiographs showed a definite loss of bone of the body and left ascending portion of the mandible with a pseudocystic halo, These findings were confirmed by a posterior-

In the maxillo-facial region, there is a case of a boy 13-years-old described j in 1968 by Rabban and Rosai, one by Gri[fith and Irby in 1965 of a boy of 8 years, another by Hinds et al. 1969 of a boy 15-years-old and finally one by Bullens et al. 1975 of a girl t5-months-old. All were in the mandible. Clinical Case The Patient, M.G.D., 2-years-old, attended the Maxillo-Facial Surgery Service of the Central Air Force Hospital for the first time on 10th May 1974. Approximately two months earlier a diffuse enlargement had been observed in the left pre-auricular region. This was diagnosed and treated as parotitis at another clinic, but the i6 J. max.-fac. Surg. 4/76

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L. Calatrava, M. Donado

Fig. 3 Desmoptastic fibroma (Jaff6): General aspect. Evident resorption of bone. HaematoxilinEosin, x20.

Fig. 4 Desmoplastic fibroma (Jaff6): Detail of tumour: Ratio of cells to collagen fibres. Collagen as prominent feature. Haematoxilin-Eosin, x200.

anterior radiograph (Fig. 2). The Waters projection showed an absence of the left ascending portion of the mandible. The radiographs also suggested a slight increase in the digital impressions of the calvarium. There was no evidence of thoracic metastases.

external carotid artery. The weekly dosage schedule was as follows:

Clinical appearance and histological examination of biopsy A biopsy was performed on 16th May. Upon opening the mucous membrane, it was observed that the periosteum was raised at the point of incision. Once this was opened, it was seen that the bone had been replaced by a soft mass of greyish, but nonhaemorrhagic tissue. Macro: Irregular fragment of tissue 1.5 cm in diameter, of soft, fibrous consistency; the tissue was, yellowish white with some haemorrhagic streaks. Micro: The biopsy showed a neoplastic tissue consisting of long, fusiform fibroblasts arranged irregularly, but in parallel fashion; between these, there was immature, dense collagen tissue with clefts formed by narrow capillaries. The collagen bundles sometimes formed whorled patterns. Mitoses were not observed, nor was there any marked anisocytosis. Diagnosis: Fibrosarcoma of low-grade malignancy. The case was presented to the "Tumour Committee" on 22nd May, which decided on the administration of cytotoxic drugs, given via the

Days

1

2

Vincristine (gg) Doxorubicine (Fg)

0.5 0.5

3

4

5

6

10

10

10

10

with intervals of one to two weeks with haematological checks. The treatment was well tolerated. Anaemia and leuco,poenia occurred during cyclical treatment. Pathological T waves (a possible toxic effect of the medication) were observed in the E.C.G. Surgery was considered after the fifth cycle, but was not performed because of the leucopenia. Therefore the drugs were continued for a sixth cycle.

Surgical intervention (19th December): Under general anesthesia (fluotan) a hemimandibulectomy was performed from the canine backwards, via a submandibular incision. One centimeter distal to the osteotomy line in the canine region, the bone appeared to be destroyed, and appearing to be replaced practically by a tumour mass deep to the periosteum. The tumour was rosy white and of a consistency of hard rubber. No areas of ossification could be found macroscopically when the tumour was sliced. The tumour had grown to such an extent that it was necessary to deepen the resection to the point of clearing the zygomatic fossa, reaching by touching and visualizing what seemed to be the trans-

Desmoplastic Fibroma of the Mandible verse process of the atlas. The horizontal and ascending portions of the mandible, the subm a x i l l a r y gland on the same side and the invading mass in the zygomatic-fossa were reseeted, attempting to conserve the periosteum, bearing in mind the tendency to new bone f o r m a t i o n in a patient of this age. Microscopic e x a m i n a t i o n (Figs. 3-5) showed that the tumour was composed of well differentiated fibroblasts with an abundance of collagen fibres: in some areas the fibre bundles were dence, whilst in other areas they showed a w h o r l e d pattern. The cells showed neither hyperchromatism nor mitotic activity. T h e r e was no evidence of osteoid, bone or cartilage formation. T h e periosteum was not affected, and there were no alterations in the s a l i v a r y gland. Post-operative diagnosis: ]affd's desmoplastic fibroma. Progress and present condition: Progress was highly satisfactory and no complications on any kind arose. T h e present condition of the patient is good. Conclusion T h e desmoplastic f i b r o m a of bone is a rare tumour, and few have been described in the jaws. As in the present case, all previously described j a w lesions have been in the mandible. W i t h such a rare tumour, the diagnosis can not be m a d e on

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Fig. 5 Desmoplastic fibroma (Jaff6): Detail of fibroblasts and their relation to collagen. Haematoxilin-Eosin, x350. clinical and r a d i o g r a p h i c grounds, and the presentation is not diagnostic. In addition, the case described here, the r a p i d growth and spontaneous pain suggested a m a l i g n a n t neoplasm, although in the earlier stages an i n f l a m m a t o r y lesion was suspected. This case also illustrates the diagnostic difficulties encountered by the pathologist in the differentiation of certain tumours of fibrous tissue. Once the diagnosis of desmoplastic f i b r o m a of bone has been established, treatment is by careful curettage or b y resection. T h e lesion does not metastasise, and i r r a d i a t i o n is contra-indicated.

References Bullens, R., ]. Boddaert, H. Schautteet, E. Defloor: Fibrome desmoide osseux du maxillaire inffirieur. Cas exceptionnel d'un enfant de quinzc tools. Rev. Stomat. 76 (1975) 45 Cohen, P., R. Goldenberg: Desmoplastic fibroma of bone; report of two cases. J. Bone Jt Surg. 47-A (1965) 1620 Dahlin, D. C., N. W. Hoover: Desmoplastie fibroma of bone; report of two cases. J. Amer. reed. Ass. 188 (1964) 685 GrifIith, J. G., W. B. Irby: Desmoplastic fibroma; report of a rare tumor of the oral structures. Oral. Surg. 20 (1965) 269 Hardy, R., H. Lehrer: Desmoplastic fibroma vs. desmold tumor of bone. Two cases illustrating a problem in differential diagnosis and classification. Radiology 88 (1967) 899 H~nds, E. C., J. N. Kent, R. E. Fechner: Desmoplastic fibroma of the mandible; report of case. J. oral. Surg. 27 (1969) 271

Jaffd, H. D.: Tumours and tumorous conditions of the bones and joints. Lea & F.ebiger, Philadelphia 1964 Rabhan, W. N., ]. Rosai: Desmoplastic fibroma; report of ten cases and review of the literature. J. Bone Jt Surg. 50-A (1968) 487 Ribbert, H., H. Hamperl: Tratado de Patologla General y Anatomla Patoldgica. 7~ Edit. Edit. Labor. 1959 Whitesides, T. E., L. V. Ackerman: Desmoplastic fibroma: a report of three cases. J. Bone Jt Surg. 42-A (1960) 1143

Pro]. L. Calatrava,M.D., Escuela de Estornatolog~a, Faculta de Medlcina, Ciudad Universetaria, Madrid 3, Spain

Desmoplastic fibroma of the mandible: case report.

This report describes the case of a 2-year-old girl with desmoplastic fibroma of the mandible, with swelling in the region of the mandibular angle whi...
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