Oral Oncology 36 (2000) 129±133
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Aggressive cemento-ossifying ®broma of the jaws A. Zupi a,*, A.M. Ruggiero a, L. Insabato b, N. Senghore c, L. Califano a a
Department of Maxillofacial Surgery, School of Medicine and Surgery, ``Federico II'' University of Naples, Via Pansini 5, 8013-1 Naples, Italy b Department of Anatomic Pathology, School of Medicine and Surgery, ``Federico II'' University of Naples, Via Pansini 5, 80131 Naples, Italy c Dentistry and Head and Neck Surgery, Royal Victoria Hospital, Banjul, The Gambia Received 22 June 1999; accepted 1 July 1999
Abstract The cemento-ossifying ®broma is probably the most frequent ®bro-osseous lesion seen by oral pathologists. Occasionally, reports of lesions behaving in an aggresive fashion appear in the literature. At the present time, the relationship if these ``aggressive'' lesions to the ``usual'' ®bromas is unclear. Two cases of ``aggressive'' cemento-ossifying ®broma are reported. Certainly, clinical, radiological, and histological characteristics of aggressive and usual ®bromas do coincide. There are, however, diverging features warranting separation of dierent forms. These mild dierences may be detected during preoperative assessment of the lesion, allowing a diagnosis of aggressive form to be made. # 2000 Elsevier Science Ltd. All rights reserved. Keywords: Cemento-ossifying ®broma; Jaws
1. Introduction The ®bro-osseous lesions are a numerous and heterogeneous group of tumours of the jaws and they pose diculties in classi®cation, diagnosis, and treatment. Among these lesions, the cemento-ossifying ®broma (COF) is probably the most frequent ®bro-osseous lesion seen by oral pathologists and, perhaps, it has had more synonymous than any other jaw lesion [1±5]. It is a benign, well-de®ned unilocular or multilocular ®broosseous tumour with a slowly progressing enlargement of the aected bone; complete surgical resection is necessary to prevent recurrence [6±8]. Occasionally, it may grow to massive size and cause serious cosmetic and functional problems [7] and, rarely, reports of lesions behaving in an aggressive fashion, diagnosed as `aggressive', `juvenile', or `active' COF, appear in the literature [3,9±14]. At the present time, the relationship of these `aggressive' lesions to the `usual' COFs is unclear; whether they actually represent dierent lesions or are simply variants of COFs needs careful documentation of additional cases. * Corresponding author. Present address: Via Michelangelo Schipa 115, 80123 Naples, Italy. Tel.: +39-081-746-2075; fax: +39-081-5453491. E-mail address:
[email protected] (A. Zupi)
To contribute to the de®nition of this lesion, two cases of `aggressive' COF (ACOF) are reported. 2. Case 1 In December 1997, a 24-year-old female was referred to the Department of Maxillofacial Surgery of the School of Medicine of the ``Federico II'' University of Naples (Naples, Italy) with a neoformation of the right hemimandible discovered during a routine radiographic examination. The lesion was a 4545 mm oval radiolucent area, situated in the horizontal portion of the right hemimandible from the second to the third molar area. The corresponding teeth did not appear involved or reabsorbed. Clinically, a 2530 mm oval swelling in the lower buccal sulcus was evident; it was covered by normal mucosa. A mild paraesthesia of the right lip was observed. Computed tomography (CT) revealed an irregularly dense and well-de®ned osteolytic lesion of the right mandibular body. The cancellous bone appeared totally substituted by the lesion; the cortical layers were reabsorbed and expanded. The surrounding soft tissues did not appear to be involved. Surgical removal using enucleation was performed intra orally. Microscopically, a diagnosis of ®brous dysplasia of COF type was made. In July 1998, at the 6-month
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Fig. 1. Case 1: (a) CT scan and (b) radiographic view; surgical specimen of primary lesion showed (c) rounded psammoma-like masses resembling cement and immature bone within unduly cellular ®brous stroma (haematoxylin±eosin, 100); (d) at the periphery of the lesion a proliferating ®broblastic stroma lining a bony trabeculae (haematoxylin±eosin, 100).
follow-up, the patient was suering from a painful 22 cm swelling situated on the right hemimandible. The patient indicated that the swelling had developed in the last month. A radiographic examination showed a 34 cm oval radiolucent area in the right molar region, with an interruption of the lower border of the mandible. A CT scan was performed revealing a large osteolytic lesion in the right hemimandible (Fig. 1a, b). The cancellous bone was totally substituted; the cortical layers were reabsorbed and expanded. The seventh and eighth teeth did not appear reabsorbed, but their roots were involved by the lesion. The surrounding soft tissues did not appear to be involved. A right hemimandibulectomy was performed intraorally, from the bicuspid region to the Spix lingula. The surgical specimen was submitted for histologic examination and a diagnosis of COF was made (Fig. 1c, d). In November 1998, at the routine monthly follow-up, no sign of recurrence was detected.
3. Case 2 A 30-year-old female presented at the Dental Clinic of the Royal Victoria Hospital of Banjul (Banjul, Gambia) on December 1993 with a 2-year swelling of the right mandible. According to the patient it had started as a small swelling and gradually increased in size. There was associated pain initially. The patient had been receiving traditional treatment. There was no signi®cant past medical or dental history. On extra-oral examination there was a huge swelling of the right mandible with part of it protruding through the lips causing lip incompetence (Fig. 2a, b). The swelling extended from the angle of the right mandible to the cuspid region. The swelling was ®rm but not attached to overlying skin. There was no anaesthesia or paraesthesia. On intra-oral examination, mucosa overlying the swelling was not ulcerated. All the teeth on the right mandible were
A. Zupi et al. / Oral Oncology 36 (2000) 129±133
missing except the third molar. The mucosa of the tongue, ¯oor of the mouth, and palate appeared normal. On X-ray examination, the lateral oblique view of the mandible showed a huge radio-opaque mass. The inferior border of the mandible was intact. The patient underwent the operation and the tumour was removed wholly by blunt dissection (Fig. 2c). Histologically, a diagnosis of COF was made (Fig. 2d). The post-operative course was uneventful. At the 10-month follow-up the patient showed a 2-month old swelling of the right side of her lower jaw. 4. Discussion and conclusions It is generally claimed that COFs are lesions which are well separated from adjacent cancellous bone or soft tissue by a thin layer of cortical bone [3,5±7,9]. At surgery they tend to be removed with ease. Some COFs
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behave in an aggressive fashion (ACOFs), reaching massive proportions with extensive cortical expansion [3,6,10±17]. Eversole et al. [3] underline the fact that no de®nitive predictor variables with regard to histopathologic features have been uncovered as an aid in determining the potential for aggressive behaviour or propensity of recurrence. Thus, the main diculty in the management of the ACOF is to distinguish it from the COF and other ®bro-osseous lesions. The literature allows us to reach several conclusions on the ACOF. Age distribution and mean age of the cases warrant the adjective `juvenile'; 60±80% of the cases observed are aged between 5 and 15 years of age and only 21% of the patients are aged over 15 years [5,16]. Johnson et al. [17] suggest that these tumours usually arise in the paranasal sinuses and, secondarily, the surrounding structures are involved. Clinically, the presenting symptom is swelling. In fact, ACOFs are commonly characterised by a progressive and sometimes rapid expansion
Fig. 2. Case 2: (a) frontal and (b) lateral clinical view; (c) surgical specimen; (d) ultrastructural feature (haematoxylin±eosin, 100).
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of the aected area. When the orbital bones and paranasal sinuses are involved, an exophthalmos or bulbar displacement may be observed [6,16]. The radiological features are variable. Depending on the site and the extent of ossi®cation, the uni- or multilocular lesion may be radiolucent or mixed radiolucent±radiodense but there is always a distinct radiodense border; it may show invasion and erosion of the surrounding bone [6,16]. As regards ultrastructural features, Eversole et al. [3] suggest that the histopathological ®ndings of all ®broosseous lesions, including the ACOF, are a spectrum with lamellar bone, woven bone, osteoblastic rimming of osseous trabeculae, spheroid calci®cations, and cementicles. Some COFs do in fact contain prevalent cementum-like calci®cations and others show only bony material, but a mixture of the two types of calci®cation is commonly seen in a single lesion [5,7]. Slootweg and Muller [16] report that the COF contains lamellar bone and cementicles as well as smoothly contoured, cellpoor, curvilinear trabeculae; features that are absent in the ACOF whereas the osteoblastic cords and the loose, active-appearing ®brous stroma that characterise the ACOF are absent in the COF. In their opinion, it appears that histomorphology is speci®c enough to make separate categorisation of ACOFs and COFs possible. The relationship between histological features and clinical behaviour is not suciently clear. Indeed, the next point to clarify is whether there is any reason to distinguish between ACOFs and COFs at all. Lesions have been reported as being removed by radical resection or conservatively by local excision or enucleation with curettage [18±23]. Data from the literature as well as our own follow-up data indicate that all patients have been cured of their tumour, although some required two or more surgical attempts because of recurrent tumour. As there is no dierence in outcome between patients treated in a more limited way and those treated by major surgery, Slootweg and Muller [16] recommend a conservative surgical approach when managing the ACOF. Moreover, the present data do not support the assumption that the ACOF may be locally invasive and fatal [24], and we have not been able to trace any documented report of a case of ACOF transforming into osteosarcoma, a possibility mentioned by Homan et al. [25]. In conclusion, within the group of craniofacial ®broosseous lesions, the ACOF is probably an entity in its own right. Certainly, clinical, radiological, and histological characteristics of ACOFs and COFs do coincide, as in both cases they point towards a sharp delineation between tumour and surrounding tissue. There are, however, diverging features warranting separation of ACOFs from COFs:
1. clinically, the ACOF occurs at a far lower mean age than the COF: 8.5 years as opposed to 25.1 years [3,16]; and 2. histologically, the ACOF seems to be unique in showing highly cellular garland-like strands with entrapped osteoblasts. These mild dierences may be detected during preoperative assessment of the lesion, allowing a diagnosis of ACOF to be made, but there is no dierence in outcome between patients treated conservatively and those treated by major surgery. Consequently a lesion, even if pre-operatively assessed as ACOF, should be treated, like the COF, with a conservative surgical approach and a frequent and careful follow-up to detect and rapidly treat any possible recurrence.
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