Isolated cementoossifying fibroma of the ethmoid bulla: a case report.

Abstract

Cementoossifying fibroma is a rare nonodontogenic tumor of the periodontal membrane that arises from the mesodermal germ layer This nonneoplastic, locally destructive tumor has occurred as an osseous lesion in the mandible, the maxilla, the zygoma, all the paranasal sinuses, and the orbital and petromastoid regions. It has occurred as an extraosseous lesion in the gingiva and the auricle. The diagnosis requires correlating a variety of clinical, radiologic, and histologic factors. The recurrence rate is high, particularly for lesions in the paranasal sinuses. Surgical management via a local excision as wide as possible is suggested. We describe the case of a 32-year-old woman with an isolated cementoossifying fibroma of the right ethmoid bulla, and we review the diagnosis, differential diagnosis, and management of this tumor

Introduction

Fibroosseous lesions are benign heterogenous tumors that have various classifications, diagnoses, and treatments. They are made up of fibroblast and collagen fibers that contain varying amounts of mineralized material rather than normal bone structure. Cementoossifying fibroma (COF) is the most common benign fibroosseous neoplasm of the jaw. This lesion, which can be either unilocular or multilocular, may give rise to a slight growth of the jaw, thereby causing cosmetic and functional deformities. (1) The lesion is generally believed to originate in the ectopic multipotential blast cells of the periodontal membrane (2) (i.e., the mesodermal germ layer surrounding the roots of the teeth) and in endosteal fibrous tissue. (3)

While these lesions are commonly seen in bone, their existence has been reported in soft tissue, as well. Most osseous lesions have been reported in the mandible; other sites include the maxilla, the zygoma, all the paranasal sinuses, and the orbital and petromastoid regions. (2-4) Most extraosseous lesions have been reported in the gingiva (5) and the auricle. (6)

Trauma is widely accepted as the predisposing factor for these lesions. (2,4-7) Exodontia and periodontitis have been shown to induce jaw lesions. (5) COF generally appears during the second, third, and fourth decades of life, most often in the mandibular premolar-molar region. (5,8,9) Lesions are more common in women than in men. (1,2)

We present an unusual case of COF that was localized and isolated to the ethmoid bulla.

Case report

A 32-year-old woman presented to our clinic complaining of headache. Her history was significant for surgery to reduce the inferior nasal concha 10 years earlier. Nasal endoscopy revealed a bilateral defect of the inferior concha that was attributable to the earlier operation and to the plenitude of the right ethmoid bulla determined during 0[degree] nasal endoscopy (figure 1). Findings on the remainder of the ENT examination were normal. Computed tomography (CT) of the paranasal sinus demonstrated that a mixed radiopaque/ radiolucent lesion was occupying the entire right ethmoid bulla and that it was filled with patch-like calcifications (figure 2). We made a diagnosis of a fibroosseous lesion, a fungal infection, and a nonspecific infection.

The patient underwent endoscopic sinus surgery. During the operation, the frontal wall of the ethmoid bulla was completely removed, revealing the lesion. The mass was round and contained no bleeding points. Its appearance was that of an accumulation of particles resembling white sand surrounded by a network of thin bones (retiform bony trabeculae). After the lesion was completely removed, the walls of the ethmoid bulla were resected. The size of the entire mass was 2 x 2 x 2 cm.

A sample of the mass was gray-brown and made up of soft-tissue components with the unevenly distributed sand-like particles. Hematoxylin and eosin (H&E) staining revealed different forms of bony trabeculae in the stroma, which was made up of fibroblastic cells arrayed in uneven storilorm patterns (figure 3, A). Of note, the basophilic cementoid bodies around the core of some of the bony trabeculae were surrounded by osteoblastic cells. The appearance of the cementoid bodies and bony trabeculae after staining with Masson trichrome was typical (figure 3, B). Based on these histopathologic findings, we made a diagnosis of COF.

At follow-up 10 months postoperatively, intranasal examination and paranasal sinus CT detected no evidence of recurrence.

Discussion

The World Health Organization lists four kinds of fibroosseous lesions: COFs, fibrous dysplasias, ossifyin fibromas, and cementifying fibromas. (10) COFs are mixed lesions that contain both cementum-like calcifications and bony materials. During the embryonic period, the medial portion of the nasal drill passes through the ethmoid region during normal transposition, and a minor part of this mesenchyme differentiates into the periodontal membrane. Therefore, as was demonstrated in the case of our patient, ethmoid localization of COF may be explained as an incomplete migration and differentiation of the periodontal membrane. (11)

Trauma has been reported to be the most important factor in the development of COF. (2,4-7) Our patient had undergone an operation involving the inferior nasal concha 10 years earlier, but we do not know whether she experienced damage to the ethmoid region during that operation. Our patient was a middle-aged woman, and several articles have indicated that this is a characteristic finding in COF. (1,2,5,8,9)

Radiographically, COF appears as an intraosseous lesion surrounded by a unilocular, well-defined sclerotic border. This characteristic easily distinguishes COF from fibrous dysplasia. (4) COF can manifest in a radiolucent, radiopaque, or mixed form depending on the amount of mineralized tissue that is present. The degree of opacification increases with the degree of maturation of the lesion. (8) The lesion in our patient appeared to be of the mixed variety.

From a histologic perspective, the term droplets of cementum (or cementicles) is frequently used in defining COF. (10) COFs contain thin bony trabeculae, ovoid cementoid deposits, a storiform stroma (which sometimes includes giant cells), a blood-free area defining the specimen's borders, and dense collagen fibers mixed with a cellular stroma.

Three basic subtypes of COF have been identified. The most common subtype contains equal quantities of calcified material and fibroblastic stroma, where round and lobulated cementum-like masses may spread over the entire lesion. The connective tissue is made up of fibroblastic or stellate cells. Typical radiologic findings include a sharp demarcation between radiolucency and radiopacity. A second subtype is dominated by storiform cellularities in the stroma and small amounts of osteoids and bony trabeculae; some cells in the storiform pattern may be compared with potential osteoblasts. This type of COF is radiolucent. The third subtype is best described as a combination of the other two. Our patient's lesion was of the first subtype.

The differential diagnosis includes fibrous dysplasia, osteoid osteoma, osteoblastoma, cementoblastoma, periapical or focal cementoosseous dysplasia, juvenile COF, chronic osteomyelitis, Garre's osteomyelitis, and psammomatoid ossifying fibroma. (1,4,5,8,9) Focal cementoosseous dysplasias contain multiple small fragments without a real capsule, thin bony trabeculae (ginger roots), irregularly shaped cementoid masses, and some loose collagen fibers between the trabeculae and cementoid masses. (1) Fibrous dysplasias characteristically involve irregularly shaped and woven bone trabeculae or vascularized fibrous stromas in various quantities; they do not involve the osteoblastic rims of the trabeculae. Ossifying fibromas involve lamellar bone trabeculae, vascularized fibrous stromas in various quantities, and trabeculae with osteoblastic rims. Psammomatoid ossifying fibromas are notable for the presence of bony spicules and mineralized/calcified psammomatoid material. (12) Osteomas are well-differentiated benign tumors that predominantly contain lamellar bones; ivory and mature types may contain fibrous tissues. The spongy type of osteoma is generally not locally aggressive, as are ossifying fibromas, but these two types of tumors share many histologic similarities. Osteoblastomas contain ample amounts of osteoid produced by overabundant osteoblasts. These tumors vary considerably in size, shape, and staining characteristics; they are both benign. (13)

It is generally agreed that the treatment for COF, which is known to be aggressive and locally destructive, should involve extensive surgical excision to prevent recurrence. (2,4-6,8) Lesions of the paranasal sinuses are associated with a high recurrence rate owing to the difficulty involved in removing lesions from this part of the body. (14) Even though our patient's lesion was located in the ethmoid bulla, endoscopic sinus surgery combined with excision under direct vision was performed, and the entire lesion was removed. Radiotherapy for COF is contraindicated because this tumor is radioresistant and there is a risk of malignant transformation. (15)

References

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(15.)Bundgaard T, Frost-Jensen V, Buhl L. Sarcomatous change in a previously benign osteofibroma in the maxillary sinus. Arch Otorhinolaryngol 1988;245:22-4.

Ismail Yilmaz, MD; Nebil Bal, MD; Levent N. Ozluoglu, MD

From the Department of Otolaryngology-Head and Neck Surgery (Dr. Yilmaz and Dr. Ozluoglu) and the Department of Pathology (Dr. Bal), Baskent University Faculty of Medicine, Ankara, Turkey.

Reprint requests: Ismail Yilmaz, MD. Department of Otolaryngology--Head and Neck Surgery, Baskent University Facuhy of Medicine, Adana Seyhan Hospital, Baraj yolu I.durak No: 37 Seyhan 01110, Adana, Turkey. Phone: [phone]: fax: [phone]; e-mail: [e-mail]

Originally presented as a poster during the XXth Congress of the European Rhinologic Society and the XXIIIrd International Symposium on Infection and Allergy of the Nose: June 18-25, 2004; Istanbul, Turkey.