Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Fibro-osseous Lesions of the Facial Bones The concept of "fibro-osseous lesions" of bone has evolved over the last several decades to include two major entities: fibrous dysplasia and ossifying fibroma, as well as other less common lesions such as florid osseous dysplasia, periapical dysplasia, focal sclerosing osteomyelitis, proliferative periostitis of Garré, and osteitis deformans. Historically, ossifying fibroma has had a relatively short recognition and the literature has been intertwined with that involving fibrous dysplasia, which is now known to represent a distinctive clinicopathologic entity. In 1872, Menzel gave the first description of a variant of ossifying fibroma called cemento-ossifying fibroma, in a 35 year old woman with a long-standing large tumor of the mandible. Montgomery was the first to coin the term "ossifying fibroma" (1927), and Liechtenstein and Jaffee were the first to introduce the term "fibrous dysplasia" (1938). Liechtenstein and Jaffee also described the monostotic form of fibrous dysplasia, which had been recognized in the polyostotic form by Albright in 1937 as osteitis fibrosa disseminata, in association with precocious puberty and abnormal pigmentation; i.e., Albright's syndrome. Liechtenstein and Jaffee also classified ossifying fibroma as a histologic variant of fibrous dysplasia, a concept that persisted in the literature until 1963, when Reed separated the lesions into distinct clinicopathologic entities. Reed viewed fibrous dysplasia as an arrest of bone maturation at the woven stage of development, whereas ossifying fibroma was believed to be a benign neoplasm of bone, in which normal bone architecture was replaced by a tissue composed of collagen fibers, fibroblasts, and various amounts of calcified tissue with the potential for unlimited and destructive growth. In 1973, Waldron and Giansanti, espoused a now generally held view that no clinicopathologic parameters predict aggressiveness in the usually benign-behaving ossifying fibroma. However, Margo et al have recently challenged this view in describing a series of patients with an aggressive variant, psammomatoid ossifying fibroma, which may histologically predict destructive behavior. The histologic classification of fibro-osseous lesions of the bone has been widely debated and remains a source of confusion and controversy. In short, the major controversies are: 1. identifying the tissue of origin, 2. distinguishing ossifying fibroma from fibrous dysplasia, 3. separating ossifying fibromas into meaningful pathologic categories, and 4. identifying and predicting aggressive lesions histologically. The debate as to tissue of origin is of little clinical significance, as long as one differentiates ossifying fibromas from fibrous dysplasia. Hamner et al advocated the periodontal origin of ossifying fibroma. The periodontal ligament has been shown to be capable of producing cementum and osteoid, both of which are characteristically found in ossifying fibromas. Krausen et al and Spjut et al, however, postulated that primitive mesenchymal cells in areas such as the ethmoid bone and long bones may produce cementum at sites distant from odontogenic tissue. They discredited the notion that these tumors arise from ectopic periodontal tissue in these locations. Histologically, fibrous dysplasia has been most difficult to separate from ossifying fibroma. Reed used the presence or absence of woven and lamellar bone. In uncomplicated cases, fibrous dysplasia contains no lamellar bone, but rather has arrested woven bone. Ossifying fibroma, on the other hand, contains woven bone often rimmed by osteoblasts that have laid down layers of lamellar bone. Additionally, ossifying fibroma may have areas of cementum, appearing as psammoma bodies embedded in a benign fibrous stroma. Spjut et al supported Reed's classification and stated that the predominant pattern of bone architecture is usually apparent and allows one to differentiate between these two lesions. Although histologic variants such as cemento-ossifying fibroma and cementifying fibroma are identified by some authors, most are considered to behave similarly and have no predictive or prognostic value. Ossifying fibroma is usually seen between the second and fourth decades of life, with women being affected more often than men, with ratios on the order of between 1.8:1 and 5:1. Unlike fibrous dysplasia, ossifying fibromas are characteristically monostotic. Less than 5% involve more than one bone and they are almost exclusively found in the cranial bones, with only a rare case reported in the long bones. Of the cranial bones, the mandible is the most common site (75% in some series), followed by rarer reports of the ethmoid, frontal, and sphenoid sinuses, as well as the orbit, occiput, and temporal bone. Although ossifying fibroma is usually a benign, slow-growing, painless, and often asymptomatic tumor, a rapid growth pattern with a "malignant" or aggressive behavior is sometimes noted, particularly as stated earlier, when the tumor is located outside the mandible. With involvement of the mid-face and paranasal sinuses, patients commonly have a painless swelling of the cheek, unilateral proptosis with diplopia, persistent nasal obstruction, rhinorrhea and epiphora, and recurrent epistaxis and hemoptysis. Although recurrences and disfigurement occur, death from these lesions is rare and usually results from intracranial extension and meningitis. One problem not adequately addressed in the literature is the behavior of ossifying fibromas located outside the mandible. Waldron and Giansanti's often quoted article mentions 37 mandibular and six maxillary lesions, from which they concluded that there was no consistent relationship between location and aggressiveness. Hammer et al retrospectively studied patients in the files of the Armed Forces Institute of Pathology and the National Institutes of Health. Of 237 patients reviewed, 30 had maxillary lesions. Hammer observed that in the maxilla these lesions tended to show more aggressive behavior and osseous destruction. Dehner also found, in a retrospective review of eight children with ossifying fibroma, that maxillary lesions were more aggressive. The five children with maxillary disease required 14 operations to eradicate disease, compared with five operations in the three with mandibular disease. Traditionally, the initial treatment of ossifying fibroma has been simple curettage. More definitive resection has been reserved for recurrent disease. This has particularly been espoused in the oral surgery literature, with reference to treatment of mandibular lesions. As postulated earlier, however, these lesions behave less aggressively and are less likely to recur. A review of the literature concerning ossifying fibromas of the mid-face, however, suggests that a more aggressive en bloc resection may be advisable initially to prevent recurrence and the potential for more complicated and mutilative surgery. This view is supported by Margo et al, who described 21 patients with ossifying fibromas of the ethmoids, frontal sinus, and orbit. Nine had one or more recurrences after curettage and required subsequent operations. Janecka and Hausepian reported 10 patients who required craniofacial resection for ossifying fibroma involving the orbit, and Blitzer et al recently added five more. Case Presentation A 7-year-old boy was referred to the Texas Children's Hospital for evaluation of a right nasal mass. Physical examination revealed a hard friable mass that totally obstructed the nares, severely displacing the septum into the left nasal cavity. Mild proptosis of the right eye was also noted. A transnasal biopsy revealed ossifying fibroma. Further workup consisted of axial and coronal computerized tomography. The child underwent a lateral rhinotomy with medial maxillectomy, ethmoidectomy, and sphenoidectomy. Postoperatively, he had an uneventful recovery. He has been seen in follow-up at three years without evidence of recurrent disease. Bibliography Blitzer A, Post KD, Conley J: Craniofacial resection of ossifying fibromas and osteomas of the sinuses. Arch Otolaryngol Head Neck Surg 115:1112-5, 1989. Dehner LP: Tumors of the mandible and maxilla in children: A clinicopathologic study of 46 histologically benign lesions. Cancer 31:364-84, 1973. Eversole LR, Leider AS, Nelson K: Ossifying fibroma: A clinicopathologic study of sixty-four cases. Oral Pathol 60:505-11, 1985. Hamner JE III, Lightbody PM, Ketcham AS, Swerdlow H: Cemento-ossifying fibroma of the maxilla. Oral Surg 26:579-87, 1968. Hamner JE III, Scofield HH, Cornyn J: Benign fibro-osseous jaw lesions of periodontal membrane origin: An analysis of 249 cases. Cancer 22:861-78, 1968. Janecka IP, Hausepian E: Craniofacial approach to ossifying fibromas. Laryngoscope 95:305-6, 1985. Khalil MK, Leib ML: Cemento-ossifying fibroma of the orbit. Can J Ophthalmol 14:195-200, 1979. Krausen AS, Gulmen S, Zografakis G: Cementomas. II. Aggressive cemento-ossifying fibroma of the ethmoid region. Arch Otolaryngol 103:371-3, 1977. Kridel RWH, Miller RH, Greenberg SD: Ossifying fibroma: Diagnostic clarification. Otolaryngol Head Neck Surg 91:568-73, 1983. Levine PA, Wiggins R, Archibald RWR, Britt R: Ossifying fibroma of the head and neck: Involvement of the temporal bone - an unusual and challenging site. Laryngoscope 91:720-5, 1981. Liechtenstein L, Jaffee HL: Fibrous dysplasia of bone: Conditions affecting one, several or many bones, graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism, and still other extra-skeletal abnormalities. Arch Pathol 33:777-816, 1942. Margo CE, Ragsdale BD, Perman KI, Zimmerman LE, Sweet DE: Psammomatoid (juvenile) ossifying fibroma of the orbit. Ophthalmology 92:150-9, 1985. Margo CE, Weiss A, Habal MB: Psammomatoid ossifying fibroma. Arch Ophthalmol 104:1347-51, 1986. Marvel JB, Marsh MA, Catlin FI: Ossifying fibroma of the mid-face and paranasal sinuses: Diagnostic and therapeutic considerations. Otolaryngol Head Neck Surg 104:803-808, 1991. Miller SI: Resident's page. Pathologic Quiz - Case I. Arch Otolaryngol 105:742-5, 1979. Montgomery AH: Ossifying fibroma of the jaw. Arch Surg 36:874-98, 1938. Reed RJ: Fibrous dysplasia of bone: A review of 25 cases. Arch Pathol 75:480-95, 1963. Spjut HJ, Dorfman HD, Fechtner RE, Ackerman LV: Tumors of bone and cartilage, in: Atlas of Tumor Pathology, Sect II, Fasc. 5. Washington, DC, Armed Forces Institute of Pathology, 1971. Waldron CA, Giansanti JS: Benign fibro-ossesous lesions of the jaws: A clinical-radiologic-histologic review of sixty-five cases. Oral Surg 35:190-201, 1973. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
|