Volume 36, Issue 1 , Pages 82-85, January 2007
Juvenile ossifying fibroma of the maxilla
Article Outline
Abstract
Juvenile ossifying fibroma is a rare fibro-osseous neoplasm in young children. This lesion is locally aggressive and spreads quickly, and because it has a very high recurrence rate complete excision is essential. Reported here is a case of a massive juvenile ossifying fibroma of the maxilla in an 11-year-old male child. A titanium mesh was used to reconstruct the facial contour after a left total maxillectomy, achieving a satisfactory facial appearance.
Juvenile ossifying fibroma (JOF) is a rare fibro-osseous neoplasm that arises within the craniofacial bones in individuals under 15 years of age. Histologically, it consists of a cell-rich fibrous stroma, containing bands of cellular osteoid without osteoblastic rimming, together with trabeculae of more typical woven bone. Small foci of giant cells may be present. The lesion is non-encapsulated but well demarcated from surrounding bone. It is usually asymptomatic, achieving a large size and exhibiting aggressive behaviour1, and is often diagnosed as ‘juvenile ossifying fibroma’, ‘aggressive ossifying fibroma’ or ‘active ossifying fibroma’ in the literature. Owing to of this lesion's aggressive nature and high recurrence rate, early detection and complete surgical excision are essential. Here is reported a case of a massive JOF of the maxilla in an 11-year-old male child.
Case report
An 11-year-old male child presented in August 2005 with a painless, progressive swelling of the left face for 2 weeks. He had no noteworthy family history or past history. Clinical examination revealed a moderately large left facial mass localized to the maxilla. Intraoral examination revealed gross expansion of the left maxillary alveolar process extending to the hard palate (Fig. 1A). There was minimal tenderness on palpation. The left maxillary molar and premolar were loose. There were no palpable cervical or submandibular lymph nodes, and a chest radiograph was also normal.
Fig. 1.
(A) Gross expansion of the left maxillary alveolar process. (B) The lesion was exposed during surgery. (C) The tumour was removed wholly. (D) Postoperative facial appearance.
Plain X-ray radiographs (Water's view and panoramic radiograph) showed a homogeneously hyperdense whole left maxilla and maxillary sinus, and bone destruction and invasion in the molar part of the left maxilla, zygomatic buttress and part of the zygomatic bone. The left maxillary molar and premolar were drifting. CT scan revealed a mixture of radiolucency and radiodensity, and a well-defined osteolytic mass involving the left maxilla, maxillary sinus, alveolar bone and nasal cavity. There was destruction of the medial and antero-lateral walls, and the roof of the left maxillary sinus. The eyeball and cranial nerve were not involved (Fig. 2 A and B).
Fig. 2.
(A and B) Computed tomography (CT) of the skull showing a mixture of radiolucency and radiodensity, and a well-defined osteolytic mass involving the left maxilla, maxillary sinus, alveolar bone and nasal cavity (arrows). (C and D) CT and three-dimensional CT of the skull show that the lesion was completely excised, and the bony defect was bridged using a folded titanium mesh.
A diagnosis of left maxillary tumour was made. As the lesion was so big, a left total maxillectomy was performed. The tumour was removed wholly (Fig. 1 B and C). The bony defect was bridged using a folded titanium mesh to reconstruct the facial contour and to support the orbital contents. The titanium mesh was fixed to the residual zygoma laterally, and the nasal bone and edge of the anterior nasal aperture medially (Fig. 2 C and D). Rotation of a mucoperiosteal palate flap was used to seal the oral cavity from the nasal space. After the operation, the patient had a good facial appearance and oral function, and no obvious facial deformity (Fig. 1 D).
Microscopically, the lesion was non-encapsulated, showing infiltration of surrounding bone structures and reactive new bone formation at the periphery. The lesion consisted of cell-rich fibrous tissue containing bonds of cellular osteoid without osteoblastic rimming, together with slender trabeculae of immature bone containing coarse lacunae with plump osteocytes and lined by a dense rim of enlarged osteoblasts6. Pseudocysts were seen in the adjacent fibrocellular and myxoid stroma (Fig. 3. A, B and D). Mitotic figures were observed in the stroma, especially in the cell-rich areas, but were never numerous (Fig. 3C). On the basis of these findings, the lesion was diagnosed as a JOF of the maxilla (a juvenile trabecular ossifying fibroma).
Fig. 3.
(A) Immature bone trabeculae are lined by a dense rim of enlarged osteoblasts (H&E, ×400). (B) The stroma is rich in cells (H&E, ×200). (C) Mitotic figures are visible in the stroma (arrow) (H&E, ×400). (D) Cellular osteoid without osteoblastic rimming (H&E, ×400).
Discussion
Review of the literature shows that it is difficult to establish definitive diagnosis criteria for JOF. This lesion is often difficult to diagnose from both a clinical and a histopathologic point of view. The main characteristics are: a patient under 15 years of age, the location of the tumour, the radiologic pattern, and tendency to recur.
JOF is often seen in a very young child. In reviews published by Hamner et al.2 and Slootweg et al.7, the mean age of onset was 11.5 and 11.8 years old, respectively. Clinically, this lesion has in general a more aggressive growth rate than ossifying fibroma5. Most cases of maxillary JOF are asymptomatic, as was the present case. The first clinical manifestation is a swelling of the maxilla. When the orbital bone and paranasal sinuses are involved, the patients may develop exophthalmos, bulbar displacement and nasal obstruction. The radiologic features are variable and depend on the tumour's location and the amount of calcified tissue produced by the tumour, and the lesion will show varying degrees of radiolucency.
Histologically, the lamellae are of variable shape, from trabecular to more osteoid, but at no time show ‘Chinese character’ shapes as are seen in fibrous dysplasia of bone. Haemorrhage is not prominent and when present is scattered rather than seen as foci, the latter being common in central giant cell granuloma. No obvious islands of epithelium or individual epithelial cells are present9 as seen in odontogenic fibroma. The stroma shows focal areas with stromal cysts and occasional mitoses. Cementicle-like particles are not seen. At the periphery of the lesion, cortical and reactive bone is sometimes seen.
The clinical management and prognosis of JOF is somewhat uncertain. The recurrence rate ranges from 30% observed by Johnson et al.3 to 58% reported by Makek4. Although many authors favour conservative surgery rather than radical en-bloc resection, immediate recurrence characterized by a high aggressive growth rate and the absence of a distinct separation between tumour and the adjacent bone requires extensive surgery, with wide demolition of the involved bone8, 10. In the present case, because the lesion had grown to a massive size and caused cosmetic and functional problems, a left total maxillectomy was performed. As the patient was a young child, a titanium mesh was used to reconstruct the facial contour and achieve a satisfactory facial appearance. At the same time, rotation of a mucoperiosteal palate flap was used to close the palatal defect, to acquire acceptable oral function. After 3 months of follow up, there was no recurrence.
References
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PII: S0901-5027(06)00312-2
doi:10.1016/j.ijom.2006.06.024
© 2006 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.
Volume 36, Issue 1 , Pages 82-85, January 2007
