Fibromyxoma is a rare odontogenic tumour which is benign, but locally aggressive. The etiology of these tumours is unknown, but because of its limitation to the teeth bearing areas and occasional presence of odontogenic epithelial fragments within the tumour which suggest that it is of odontogenic origin. It is a slow growing painless tumour that frequently occurs in second and third decades of life. Females are more commonly affected than males. The tumour can cause gradual expansion of the cortical plates and cause loosening and displacement of teeth, although root resorption may be rare. The surgical treatment of these tumours consists of complete enucleation or radical excision. The aim of this paper is to present the rarity of a fibromyxoma of the maxilla.
Fibromyxoma is a benign tumour of ectomesenchymal origin with or without odontogenic epithelium [
A 12-year-old female patient visited our unit with a chief complaint of swelling on left side of the face since 6 months. Extraoral examination revealed diffuse swelling over the left cheek involving lateral part of the nose and infraorbital region (Figure
Preoperative frontal view showing swelling on left side of the face.
Orthopantomograph showing ill-defined mixed radiolucency in the left maxilla with displacement of the adjacent teeth.
Coronal CT scan section showing the lesion completely obliterating the left maxillary sinus.
Axial CT Scan sections showing the lesion perforating the anterior wall of the antrum and its extension in to the nasal cavity.
Exposure of the tumour by an intraoral approach.
Curettage and complete removal of the lesion.
Gross appearance of the tumour mass.
Histopathological examination showing spindle-shaped fibroblasts in loose myxoid tissue.
Postoperative image showing intraoral healing.
Fibromyxoma is a rare aggressive intraosseous lesion derived from embryonic mesenchymal tissue associated with odontogenesis. The maxilla and anterior region of the mandible are rarely affected. When found in the maxilla it usually behaves more aggressively than that of the mandible. It involves the zygoma, maxillary sinus, and even the orbits.
Evidence that may support the odontogenic origin of fibromyxoma of the jaws was contributed by Thoma in 1934. He stated that the greater portion of myxomas that occur in the jaws are derived from embryonic tissues of the dental papilla, the dental follicle, or the periodontal membrane [
Radiographically, the fibromyxomas present themselves as multilocular or unilocular radiolucency with well-defined borders. Most of them are multilocular. Radiological investigations reveal homogenous radiolucencies with different appearances like honey comb, soap bubble, or tennis racket [
Grossly the tumour is greyish-yellow multinodular tissue. Its consistency varies. Some portions of the tumour may be sticky, gelatinous, or semisolid, and others may be firm. The surface of the tumour is shiny and glistening.
Histologically the fusiform or stellate cells are elongated, having cytoplasmic processes stretching in various directions. It contains few cells that lie in a myxoid ground substance being elongated with spindle-shaped nuclei and having the appearance of fibrocytes. It is of diagnostic challenge to the examiners because it is difficult to differentiate from the other odontogenic tumours. Mitotic figures are rarely seen. The ultrastructural studies of an odontogenic myxoma demonstrate the presence of two basic functional types of cells: secretory and nonsecretory. The secretory cell type is the principal tumour cell and resembles the fibroblast [
The tumour is not radiosensitive, and hence surgery is the only treatment of choice. The surgical treatment of the fibromyxoma involves enucleation and curettage, radical excision, en bloc resection. The avoidance of recurrence is strongly related to the complete resection of the lesion. Thomas has stated that recurrence of fibromyxoma is uncommon if enucleation is complete. Myxomas or fibromyxomas show a recurrence rate between 25% and 43%. Harder et al. stated that rate of recurrence varies widely as does the choice of treatment. Their study supports conservative surgery as the appropriate treatment for odontogenic myxomas, as they have found no evidence of malignant change or increasingly aggressive behaviour in any of the recurrences [
Chen et al. proposed conservative treatment as the preferred method for excision with extensive resection reserved for large tumors. In the long bones the tumour is frequently malignant and tends to recur with great frequency after removal, but fibromyxomas of the jaws appear to have a better prognosis.
In conclusion, fibromyxomas of the jaws are rare but not uncommon. The prime treatment considerations include the age of the patient and recurrence of the lesion. The tumour is not radiosensitive, and surgery is the only choice of treatment. In this young unmarried girl as the extent of lesion is confined to the maxillary sinus, we have opted for an intraoral conservative surgical approach.
We sincerely thank Dr. Vittal Kavuri MD Chief anaesthesiologist, Mediciti Hospital, Hyderabad for his support and contribution in the surgical intervention of this patient.