Desmoplastic fibroma, report of a rare case in infraorbital rim

Abstract

Desmoplastic fibroma (DF) is a rare and locally aggressive intraosseous tumor with unknown etiology. The mandible is the common site of involvement in the maxillofacial region. However, it is believed that DF can arise in any bone of the body. A wide age distribution has been reported for DF occurrence, extending from birth to the sixth decade of life, with a peak incidence at 10 to 19 years of age. In this study, diagnostic and therapeutic management of a 6-year-old girl with a desmoplastic fibroma of the inferior orbital rim and zygomatic buttress are discussed. Cone beam computed tomography (CBCT) revealed a mixed lesion in infraorbital rim, which had ill-defined borders and a straight thick bony septum inside the lesion. It also involved the zygomatic process of maxilla and zygomatic bone. According to radiologic concepts, this rare lesion may mimic fibro-osseous, benign and especially malignant lesions. Regarding different treatment plans, identification of this lesion is essential. Furthermore, presence of coarse and irregular or straight septa along with some imaging criteria for malignant lesions such as destruction of the cortex, periosteal reaction and soft tissue invasion would be helpful to differentiate this lesion from malignant and multilocular benign lesions.