Pediatric facial masses are infrequently encountered and consequently include a wide differential diagnosis. Desmoplastic fibromas (DFs) are rarely encountered in the maxillofacial region and are hence seldom considered in a child with a facial mass; they are classified as fibrous intraosseous tumors with a poorly understood pathogenesis.
Although benign, DFs are locally aggressive, with a high likelihood of recurrence. These lesions rarely involve the maxillary bone with only a few reports in the literature of involvement of that site. Furthermore, the reports suggest that these tumors originate from the posterior part of the maxillary sinus, with extension into the skull base and nasopharynx. We present a case of a DF arising from the anterior maxillary sinus in a pediatric patient.
Figure 1. Axial computed tomographic images demonstrating a well-circumscribed, expansile, lucent lesion centered in the left maxillary bone.
Figure 2. Coronal reconstructed computed tomographic image showing expansion and thinning of the left inferior orbital rim by the tumor.
Figure 3. Plump to slender nuclei demonstrate no atypia and a rare mitotic figure (hematoxylin-eosin, original magnification ×400). There is diffuse nuclear and cytoplasmic β-catenin expression with immunohistochemical staining (inset, original magnification ×400).
Figure 4. Postoperative coronal reconstructed computed tomographic image demonstrating resolution of all tumor-related mass effect and no evidence of residual disease.
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