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A 17-year-old girl with a history of chronic sinusitis presented with left-sided epiphora, purulent drainage, and pain and right-sided jaw pain. She had previously been treated with antibiotics and mupirocin nasal irrigation, with temporary improvement. The patient and her identical twin sister had required multiple surgical procedures in the past for osseous lesions in the upper and lower jaws, including an aggressive resection via bilateral Caldwell-Luc approach 2 years previously; a computed tomographic scan from that initial presentation is shown in the Figure, A. At the current presentation, examination using flexible nasal endoscopy revealed bulging of the left lateral nasal wall; imaging revealed a 14-mm, dense, expansile, sclerotic mass with moderate calcifications obstructing the left nasolacrimal duct; a similar 2.3 × 1.8–cm lesion of the right mandibular body; and evidence of previous sinus surgery (Figure, B). Because of these findings, left-sided endoscopic dacryocystorhinostomy and sinus surgery was performed. Histologic findings are shown in the Figure, C and D.
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B. Gigantiform cementoma
Microscopic study showed a lesion formed by whorling, swirling spindle cells with islands of basophilic cementum that appeared concentrically laminated. There were also fragments of bone with marrow spaces containing fibrous tissue and toothlike fragments. On immunohistochemical staining, lesional cells were positive for vimentin and negative for epithelial membrane antigen, cytokeratin, smooth-muscle actin, CD31, CD34, S-100, and β-catenin. The pathologic studies confirmed the diagnosis of familial gigantiform cementoma.
Gigantiform cementoma is a rare form of a fibro-cemento osseous lesion that is usually inherited in an autosomal dominant fashion with variable penetrance; when this occurs in families, it is known as familial gigantiform cementoma (FGC). In the present case, both the patient and her identical twin were found to have a de novo, balanced 1;13 translocation mutation presumably causing osseous dysplasia affecting the maxilla and mandible. Although there was no family history of GC for this patient and her twin, there have been reports of FGC in other families in the literature, with discussion of the varying characteristics of this disease.1- 4
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Corresponding Author: Amrita Ray, DO, MPH, Department of Otolaryngology, University of Mississippi Medical Center, 2500 N State St, Jackson, MS 39216 (firstname.lastname@example.org).
Published Online: February 21, 2019. doi:10.1001/jamaoto.2018.4264
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient’s father for granting permission to publish this information. Tina R. Woods, DMD (Department of Oral Maxillofacial Surgery and Pathology, School of Dentistry, University of Mississippi Medical Center, Jackson), performed the histopathologic analysis; she was not financially compensated for her contribution.
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