Aggressive fibrous dysplasia of the maxillary sinus

Lorraine G. Shapeero, Daniel Vanel, Lauren V. Ackerman, Marie Josée Terrier-Lacombe, Dominique Housin, Guy Schwaab, Robert Sigal, Jacques Masselot

Research output: Contribution to journalArticlepeer-review

Abstract

Fibrous dysplasia is usually a slowly progressive, benign disease that develops over several years and presents with deformity or mild symptomatology. Five of 34 patients (ages 4-21 years), who were subsequently diagnosed histologically as having fibrous dysplasia of the maxillary sinus, rapidly developed soft tissue masses of the malar region over a period of less than 4 months with accompanying pain (2 patients) and nasal obstruction and exophthalmos (2 patients). Each was clinically suspected of having a sarcoma; two had been thought to have an "osteofibrosarcoma" on initial biopsy at outside hospitals. After resection, all lesions developed regrowth. At histopathologic examination, both initial and recurrent masses proved to be typical fibrous dysplasia with spicules of woven bone in cellular, sometimes vascular, fibrous tissue. No malignant degeneration was found. On conventional radiography, aggressive fibrous dysplasia produced opacification and expansion of the maxillary sinus and apparent disruption of its wall with an associated soft tissue mass. Computed tomography (CT) demonstrated voluminous heterogeneous masses with "ground glass appearance", calcifications, areas of enhancement, low attenuation, cystic areas, and a thinned, sometimes interrupted, maxillary wall. Despite the aggressive clinical course for both initial and recurrent lesions, the CT findings of a "ground glass" mass with calcifications surrounded by a maxillary sinus wall, even if incomplete, can suggest the diagnosis of aggressive fibrous dysplasia.

Original languageEnglish
Pages (from-to)563-568
Number of pages6
JournalSkeletal Radiology
Volume22
Issue number8
DOIs
Publication statusPublished - Nov 1993

Keywords

  • Bones, dysostoses
  • Bones, fibrous dysplasia
  • Bones, oseochondrodysplasia
  • Paranasal sinuses, computed tomography

ASJC Scopus subject areas

  • Radiological and Ultrasound Technology
  • Radiology Nuclear Medicine and imaging

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