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Clinical Challenge
September 2016

A Woman With Painful Soft-Tissue Nodules of the Forehead

Author Affiliations
  • 1Naval Medical Center San Diego, San Diego, California
JAMA Otolaryngol Head Neck Surg. 2016;142(9):911-912. doi:10.1001/jamaoto.2016.0079

A woman in her 70s had a 2-week history of frontal headaches and forehead nodules (Figure, A) with no associated fevers, vision changes, or neurologic symptoms. Her medical history was significant for extensive craniofacial polyostotic fibrous dysplasia (FD), breast adenocarcinoma, and stage IV clear cell renal cell carcinoma with metastasis to the ipsilateral adrenal gland treated with radical nephrectomy. Physical examination was remarkable for soft, tender nodules at the forehead and right orbit with right proptosis. Neurologic examination results were normal, except for hypesthesia in a V1 distribution. Sinus computed tomography showed a soft-tissue mass with involvement of multiple craniofacial bones with orbital, anterior, and posterior table defects. Magnetic resonance imaging with contrast (Figure, B) showed interval growth at the frontal bones in the region of previously diagnosed FD. A fine-needle aspiration (FNA) biopsy established the diagnosis. Romanowsky-stained direct smears (Figure, C) showed loose billowing clusters of epithelial cells involved by strands of magenta extracellular tissue (black arrowhead). Cytoplasm was abundant with coarse and fine textures. Nuclei were large and round, occasionally with conspicuous nucleoli (blue arrowheads). Hematoxylin-eosin staining (Figure, D) showed cells with abundant clear cytoplasm and nuclei with varying nucleolar prominence (blue arrowheads). Immunohistochemical stains were positive for pancytokeratin, vimentin, CD10 and PAX-8. A carbonic anhydrase IX (CA-IX) stain was noncontributory.