PMC10242096_01
(A) Frontal photograph revealing bilateral swelling, right more severe than left, with bitemporal narrowing and a long philtrum. (B) Lateral photograph showing retrognathia. (C) Marked brachydactyly with broad distal phalanges. (D) Intraoral mass with dental crowding, malocclusion, and a high-grooved palate.
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F1
Additional clinical findings included bifrontal narrowing with frontal prominence, proptosis, a flattened nasal bridge with a convex nasal profile, a long philtrum, and retrognathia (Figures 1A,B). He had brachydactyly with broad short distal phalanges (Figure 1C). Oral examination showed prominent dental decay, over-retained primary teeth, impacted permanent teeth with displacement and overcrowding, and intraoral extension of the mass lesions (Figure 1D).
Radiological investigations. (A) CT scan of the face showing expansile, mixed-density masses involving the maxilla and mandible bilaterally. The maxillary lesions are large, resulting in remodeling of the maxillary bones causing obliteration of the maxillary sinuses, nasal cavity remodeling and obstruction, atrophy of the hard palate, and displacing the teeth. (B) Chest x-ray with generalized sclerosis. (C) Osteolysis of distal phalanges and generalized sclerosis.
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F2
Several special investigations were conducted; skeletal x-rays showed generalized sclerosis with acro-osteolysis, and a CT scan of his head revealed expansile mixed-density masses involving the maxillae and mandible bilaterally (Figure 2).
(A) Low-power view showing mucosal stratified squamous epithelium with an unencapsulated lesion in the subepithelial stroma. (B) Lesion is predominantly composed of aggregates of multinucleated osteoclast-type giant cells with admixed mononuclear spindle cells.
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F3
The histopathology of the biopsied lesion showed aggregates of multinucleated osteoclast-type giant cells with admixed mononuclear spindle cells and intervening fibrous bands (Figure 3).