Ethmoidal fibro-osseous lesion in a child. Diagnostic and therapeutic problems

A 4-year-old Caucasian male presented with marked proptosis of the right eye of 3 months' duration. A large soft tissue mass occupied fully the right ethmoid sinus; radiologic evidence of extensive bone destruction suggested a malignant tumor. Unexpectedly, a biopsy of the ethmoid tumor was interpreted as benign. Over the next 2 months, this lesion progressed rapidly, with further bone destruction and extension into the anterior cranial fossa. The histopathologic features of a resection specimen were so variable that a wide range of diagnoses was offered by several consultants who examined the material. This lack of agreement among experts in the field of bone diseases considerably influenced the treatment program selected for this patient.