11/13/2005 Case 3: HG malignant extraskeletal myxoid chondrosarcoma

Clinical History:

Case involved an orbital mass in a 15 year old female.  The mass involved the right sphenoid and ethmoid sinuses, optic canal, anterior skull base and right medial maxilla.  It was biopsied in Manhattan and resection carried out in Brooklyn.   Slides showed a vascular, bone forming tumor with lamellar bone and nidus of woven bone.  There were rare mitoses.  There were occasional osteoclasts.  The differential diagnosis includes osteosarcoma, fibrous dysplasia, meningioma, osteoid osteoma (but the mass is >2 cm), and osteoblastoma. 

Diagnostic Notes:

This mass was diagnosed as a “meningioma” in Manhattan.  This mass was called “Fibro-osseous lesion consistent with cementifying variant of ossifying fibroma” by Dr. May Parisien of Columbia.  This entity has also been called “aggressive psammomatoid ossifying fibroma” and “cementifying fibroma.”  This is a locally invasive often (30-58%) recurrent lesion that does not metastasize and for which total excision is the treatment of choice.  It arises in the nasal sinuses and orbit (70%) and also in the palate and anterior fossa.  It may show intracranial extension.  It is most common in teenagers (15 years old) but has been reported from 5-54 years of life.  The male/female ratio is 1.3/1.  It is EMA negative but CK may be positive.  Vimentin is positive.  Reticulin occurs around woven bone.  If you polarize the lesion you well see woven and lamellar bone.  The question may be asked, do reports of “Sino nasal psammomatous meningioma” really refer to this entity?


  • Margo CE, Weiss A, Habal MB.  Psammomatoid ossifying fibroma.  Arch Ophthalmol 1986;104:1347-51.
  • Wells DL, Kaplan K.  Pathologi Quiz Case.  Arch Pathol Lab Med 2003;127(8):e359-e360.
  • Rhodes RH, Davis RL.  An unusual fibro-osseous component in intracranial lesions.  Hum Pathol 1978;9(3):309-19
  • Wenig BM et al.  Aggressive psammomatoid ossifying fibromas of the sinonasal region.  Cancer 1995;76:1155-65.
  • Granado R et al.  Psammomatoid ossifying fibromas:  immunohistochemical analysis and differential diagnosis with psammomatous meningiomas of craniofacial bones.  Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:614.

Slide Image:

view high resolution slide image