Background: Extraskeletal mesenchymal chondrosarcoma is a rare tumor c
haracterized by undifferentiated mesenchymal cells with islands of mat
ure hyaline cartilage. Considering all sites, long-term survival is ap
proximately 30%. Only seven cases of orbital mesenchymal chondrosarcom
a have been reported. Methods: The records of three cases of orbital m
esenchymal chondrosarcoma treated at the Columbia-Presbyterian Medical
Center, and the seven previously reported cases of this tumor were re
viewed to determine clinical characteristics and appropriate therapy.
Results: Nine of the 10 patients were female; age of onset ranged from
10 to 35 years. Of eight patients with at least 2 years of observatio
n, five survived 5 or more years after resection, or after resection p
lus adjuvant therapy. Two patients died of metastatic disease 2 and 5
years, respectively, after the initial treatment, and one died of pneu
monia 6 months after surgery. Review of the histology of the three cas
es treated at Columbia-Presbyterian Medical Center did not identify di
stinct histologic types that might guide therapy. Presenting symptoms
were typical of symptoms of an orbital mass: proptosis, pain, diplopia
, change in visual acuity, ptosis, and tearing. Conclusion: The small
number of reported cases of mesenchymal chondrosarcoma of the orbit pr
events definitive conclusions, but it appears that resection is adequa
te therapy in some cases. Extraskeletal mesenchymal chondrosarcoma of
the orbit may have a better prognosis than tumors in other sites.