Mesenchymal chondrosarcoma of the orbit. Report of three new cases and review of the literature

Abstract

Background: Extraskeletal mesenchymal chondrosarcoma is a rare tumor characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. Considering all sites, long-term survival is approximately 30%. Only seven cases of orbital mesenchy-ma1 chondrosarcoma have been reported.Methods: The records of three cases of orbital mesenchymal chondrosarcoma treated at the Columbia-Presbyterian Medical Center, and the seven previously reported cases of this tumor were reviewed 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 observation, five survived 5 or more years after resection, or after resection plus adjuvant therapy. Two patients died of metastatic disease 2 and 5 years, respectively, after the initial treatment, and one died of pneumonia 6 months after surgery. Review of the histology of the three cases treated at Columbia-Presbyterian Medical Center did not identify distinct 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 From the *College of Physicians and Surgeons, tEdward S.

Mesenchymal Chondrosarcoma of the Orbit

“…1,6,23) CT characteristics included relatively clearly outlined isodense mass with moderately enhanced calcification. 2,5,9,11,20,21) MR imaging characteristics were lower than or equal to brain tissue intensity on T 1 -weighted imaging, isointensity to brain tissue on T 2 -weighted imaging, and moderate enhancement after gadolinium administration. 21) Surgical resection has been the main treatment modality for intraorbital MC ( cases of intraorbital MC, including the present case, treated with surgical removal had good outcomes if the tumors were removed completely.…”

“…8,14,18) The mesenchymal cells are positive for CD99 and vimentin, and the chondroid tissue is positive for S-100 protein. 5,9,11,23) Since the tumor is associated with high rates of recurrence and formation of distant metastasis, the prognosis is generally poor. 8,12,14,18) MC is usually resistant to chemotherapy, and the primary modality of treatment is surgical resection.…”

“…14) Radical resection seems the best option, but there is no consensus on treatment protocols, including appropriate surgical procedures and the effectiveness of chemotherapy and radiotherapy. 8,9,14,20) Intraorbital MC is extremely rare, with only a few reported cases. [2][3][4][5][6][9][10][11][15][16][17][18][19][20][21][22][23] We describe a case of intraorbital MC successfully treated with intraorbital exenteration.…”

Extraconal orbital tumors in children—a spectrum

“…He was treated with radical surgery; however, he was lost to follow-up. This tumor has a tendency to involve soft tissues and bones of the jaw and ribs, and is rarely seen in the orbit [24]. However, microscopically a small biopsy can show only the round cell component with the absence of chondroid areas and hence they need to be differentiated from other round cell tumors.…”

Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults

“…[4][5][6]8,23 In addition, all larger reports about MCS were published more than 2 decades ago. More recent publications encompassing at least 10 patients focused on MCS in specific sites, eg, the sinonasal tract, 24 jaws, 22,26 mandible, 36 orbit, 37 or the central nervous system (CNS). 21 Prognosis in these locations seemed to be better than the historical results from the larger unselected series (best 10-year survival: 56% 26 ).…”