Embryonal sarcoma of the orbit. A clinical review of 19 Danish cases.
The present study deals with 19 Danish cases of embryonal sarcoma of the orbit. Eight patients are still alive, however, only six with long-period survival (8-21 years). The contralateral orbit (and eye) remained uninvolved in the 19 cases; accordingly the six long-period survivors all have normal "social" vision. In 17 cases the homolateral eye was removed early or late in the clinical history. Only one of the long-period survivors has preserved the homolateral eye (and with useful vision, 6/24 after surgery for irradiation cataract). The nation-wide investigation covers a period of 22 years. It reflects the difficulties in diagnosing orbital tumours and the changes in therapeutic approach to date. Megavoltage radiotherapy plus chemotherapy immediately after the initial biopsy is now considered the therapy of choice; exenteration of the orbit is performed only in the case of a recurrence. Against the background of the present and an earlier study, centralization of Danish orbital malignancies in infants and children is advocated.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003718:Denmark; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008637:Mesenchymoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D009364:Neoplasm Recurrence, Local; D009918:Orbital Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy