[Neoplastic space-occupying lesions of the orbit. I. Review; hemangioma, lymphangioma and embryonal rhabdomyosarcoma]

Abstract

From 1987 to 1990 182 patients were treated for orbital lesions in the Department of Ophthalmology of the University of Zürich. 37% were tumors (without tumors of the vascular system), 20% inflammatory lesions, 12% lesions of the vascular system and tumors of it, the rest was not diagnosed. On this background the first of two chapters on orbital tumors deals with diagnosis, differential diagnosis, therapy and follow up of the capillary hemangioma, cavernous hemangioma, lymphangioma and embryonal rhabdomyosarcoma. Although ultrasound echography, CT, MRI and angiography have greatly changed the diagnostic work up of orbital lesions, diagnosis is by far not made straight forward but often only after errors. To enhance diagnosis in the future this study is meant to analyse our misinterpretations. In the case of capillary hemangioma only complications justify an active treatment, normally the lesion resolves spontaneously. The infiltration of the upper eyelid with ptosis needs to be treated because of the risk of amblyopia. Our cases demonstrate, that the attempt of treatment with steroids is justified. Embolisation in the region of the a. ophthalmica is quite dangerous. The cavernous hemangioma only needs treatment in the case of compression of the optical nerve and total excision is not mandatory. The lymphangioma, although classified as benign tumor, can only be excised subtotally and with the danger of traumatising important structures of the orbit. The infiltrative growth and tendency of recurrence in fact is dangerous. It is important to diagnose the tumor without biopsy and to wait with surgical treatment as long as possible. The embryonal rhabdomyosarcoma itself is rare but it is the most frequent primary malignant tumor in childhood. Even accounting for being familiar with mimikri-false history of trauma, inflammation, hemangioma-like angiography-the diagnosis is often made rather late. Our cases demonstrate this and also the change of treatment in the last 30 years. Unfortunately the prognosis of the good results of chemotherapy in combination with radiation if necessary is clouded by the occurrence of secondary malignant tumors.