Krieglsteiner S., Lüchtenberg M., Rosenkranz C., Vanselow K., Zubcov A.

Augenklinik, Johann Wolfgang Goethe-Universität, Theodor-Stern-Kai 7, D -60590 Frankfurt/Main

Case report: An 11-year-old patient presented to our department of paediatric ophthalmology with a 2-week-history of a cystic tumor in the anterior part of the left eye and a slight deterioration in visual acuity without response to topical antiobiotic therapy. The clinical examination showed a cystic irregular conjunctival tumor of 1 x 2 cm at the temporal inferior part of the limbus of the left eye, salmon-pink colour, not firmly attached to the sclera and did not induce an exophthalmos. Motility was not disturbed, the pupillary reaction was normal. Best corrected visual acuity was 0.9. As a malignant process was suspected (rhabdomyosarcoma or lymphoma) a biopsy was taken immediately. The histology revealed a neurofibroma. Intraoperatively two more tumors were found. In a second operation the tumor was removed completely and the histological finding confirmed a neurofibroma. The pediatric examination didn`t show any systemic manifestation of a generalized neurofibromatosis. In rapidly growing orbital and epibulbar tumors in children the high malignant rhabdomyosarcoma have always to be considered. The tumor has a salmon-pink appearance and has its origin in the striated musculature of the extraocular muscles. It typically occurs in the first decade of life. The diagnosis should be confirmed by biopsy immediately and therapy has to follow (chemotherapy, radiotherapy, orbital exenteration) depending on the staging. The lymphoma has its climax in the 5. - 8. decade of life and is rarely found in childhood in the orbita. It frequently presents bilateral and is treated by chemo- or radiotherapy depending on the staging.
Conclusion: In fast growing tumors of the orbit biopsy has to be performed quickly to exclude a malignancy.

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