OCULAR MALIGNANT-MELANOMA IN A PATIENT WITH VONRECKLINGHAUSENS-DISEASE

The occurrence of cutaneous malignant melanoma in Von Recklinghausen's disease (neurofibromatosis type 1: NF1) Is very rare despite almost c onstant pigmented skin lesions and a general tendency to neoplasia. Ho wever, uveal malignant melanoma has been reported In 13 patients. We d escribe a further case in a 66-year-old woman. She was treated with se quential monochemotherapy with fotemustine alone, without exenteration . NF1 is perhaps a risk factor regarding the occurrence of uveal malig nant melanomo but there is little available epidemiological evidence. Nevertheless, careful repeated ophthalmological examinations in patien ts with NF1 are essential, especially in cases of pigmented patches of the Iris or choroid. Systematic surveys of large series are required to gain more insight into this problem.