Ancillary testing for metastasis was carried out in all cases with newly diagnosed retinoblastoma. Fifty-two out of 58 tumors showing systemic involvement had either optic nerve or extrascleral extension at the histopathologic examination of enucleation material. Malignant tumours of the eye and adnexa are generally rare, and in the absence of a French national register of declaration of cancers, only an approximate evaluation can be made. We studied the epidemiology of tumours of the eye and adnexa from the laboratory results of anatomical pathology of biopsies examinated at the laboratory of Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts in Paris. Statistical analysis of the results of histological diagnoses, their sites and the personal histories recovered from the laboratory registers from 1925-1995, a period of 70 years, were carried out.
Carcinomas are the most frequent lid malignant pathology whose presence increases with age.
Tumours originating from haematopoetic tissues are often seen in the sub-epithelial tissues of orbit, lids, conjunctiva and lacrimal gland. Seventeen cases displayed massive proptosis, ocular damage and blindness at initial presentation and underwent exenteration as the initial treatment. Two cases were subjected to external beam radiotherapy without invasive surgical procedures. Ten cases regressed spontaneously without treatment. Spontaneous bilateral regression was noted in one case.