Histopathological changes in malignant melanomas of the choroid after cobalt plaque therapy.

A series of 23 eyes removed after cobalt plaque therapy for malignant melanoma of the choroid is presented, and the histological changes are described with particular reference to the presence or absence of necrosis in the tumours. In 17 of the 23 eyes no evidence of tumour necrosis was found, though all showed evidence of radiation changes in the adjacent tissues. Necrosis was found in 6 cases but was prominent in only 2, one of which was the only example of a mixed-cell tumour, the other 22 being spindle-cell tumours. Enucleation became necessary on average at about 32 months after treatment (23 out of 100 treated eyes) because of obvious failure of treatment with evidence of continued growth, or because of radiational complications leading to a painful blind eye. The 5-year survival rate was 86% compared with about 50% when the treatment is enculeation.

Formes frustes of Marfan's syndrome presenting with severe aortic regurgitation. Clinicogenetic study of 18 families.

Eighteen patients who presented with severe aortic regurgitation and dilatation of the ascending aorta were found to be formes frustes of Marfan's syndrome and formed the basis for this clinicogenetic study. All had aortic valve replacement and reconstruction of part of the ascending aorta. The diagnosis was confirmed by histological examination of the aortic tissue. There were 126 first degree relatives; 85 were living and 67 (78-8%) of these were examined. Limited information was available about 32 of the 41 relatives who had died. No relative had the classical clinical features of Marfan's syndrome but stigmata of the disease were found in 25 (37-3%) of the 67 first degree relatives examined. In 21, the abnormality was confined to the cardiovascular system, the skeleton, or the eye, but in 4, abnormalities involved 2 systems. Cardiovascular abnormalities affecting the aortic valve or aortic wall were present in 6 (9-0%) of the 67 first degree relatives examined. One or more of the skeletal indices measured (height-span difference, metacarpal index, phalangeal index) was abnormal in 18 (26-9%) and ocular abnormalities were found in 5 of 51 (9-8%) examined. There were no relatives with dislocation of the lens or iridodonesis. Using strict diagnostic criteria, a minimum of 37-3 per cent of the first degree relatives examined were affected; this involved 12 of the 18 families studied. There was nothing in our data to suggest that the formes frustes of the disease had a different mode of inheritance from the classical syndrome.