Retinal Haemangioblastoma.

ANGIOMATOUS tumours of the retina have been recognized and reported in the literature for many years. Although the first case was recorded by Panas and Remy (1879), and several others followed this publication, retinal angiomatosis was established as a clinical entity, presenting typically in infancy or early childhood, by von Hippel (1904) and was associated with angiomatosis of the central nervous system by Lindau (1927). There is not infrequently a hereditary and familial disposition. Retinal angiomatosis is an uncommon condition. Usher (1935) collected 119 cases from the literature, and Cordes and Hogan (1940) reviewed 135 recorded cases, but the incidence is thought to be higher than suggested by these figures (Reese, 1951). Angiomata of the retina have also been observed in premature infants (von Hippel, 1934; Ashton, 1961). A case of angiomatous tumour of the retina, recently examined in the Pathology Department at the Institute of Ophthalmology, is here reported, not only because of its rarity but also because the growth showed an interesting pathological feature only occasionally seen in angiomata, namely a tendency to form pseudoxanthomatous cells within the tumour. This peculiarity was well described in a case reported by Berger and Valle'e (1931), who applied the name "angio-reticulo-xanthoma"; it has since been recognized, however, that foam cells are a feature of a particular type of cystic angioma occurring primarily in the retina, cerebellum (Anderson, 1961), and spinal cord or its membranes (Rasmussen, Kernohan, and Adson, 1940). To this tumour the term "haemangioblastoma" is now generally applied.