Presenting Secondary ever, this had been done by Collins ten years earlier", Lindau!": II in 1926 and 1930 reported for the first time that tumours of the retina and posterior fossa had the same histological appearance. He also discovered and pointed out that some patients with retinal and posterior fossa tumours had kidney, liver and pancreatic cysts, renal carcinomata and pheochromocytoma. Schuback'? in 1927 was the first to introduce the term "von Hippel-Lindau syndrome" to describe patients with retinal or posterior fossa tumours, together with cysts of carcinomas in various abdominal organs. However, it would seem that Collins has been done an injustice.
SymptomatologyAlthough the disease is recognised during the third, fourth and fifth decades of life, it has also been observed in a three year old child13. In a series of 78 patients', 12 per cent of the disease was diagnosed between the ages of 12 and 20.
SymptomsThe main presenting symptom is headache suggestive of raised intracranial pressure, occipital or frontal or of unspecified location. Other presenting or secondary symptoms are summarised in Table 1. Table 1. Presenting and secondary symptoms of von Hippel-Lindau syndrome.