Pieter Pauw's tumor oculorum: reappraisal of the presumed first description of retinoblastoma in 1597.

Cancer in a young child is unusual and has always aroused curiosity. Proptosis and an enormous, rapidly growing unilateral tumor in a 3-year-old boy prompted Pieter Pauw (1564-1617), a 16th-century anatomist, to perform an autopsy. His published notes from the autopsy were rediscovered in the 19th century, first by the German ophthalmologist and historian Julius Hirschberg and then by Edwin B. Dunphy, from Boston, Mass, who suggested that the child suffered from retinoblastoma, an interpretation that has held sway. Critical translation of the original Latin text suggests that an orbital tumor, such as embryonal rhabdomyosarcoma, would equally well explain Pauw's observations. His description also gives insight into the concept of a mole as a congenital as well as an intrauterine tumor in Renaissance medicine.