Combined medullary hemangioblastoma and syringomyelia in a patient with von-Hippel-Lindau disease. Pathological study

A patient is presented with what was clinically diagnosed as a solitary medullary haemangioblastoma. One year later she was operated from a cerebellar haemangioblastoma. The neuro-radiologic findings showed all the characteristics of von Hippel-Lindau's disease, except the retinal haemangioblastoma. The patient died five years later as a result of deglutition problems with aspiration pneumonia. Post-mortem examination showed that the tumor level was in the posterior right side of the dorsal spinal cord. A syringomyelic cavity spread above the tumor up to the medulla - without communicating with the fourth ventricle and beneath the tumor down to the lowest part of the thoracic spinal cord. A large venous piamater stasis and a thick glial wall of the cavity - mostly below the tumor - suggested a lack of reabsorption of liquids produced by the tumor.